ANZAN VIRTUAL 2021 FOREFRONTS— Handbook and MOVING ON Abstracts 19 – 21 May 2021
ASM anzan2021.com Hosted By Australian and New Zealand Association of Neurologists (ANZAN)
145 Macquarie Street Sydney NSW 2000 P: +61 2 9169 4893 E: [email protected] W: www.anzan.org.au
ANZAN Executive Officer Ms Mandy Jones
ANZAN Scientific Contents Program Committee Kaitlyn Parratt (Chair) ANZAN — Host Organisation 1 Rebekah Ahmed Scientific Program Committee 1 Andrew Bleasel Amy Brodtmann Meeting Organiser 1 Jim Burrow Welcome 3 Bruce Campbell Sponsors 5 Michael Dreyer Robert Henderson International Keynote Speakers 11 Jeannette Lechner-Scott Invited Speakers 14 Debbie Mason Awards 26 Carolyn Orr Carolyn Sue Program Information 31 Ian Wilson Daily Program and Abstracts 32 Wednesday, 19 May 33 Meeting Organiser Thursday, 20 May 62 Friday, 21 May 73 Poster Abstracts 78 Poster Listing 146
P: +61 4 2382 7488 (Lejla) P: +61 4 1884 1154 (Cathy) P: +61 4 0028 0811 (Janine) E: [email protected] W: www.anzan2021.com PO Box 2137 Glenelg SA 5045
1 Biogen Australia Pty Ltd, ABN: 30 095 760 115, Macquarie Park NSW 2113. Biogen NZ Biopharma Limited, 188 Quay Street, Auckland, New Zealand. ©Biogen is a registered trademark of Biogen MA Inc. ©2021. Biogen-106663. BIOG0911/EMBC. Date of preparation: April 2021. Welcome
Welcome to the ANZAN Annual Scientific Meeting!
This year’s meeting is a first for ANZAN – the meeting will be delivered online. While this change has been a response to the travel restrictions of the pandemic, it also gives us a chance to try something new. It can’t be denied that an online meeting has advantages in terms of time saving and reduced carbon footprint. This year, the meeting has the theme “Virtual Forefronts”. The ANZAN Scientific Programme Committee has produced an exciting programme that includes talks at the cutting edge of neurological practice. Kaitlyn Parratt and the committee has embraced the challenge of running a virtual meeting and have selected an impressive range of talks from the abstracts submitted by the members. We are grateful for their efforts. The program has undergone some last-minute changes. Unfortunately, due to personal commitments Professor Roos is no longer able to participate in our meeting this year. We wish her all the best at this time. Thankfully, due to the generosity of four renowned International and National speakers the program has not been compromised. Dr Nicolas Davies and Professor Tom Solomon will join us from the United Kingdom to discuss Neurology and COVID and Emerging Brain Infections. Dr Joshua Davis, Infectious Disease Physician from Newcastle will share his clinical pearls in Neuroinfectious disease, and Dr Tim Kleinig will provide a cutting edge update related to COVID vaccination complications. We also will have a full complement of ANZAN awards – the EG Robertson Award will be delivered by Christian Lueck, the JW Lance oration will be delivered by Ingrid Sheffer, the Mervyn Eadie lecture will be given by Mark Cook, the Ian McDonald lecture by Ernie Willoughby, the Leonard Cox award for 2020 by Rebekah Ahmed and the Leonard Cox award for 2021 by Piero Perucca. These speakers are all experts in their fields and ANZAN is proud to have such distinguished members to receive these awards. I would like to thank our sponsors for their strong ongoing support of ANZAN. I encourage everyone to support the exhibitors. Please enjoy this year’s Annual Scientific Meeting, which will showcase the scholarship and expertise of the ANZAN membership and will provide invaluable educational update.
For all the latest information on the Conference, visit Pamela McCombe anzan2021.com President, ANZAN 3 NOW PBS LISTED1
Make treatment an easy part of life:2–4
› New once-daily oral S1P › No first dose monitoring receptor modulator2 or genetic testing2 › Effectively reduces disease › Generally well tolerated†3,4 activity in RMS*2–4
* Significantly reduced annualised relapse rates and lesion activity (mean number of GdE lesions and new/enlarging T2 lesions) vs IFN ß-1a IM (p <0.001)2–4 † Only ~3% of ZEPOSIA patients in Phase III studies discontinued due to adverse events3,4
PBS Information: Authority required (STREAMLINED). For use in patients with relapsing-remitting multiple sclerosis who meet certain criteria. Refer to PBS Schedule for full authority information. Before prescribing, please review the Product Information click here. Abbreviations: GdE: gadolinium-enhancing. IFN ß-1a: interferon beta-1a. IM: intramuscular. PBS: Pharmaceutical Benefits Scheme. RMS: relapsing multiple sclerosis. S1P: sphingosine 1-phosphate References: 1. Pharmaceutical Benefits Scheme. www.pbs.gov.au. 2. ZEPOSIA® Approved Product Information. 3. Comi G et al. Lancet Neurol 2019;18:1009–20. 4. Cohen JA et al. Lancet Neurol 2019;18:1021–33. ZEPOSIA® is a registered trademark of Celgene Corporation. Celgene Pty Limited Australia. Level 15, 60 City Road, Southbank, VIC 3006. ABN 42 118 998 771. Date of preparation: April 2021. 2084-AU-2100121. BMMS21493W. Ward6. Thank you to our Sponsors
The Organising Committee of the Australian and New Zealand Association of Neurologists Annual Scientific Meeting 2021 gratefully acknowledges the support of the following Sponsors:
Gold Sponsor
Biogen Australia
At Biogen, our mission is clear: we are pioneers in neuroscience. Biogen discovers, develops, and delivers worldwide innovative therapies for people living with serious neurological and neurodegenerative diseases as well as related therapeutic adjacencies. One of the world’s first global biotechnology companies, Biogen was founded in 1978 by Charles Weissmann, Heinz Schaller, Kenneth Murray, and Nobel Prize winners Walter Gilbert and Phillip Sharp. Today Biogen has the leading portfolio of medicines to treat multiple sclerosis, has introduced the first approved treatment for spinal muscular atrophy, commercializes biosimilars of advanced biologics, and is focused on advancing research programs in multiple sclerosis and neuroimmunology, Alzheimer’s disease and dementia, neuromuscular disorders, movement disorders, ophthalmology, neuropsychiatry, immunology, acute neurology, and neuropathic pain. For more information, please visit www.biogen.com.au or www.biogen.co.nz
Company Contact | Fiona Tigar | [email protected] | +61 400 741 286
Gold Sponsor
Bristol Myers Squibb
Myers Squibb is a leading global biopharma company focused on discovering, developing and delivering innovative medicines for patients with serious diseases. Our people are focused on helping millions of patients around the world in disease areas such as oncology, hematology, immunology, cardiovascular, fibrosis and neuroscience.
The Bristol Myers Squibb Foundation, an independent charitable organization, promotes health equity and seeks to improve health outcomes of populations disproportionately affected by serious diseases and conditions, giving new hope to some of the world’s most vulnerable people.
Company Contact | Clare Cipa | [email protected] | 0409 862 055
5 Thank You to Our Sponsors
Gold Sponsor
Merck
Merck, a leading science and technology company, operates across healthcare, life science and performance materials. Around 56,000 employees (in 66 countries) work to make a positive difference to millions of people’s lives every day by creating more joyful and sustainable ways to live. From advancing gene editing technologies and discovering unique ways to treat the most challenging diseases to enabling the intelligence of devices – the company is everywhere.
Scientific exploration and responsible entrepreneurship have been key to Merck’s technological and scientific advances. This is how Merck has thrived since its founding in 1668. The founding family remains the majority owner of the publicly listed company. Merck holds the global rights to the Merck name and brand. The only exceptions are the United States and Canada, where the business sectors of Merck operate as EMD Serono in healthcare, MilliporeSigma in life science, and EMD Performance Materials.
Gold Sponsor
Novartis Pharmaceuticals
Novartis is reimagining medicine to improve and extend people’s lives. As a leading global medicines company, we use innovative science and digital technologies to create transformative treatments in areas of great medical need. In our quest to find new medicines, we consistently rank among the world’s top companies investing in research and development. We believe continued R&D is essential to innovation and in Australia last year we invested 20M in clinical trials. Novartis products reach nearly 1 billion people globally and we are finding innovative ways to expand access to our latest treatments. About 105 000 people of more than 140 nationalities work at Novartis around the world. Novartis has more than 60 years’ history in Australia and employ around 600 across its two divisions. For more information, please visit http://www.novartis.com.au
Company Contact | Danijel Jurkovic | [email protected]
6 Thank You to Our Sponsors
Gold Sponsor
Roche Products
Roche is a global pioneer in pharmaceuticals and diagnostics focused on advancing science to improve people’s lives. The combined strengths of pharmaceuticals and diagnostics have made Roche the leader in personalised healthcare – aims to boldly transform and personalise healthcare to prevent, diagnose and treat each and every patient more effectively, while creating a more sustainable healthcare ecosystem.
Roche is the world’s largest biotech company, with truly differentiated medicines in oncology, immunology, infectious diseases, ophthalmology and diseases of the central nervous system. Roche is also the world leader in in-vitro diagnostics and tissue-based cancer diagnostics, and a frontrunner in diabetes management. www.roche-australia.com.
Gold Sponsor
Teva Pharma Australia
Teva is a global pharmaceutical leader, committed to improving health and increasing access to quality health solutions worldwide. Our employees are at the core of our success, with colleagues in over 80 countries delivering the world’s largest medicine cabinet to 200 million people every day. We offer a uniquely diverse portfolio of products and solutions for patients and we’ve built a promising pipeline centred around our core therapeutic areas. We are continually developing patient-centric solutions and significantly growing both our specialty and generic medicines business through investment in research and development, marketing, business development and innovation. This is how we improve health and enable people to live better, healthier lives.
7 Thank You to Our Sponsors
Silver Sponsor
Alexion
Alexion is focused on providing innovative treatments to patients with life-threatening rare diseases. We work in partnership with healthcare providers, patient advocacy organisations and governments to best serve patients. By expanding the knowledge and awareness of rare diseases, we help the medical community improve the process for diagnosis and treatment.
Silver Sponsor
CSL Behring
CSL was established by Australia’s Government in 1916 to ensure an isolated Australia controlled its own supply of biological medicines, particularly in times of war and global pandemics. CSL Behring is Australia’s chosen national plasma fractionator and manufactures a range of life saving plasma-derived therapies for the treatment of the Australian community.
Silver Sponsor
Seer Medical
Seer Medical is an Australian medical technology company that has revolutionised home- based diagnostics for seizure investigation. Their mission is to empower people on their journey to diagnosis and by utilising the latest technology, they are able to deliver deep clinical insights to long-term video-EEG-ECG monitoring.
Silver Sponsor
UCB Australia
UCB aspires to be the patient-centric global biopharmaceutical leader, transforming the lives of people with severe diseases. At UCB our sense of purpose is to help people suffering from severe central nervous system disorders lead normal, everyday lives. Our ambition is to offer them innovative new medicines and ground-breaking solutions that go beyond the drug. We are committed to enabling cutting-edge scientific research that is driven by patients’ needs.
8 Thank You to Our Sponsors
Mobile App Sponsor
Seqirus
Poster Viewing Session Sponsors
Novartis Roche Eisai Lunchtime Lunchtime Lunchtime Wednesday 19 May Thursday 20 May Friday 21 May 12.10pm – 13.00pm 12.00pm – 1.00pm 12.30pm – 1.30pm
Symposium Sponsors
Roche Cutting Edge Updates for Neurologists Wednesday 19 May | 09.30am – 12.10pm
Merck Conceptions and Conquerings Wednesday 19 May | 1.00pm – 3.00pm
Novartis Acute Neurology for Practice Thursday 20 May | 09.30am – 12.00pm
Biogen The Young Achievers Thursday 20 May | 1.00pm – 3.00pm
Bristol Myers Squibb The Borderlands Friday 21 May | 09.30am – 12.10pm
Teva Pupils, Pressure and Pandemics Friday 21 May | 1.00pm – 3.00pm
9 Thank You to Our Sponsors
Sponsors
Allergan Australia
Compumedics
Eisai
Grifols Australia
Lundbeck
Natus Medical
STADA Pharmaceuticals Pty Ltd
10 International Keynote Speakers
Professor Mark Edwards Mark Edwards (MBBS, BSc(Hons), PhD, FRCP, FEAN) is Professor of Neurology at St George’s University of London and The Atkinson Morley Regional Neuroscience Service at St George’s University Hospital. He has a specialist clinical and research interest in Movement Disorders and Functional Neurological Disorder (FND). He did his PhD with Professor John Rothwell and Professor Kailash Bhatia at the UCL Institute of Neurology, studying the pathophysiology of genetic dystonia. During this period he was involved with the development of a novel and now widely used technique for transcranial magnetic stimulation: theta burst stimulation. Following completion of neurology training he became a Senior Lecturer and Honorary Consultant Neurologist at UCL and the National Hospital for Neurology. Here he developed an NIHR funded research program and specialist diagnostic and treatment service for patients with FND. After moving to St George’s in 2015 he expanded this work to develop one of the first integrated diagnostic and treatment services for FND alongside continued research work into the pathophysiology of the disorder and development and testing of novel treatments, including the first randomised trial of specialist physiotherapy for functional movement disorders. He is also an active part of the specialist movement disorders and deep brain stimulation team at the Atkinson Morley Regional Neuroscience Centre, and continues electrophysiological and psychophysical research work into the pathophysiology and treatment of movement disorders in general. He has published over 300 peer reviewed publications and is author of the Oxford Specialist Handbook of Parkinson’s Disease and Other Movement Disorders. He is President of the Association of British Neurology Movement Disorders Group, International Executive Committee member of the International Parkinson’s and Movement Disorders Society, Board and Founding Member of the Functional Neurological Disorder Society, Associate Editor of the European Journal of Neurology, and medical advisor for FNDHope, the UK Dystonia Society and the British Association of Performing Arts Medicine. He is the winner of the Jon Stolk Award for Movement Disorders research from the American Academy of Neurology, the David Marsden Award for Dystonia research, the Queen Square Prize and the Uschi Tschabitscher prize for research from the European Academy of Neurology.
11 International Keynote Speakers
Dr Nicholas Davies Dr Nicholas Davies is a member of the Encephalitis Society’s Medical Advisory Panel. Dr Davies is a general neurologist with a sub-specialty and research interest in neurological infection. He trained in neurology at The National Hospital for Neurology and Neurosurgery, St Mary’s Hospital and St George’s Hospital (Atkinson Morley) in London. His PhD, from King’s College London, addressed the aetiology and outcome of acute encephalitis in adults. After the completion of specialist training, Dr Davies undertook a fellowship in HIV neurology at St Vincent’s Hospital in Sydney, Australia.
Professor Tom Solomon Professor Tom Solomon is Director of the National Institute for Health Research Health Protection Research Unit in Emerging and Zoonotic Infections, and Chair of Neurological Science at the University of Liverpool. He works on emerging pathogens, heading the multi- disciplinary Liverpool Brain Infections Group. He hosts the Scouse Science Podcast, and tweets @RunningMadProf.
12 ® MAVENCLAD PBS cladribine tablets STREAMLINED AUTHORITY IN RRMS1,2
MAVENCLAD® – for all your newly diagnosed and switch RRMS patients2
RRMS, relapsing-remitting multiple sclerosis PBS information: Authority required (STREAMLINED). Refer to PBS Schedule for full authority information. Before prescribing, please review the Full Product Information available from www.medicines.org.au/files/sgpmaven.pdf References: 1. MAVENCLAD® (cladribine) tablets. PBS Streamlined Authority. Available at: www.pbs.gov.au/browse/streamlined-authority. Accessed February 2021. 2. MAVENCLAD® Product Information. Merck Healthcare Pty Ltd | ABN 72 006 900 830. Suite 1, Level 1, Building B, 11 Talavera Road Macquarie Park NSW 2113 Australia. Phone 1800 257 348. | Fax (02) 9975 1516. ® – Registered Trademark. ™ – Trademark. MAV107 | Date of preparation: April 2021 | AU-MAV-00173 Invited Speakers
Rebecca Ahmed Associate Professor Rebekah Ahmed is Director of the Memory and Cognition clinic at Royal Prince Alfred Hospital and Senior Neurologist at FRONTIER, Brain and Mind Centre, University of Sydney. She was awarded the ANZAN Fellowship to the National Hospital for Neurology and Neurosurgery Queen Square London, and Dementia Research Centre, University College London.
Bruce Campbell Professor Bruce Campbell is a consultant neurologist, Head of Stroke and Interim Head of Neurology, Royal Melbourne Hospital. He is a professorial fellow in the Melbourne Brain Centre @ RMH, Department of Medicine, University of Melbourne.
Mark Cook Mark Cook is the director of the Graeme Clark Institute of Biomedical Engineering, Sir John Eccles Chair of Medicine, University of Melbourne, and Director of Neurology at St. Vincent’s Hospital Melbourne. A clinical epileptologist, his recent work has focused on experimental models of epilepsy and seizure prediction, and implantable seizure detection and treatment systems.
Josh Davis Prof Josh Davis is an infectious diseases physician based at the John Hunter Hospital in Newcastle, and a senior clinical research fellow based at the Menzies School of Health Research in Darwin. Josh is the immediate past president of the Australasian Society for Infectious Diseases, and has broad clinical and research interests in infectious diseases, particularly in Staph aureus infections, bone and joint infection, viral hepatitis and infections associated with critical care.
14 NOW APPROVEDTGA
1
POWER† WITH FREEDOM‡ †>50% relative reduction in ARR vs teriflunomide (P<0.001), with nearly 9 out of 10 patients achieving NEDA-3 at year 2 (P<0.001)(post hoc analysis)2,3 ‡Self-administered at-home B-cell therapy1
Not representative of an actual patient.
PBS Information: This product is not listed on the PBS. PLEASE REVIEW PRODUCT INFORMATION BEFORE PRESCRIBING. APPROVED PRODUCT INFORMATION AVAILABLE HERE http://www.guildlink.com.au/gc/ws/nv/pi.cfm?product=nvpkesin10321References: 1. KESIMPTA approved Product Information. March 2021. 2. Hauser SL et al. N Engl J Med 2020; 383: 546–557. 3. Hauser SL et al. Ofatumumab vs teriflunomide in relapsing multiple sclerosis: Analysis of no evidence of disease activity (NEDA-3) from ASCLEPIOS I and II trials. Poster LB62. Presented at the 6th Congress of the European Academy of Neurology: May 23–26, 2020; Virtual. For medical enquiries please contact 1800 671 203 (phone) or [email protected] (email) ® Registered trademark of Novartis AG. Novartis Pharmaceuticals Australia Pty Limited ABN 18 004 244 160. 54 Waterloo Road, Macquarie Park NSW 2113. Ph (02) 9805 3555. April 2021. AU-16234. Invited Speakers
John Dunne Professor John Dunne is a graduate of the University of Western Australia. He has held clinical neurophysiology, EEG and Epilepsy Fellowships at the Mayo Clinic, USA. He is currently: Head of RPH/SCGH EEG labs; Professor, Department of Medicine, University of Western Australia; Secretary General, ILAE-Asia Oceania; Involved in EEG, epilepsy and clinical neurophysiology teaching throughout the Asian and Oceanian region.
Victor Fung Dr Victor Fung is Conjoint Associate Professor at Sydney Medical School, The University of Sydney, and Head of Department and Director of the Movement Disorders Unit, Department of Neurology, Westmead Hospital in Sydney, Australia. He is Co-Chair of the Publications and Communications Oversight Committee of the International Parkinson and Movement Disorder Society (IPMDS) and has been a past Treasurer and past Chair of its Congress Scientific Program Committee. He is Chair of the Asia Pacific Affairs Committee of the Australian and New Zealand Association of Neurologists, and is a past- President of the Movement Disorder Society of Australia and New Zealand (MDSANZ). He was the founding Chairperson of the MDSANZ Clinical Research and Trials Group. He is a member of the Parkinson’s Australia, Parkinson’s NSW and Dystonia Network Australia Scientific Advisory Boards. He serves on the Editorial Board of Movement Disorders, npj Parkinson’s Disease, the Journal of Clinical Movement Disorders, Movement Disorders Clinical Practice and F1000 Research. He has a clinical and research interest in Parkinson’s disease and movement disorders.
Benjamin Jonker Benjamin Jonker is a neurosurgeon whose practice encompasses surgery and stereotactic radiosurgery (Gamma Knife) for brain tumours, trigeminal neuralgia, movement disorders and epilepsy. He has fellowships in stereotactic radiosurgery (UCLA, Los Angeles, USA) and stereotactic and functional neurosurgery (Foothills Hospital, Calgary, Canada). With his neurology and neuroradiology colleagues he introduced MRI guided focussed ultrasound for tremor to Australia at St Vincent’s Hospital, Sydney. Dr Jonker serves on the Board of the Neurosurgical Society of Australasia and the Board of the World Society for Stereotactic and Functional Neurosurgery. He is on the medical advisory panel of the Australian Trigeminal Neuralgia association.
16 ocrelizumab OCREVUS: TWICE-YEARLY D SING * means patients are free 1 to get on with life
1
Please review Product Information before prescribing. OCREVUS is listed on the PBS for RRMS. OCREVUS is not listed on the PBS for PPMS. Refer to PBS Schedule for full authority information. Invited Speakers
Michelle Kiley Dr Michelle Kiley MB, BS FRACP has been the Director of Epilepsy services at the Royal Adelaide Hospital since 2001. She has an active interest in Women and Epilepsy, Status Epilepticus and EEG. She is the Immediate Past President of Epilepsy Society of Australia, and remains on the ESA executive, in addition to being a member of the Driving Committee, Pregnancy Register and chair of the ESA Education and training Committee.
Hanka Laue-Gizzi Hanka Laue-Gizzi is a Neurologist and Epileptologist at the Comprehensive Epilepsy Service at Prince of Wales Hospital and Conjoint lecturer at University of New South Wales, Sydney. Clinical interests include caring for women and young adults with epilepsy, and pre-surgical assessments of patients with medication refractory epilepsy.
Lay Kho Lay graduated from Sydney University in 1997 and underwent neurology training in Western Australia. She is clinical lead of the Stroke Unit at Royal Perth Hospital and was Head of the Neurorehabilitation unit at the State Rehabilitation Service until 2019. She has a special interest in neurological rehabilitation and is chair of the Edith Cowan University’s Neurological Rehabilitation Consultative Committee.
Tim Kleinig Professor Tim Kleinig is Head of the South Australia’s sole Comprehensive Stroke Centre and is the SA Health Telestroke Clinical lead. He is also chair of the SA Stroke Community of Practice and Vice-president of the Stroke Society of Australasia. He has been heavily involved in the recent Australian pivotal acute stroke trials, and continues to be involved in epidemiological stroke research as well as researching acute stroke imaging and treatments, for both intracerebral haemorrhage and ischaemic stroke.
18 Now is the moment to prevent migraines in your patients
Introducing the AJOVY® Momentum Program
Improving the value, outcomes and support Easy to enrol available to adults prescribed AJOVY for the 1,2 • Contact your Teva Representative or the Program Administrator to request the prevention of migraines. Patient enrolment brochure • Provide the brochure to your patients with their private prescription for AJOVY • First and third units of AJOVY provided at $0.00 RRP* • Patients register at • Other units purchased at $284 RRP* • Quality use of medicines (QUM) consultation available at each pharmacy dispense at no cost including: Contact us to gain momentum
» Advice about AJOVY » Adherence tips • 1800 318 278 » Injection training and suppor t » Access to MigraineHQ resources •
This product is not listed on the PBS. Please review the Product Information before prescribing available from http://www.medicines.org.au/files/tbpajovy.pdf
*RRP: recommended retail price. Reference: 1. AJOVY Consumer Medicine Information, September 2019. 2. AJOVY Product Information, February 2021. Zest Healthcare Communications, ABN 81 115 828 941. Suite 1 (Ground Floor), 213 Miller Street North Sydney, NSW 2060. www.zestcom.au.. Zest/TEVSY014 Sponsored by Teva Pharma Australia Pty Ltd, ABN 41 169 715 664. Level 1, 37 Epping Road, Macquarie Park, Sydney NSW 2113. P: 1800 AU TEVA (1800 28 8382) Invited Speakers
Christian Lueck Christian Lueck is Director of Neurology at Canberra Hospital and Professor at the Australian National University. He trained at Queen Square in the UK and was a consultant in Edinburgh before moving to Canberra in 2003. He is interested in many aspects of neurology, particularly neuro-ophthalmology and neurological education.
Debbie Mason Dr Deborah Mason is a consultant Neurologist at Christchurch Public Hospital with a subspecialty interest in neuro-inflammatory disorders and multiple sclerosis. Prior to her appointment in 2003 Dr Mason completed a two year fellowship in multiple sclerosis at the London Health Sciences University Hospital in London, Canada. Dr Mason is a clinical senior lecturer of the Christchurch Campus, University of Otago medical school and has been involved in a number of academic studies including being a lead investigator in the New Zealand Multiple Sclerosis Prevalence Study. She leads the MS research group whose principal focus is clinical research into the disease multiple sclerosis. She has published in the field of MS epidemiology and is the lead investigator of the New Zealand MS Incidence study. She has a number of research collaborations with leading multiple sclerosis researchers around the world and has completed an MRI study measuring perfusion, diffusion and resting-state functional MRI on grey and white matter in patients with early and established relapsing onset multiple sclerosis in collaboration with Professor David Miller, University college, London UK. Dr Mason is also the New Zealand lead investigator for the MS Research Australia funded Vitamin D intervention trial (PreVANZ), and the Australian and New Zealand (ANZ) Neuromyelitis optica study and the ANZgene collaboration. She is also the principal investigator for four sponsor-lead treatment trials in MS and Neuromyelitis optica.
Merrilee Needham Professor Merrilee Needham combines the challenging roles of Consultant Neurologist, Fiona Stanley Hospital, Director of Research, South Metropolitan Health Service, leader of the Myositis Research Group at the Centre of Molecular Medicine and Perron Institute, and Foundation Professor of Neurology at Fiona Stanley Hospital, Murdoch University and Notre Dame University.
20 SEE THE POSSIBILITIES
SOLIRIS® (eculizumab rmc) is now TGA approved for the treatment of adult patients with Neuromyelitis Optica Spectrum Disorder (NMOSD) who are anti-aquaporin-4 (AQP4) antibody-positive.1
Alexion would like to thank the members of ANZAN who have provided valuable input and assistance to the first reimbursement application for Soliris® to the PBAC. We look forward to working together with all stakeholders to ensure long term, sustainable access for people living with this devastating disease.
Reference: 1. Australian Product Information for Soliris® (eculizumab rmc) Approved 01 July 2020.
PBS Information: For the treatment of NMOSD this product is not listed on the PBS. For the treatment of aHUS, Soliris® is listed on the PBS as a Section 100 item. Refer to PBS Schedule for full authority information. For the treatment of PNH, Soliris® is not listed on the PBS but is funded on the Life Saving Drugs Program. Refer to the LSDP website for details.
Please refer to the Full Soliris® Product Information before prescribing, which can be accessed at https://secure. guildlink.com.au/gc/ws/xi/pi.cfm?product=xipsolir
©2021 All rights reserved. Alexion Pharmaceuticals Australasia Pty Ltd. ACN 132 343 036. Frenchs Forest, NSW 2086 Australia. April 2021. AU/SOL-N/0014. Invited Speakers
Piero Perucca Piero Perucca, MD, PhD, FRACP is Associate Professor of Adult Epilepsy at the Department of Medicine (Austin Health), The University of Melbourne, and Director of the Comprehensive Epilepsy Program at Austin Health. He is an academic neurologist, with clinical and research interest in the diagnosis and management of epilepsy.
Ingrid Scheffer Laureate Professor Ingrid Scheffer is a physician-scientist whose work as a paediatric neurologist and epileptologist at the University of Melbourne and Austin Health has led the field of epilepsy genetics over more than 20 years, in collaboration with Professor Samuel Berkovic and molecular geneticists. This resulted in identification of the first epilepsy gene and many more genes subsequently. Professor Scheffer has described many novel epilepsy syndromes and refined genotype–phenotype correlation of many disorders. Her major interests are in the genetics of the epilepsies, epilepsy syndromology and classification, and translational research. She collaborates on research focused on the genetics of speech and language disorders, autism spectrum disorders, cortical malformations and intellectual disability. She led the first major reclassification of the epilepsies in three decades, published in March 2017, for the International League Against Epilepsy Commission for Classification and Terminology. She has received many awards: 2007 American Epilepsy Society Clinical Research Recognition Award, ILAE Ambassador for Epilepsy Award, 2013 Australian Neuroscience Medallion, 2013 Emil Becker Prize for child neurology and the L’Oréal-UNESCO Women in Science Laureate for the Asia-Pacific region for 2012. In 2014, she was elected as a Fellow of the Australian Academy of Science and also elected as Vice- President and Foundation Fellow of the Australian Academy of Health and Medical Sciences. She was a co-recipient of the 2014 Prime Minister’s Prize for Science and she was awarded the Order of Australia in 2014.
Neil Shuey Neil originally trained as an optometrist, before a career change resulted in medical studies and training in Neurology. He is currently Head of the Neuro- Ophthalmology Clinic at the Royal Victorian Eye and Ear Hospital, and Head of the Neuro-Immunology Clinic at St Vincent’s Hospital in Melbourne.
22 Our story continues to be a part of yours CSL Behring is proud to continue to support you and your patients with IVIg and SCIg.
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Discover how CSL Behring can support you and your patients. Connect with us at ANZAN conference.
Ig: Immunoglobulin; IVIg: Intravenous Immunoglobulin; SCIg: Subcutaneous Immunoglobulin. INTRAGAM® 10, PRIVIGEN® and HIZENTRA® are funded under arrangements implemented by the National Blood Authority. Please refer to the National Blood Authority for details. Before prescribing please review Product Information available at www.cslbehring.com. au/products/products-list. CSL Behring (Australia) Pty Ltd. 189–209 Camp Rd, Broadmeadows VIC 3047, Australia. INTRAGAM® 10, PRIVIGEN® and HIZENTRA® are registered trademarks of the CSL Group of Companies. ABN 48 160 734 761. Prepared April 2021. ANZ-HIZ-0243. COR1125. Invited Speakers
Judy Spies After completing neurology training at Royal Prince Alfred Hospital in Sydney Judy undertook a PhD with John Pollard in the peripheral nerve laboratory at the University of Sydney and postdoctoral training with Jack Griffin at Johns Hopkins and Phillip Low at Mayo. She has a career-long interest in immune mediated inflammatory neuropathies and her clinical work focusses on inflammatory neuromuscular disorders, multiple sclerosis, neuroimmunology and autonomic disorders.
Ernie Willoughby Until his retirement from hospital practice in Dec 2020, Ernest Willoughby has been a Neurologist in the Dept of Neurology at Auckland City Hospital since 1979, with associated positions in the Dept of Medicine, at the University of Auckland, School of Medicine. His medical degree (MB ChB) was obtained in 1969 at the Otago School of Medicine, Dunedin, New Zealand, and Fellowship of the Royal Australasian College of Physicians in 1976. From 1976 to 1978 he was a Fellow in Neurology and Immunology at Sloan-Kettering Institute / Memorial Hospital, New York City, USA, and in1978-9 a Research Associate at New York Hospital / Cornell Medical College. Early research interest in multiple sclerosis was in neuroimmunology and later in MR scanning and clinical treatment trials, after a sabbatical year in 1986 at the Health Sciences Centre, University of British Columbia, Vancouver, Canada. His main commitment has been to clinical general Neurology, plus under-graduate and post-graduate teaching in Neurology. He has been director of the Auckland region MS clinic, chairman of the New Zealand MS treatment Assessment Committee, and a member of the International Medical and Scientific Board for MSIF.
24 Diagnose seizures early and accurately Get answers for your patients faster with at-home long-term video-EEG-ECG
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Learn more at Awards
The ANZAN Medal Awarded by the Association to individuals for outstanding contribution to, and ambassador for, the Australian and New Zealand Association of Neurologists.
ANZAN Medal Recipients
2021 Richard Frith 2016 Geoffrey Herkes 2012 Richard Stark 2019 Mervyn Eadie 2015 Pamela McCombe 2011 Not Awarded 2018 David Burke 2014 John King 2010 John Morris, Inaugural Medal 2017 Elsdon Storey 2013 Bill Carroll
The Mervyn Eadie Award For Career Achievement in Neuroscience Research The Mervyn J Eadie Award was introduced in 2001 to honour an ANZAN member who has made a significant contribution to neuroscience research. Prof Eadie is a distinguished neurologist, neuropharmacologist and author from Queensland. He has contributed to the Association in many ways, in particular as the editor of ‘Clinical and Experimental Neurology’, and as the co-author of ‘Neurology in Australia’ and ‘A Directory of Neurology in Australia’. Prof Eadie also wrote the ‘The Flowering of a Waratah – A History of Australian Neurology and of the Australian Association of Neurologists’. This award is made each year in recognition of the recipients’ career achievements and international reputation of excellence in neuroscience research.
Mervyn J Eadie Award Presenters
2021 Mark Cook, ‘Seizures 2013 Ingrid Scheffer, ‘Is the 2006 Prof Graeme Hankey, ‘Stroke and Cycles: Patterns and epilepsy exome changing treatment and prevention: an Prediction’ clinical practice?’ evidence-based approach’ 2019 Carolyn Sue, ‘An unplanned 2012 Pamela McCombe, ‘Something 2005 Not Awarded path to discovering the Old Something New’ 2004 Stephen Davis, ‘Stroke importance of chi’ 2011 Robert Ouvrier, ‘Charcot- in evolution-imaging 2018 Victor Fung, ‘The patient as Marie-Tooth Disease in the and management pearl: Personalised research 19th and 21st Centuries’ 2003 Frank Mastaglia, ‘Parkinsons and treatment in Parkinson’s 2010 Garth Nicholson, ‘Viewing Disease — what role do genes disease’ Diseases through Genes: play?’ 2017 Robert Henderson, ‘Evolution, Seeing History and Looking for 2002 John Pollard, ‘Mechanisms handedness and MND’ Preventions and Cures and management in 2016 Matthew Kiernan, ‘Chance 2009 Philip Thompson, ‘The Signs inflammatory demyelinating and Design: Translations in of a Neurologist’ neuropathy’ Clinical Neurology’ 2008 Prof Geoffrey Donnan, 2001 David Burke, ‘The Properties 2015 Sam Berkovic, ‘Precision ‘Clinical Neuroscience and Functions of Myelinated Medicine: a new Trials in Australia: Is There A Axons’ Neuropharmacology for Future?’ Epilepsy’ 2007 Dr Neil Anderson, ‘Limbic 2014 Elsdon Storey, ‘The neurologist encephalitis, viruses and the arbor vitae’ and antibodies’
26 New TGA indication1 Now approved as add-on treatment for primary generalised tonic-clonic seizures in patients ≥4 years with idiopathic generalised epilepsy1 PBS INFORMATION: Partial seizures. Vimpat® Tablets and Oral Solution: Authority Required (STREAMLINED). Refer to PBS schedule for full authority information. Injection: This product is not listed on the PBS. Primary generalised tonic-clonic seizures. This indication is not listed on the PBS. Please review the full Product Information before prescribing. The Product Information can be accessed at www.ucbpharma.com.au/Vimpat-oral www.ucbpharma.com.au/Vimpat-IV 1. VIMPAT® Australian Approved Product Information. UCB Australia Pty Ltd. (ABN 48 005 799 208). Telephone: +61 (3) 9828 1800.Facsimile: +61 (3) 9828 1860. Level 1, 1155 Malvern Road, Malvern VIC 3144. VIMPAT® is a registered trademark of UCB Pharma GmbH under license. AU-N-VI-EPOS-2100003. April 2021. #8953.
8953_UCB_ANZSGM_Ad_96x138mm_AW03_FA.indd 1 22/4/21 3:08 pm Awards
E. Graeme Robertson Award For Career Achievement and Contribution to Neuroscience and the Australian & New Zealand Association of Neurologists. In 1976 the Council of the Australian Association of Neurologists, as the Association was then known, decided to fund an invited annual lecture in honour of E Graeme Robertson at each Annual Scientific Meeting of the Association. The first E Graeme Robertson Lecture was given in Hobart in 1978. Graeme Robertson was one of five neurologists who founded the Australian Association of Neurologists in 1950, and in 1958 he became our Second President. He was perhaps the first practicising academic Clinical Neurologist in Australia. He was an international authority particularly on pneumoencephalography but, with the advent of modern imaging, pneumoencephalography and much of Robertson’s scientific legacy were consigned to history. His lasting contribution lies in his activities as a conservationist. The annual award of this lecture is made to an ANZAN member who, through their career, has made a substantive contribution to ANZAN and to neurology and neuroscience in Australia or New Zealand.
E. Graeme Robertson Award Presenters
2021 Christian Lueck, ‘The 2007 A/Prof Catherine Storey, 1992 James W. Lance, ‘Interesting Optic Chiasm: Studies in ‘From Vital Spirit to Vital Time’ neurology syndromes’ Architecture and Neurology’ 2006 Prof John Morris, ‘Cases and 1991 Louis R. Caplan, ‘Vertebro- 2019 Richard Macdonell, Faces’ basilar embolism’ ‘Achievements and Challenges 2005 Peter Doherty, ‘Immune 1990 Erik Stalberg, ‘The Value to in Multiple Sclerosis’ Memories are made of this’ the clinical neurologist of 2018 Neil Anderson, ‘The electromyography in the 1990s’ 2004 William Carroll, Spectacular Advance ‘Demyelinating optic 1989 John Morgan Hughes, of the Autoimmune neuropathy: An insight into ‘The molecular biology of Encephalopathies’ the Changing Practice of mitochondrial disease’ 2017 Michael Halmagyi, ‘Disorders Neurology’ 1988 W I McDonald, ‘Optic neuritis of the vestibular system; 2003 Rick Burns and its significance’ what have we learnt in the last 100 years?’ 2002 Sam Berkovic, ‘Causes of 1987 Robert Baloh, ‘Neuro-otology’ epilepsies: how do genetic and 2016 A/Prof Ernest Somerville, 1986 Michael Swash, ‘Neurology of acquired factors interact?’ ‘Self-induced syncope: Not the sphincters’ 2001 Thomas Brandt, ‘The for the faint of heart!’ 1985 H J Barnett, ‘Vascular disease’ Vestibular Cortex: Its 2015 Richard Frith, ‘Is Australasian Locations, Functions, And 1984 Jean Aicardi, ‘Some disorders neurology ready for the next Disorders’ of the central grey matter golden era?’ in children: clinical and 2000 Fred Mendelsohn, 2014 Frank Mastaglia, ‘Immune- radiological diagnosis’ ‘Neuroscience at the Howard mediated inflammatory Florey Institute’ 1983 G Selby, ‘The long-term myopathies’ prognosis of Parkinson’s 1999 Byron Kakulas, ‘Molecular 2013 Con Yiannikis, ‘Evoked disease’ genetics in the diagnosis of potentials: The relationship neuro-muscular disease’ 1982 J M Sutherland, ‘Multiple between structure and Sclerosis fifty years on’ function’ 1998 Not Awarded 1981 S Sunderland, ‘Stretch- 1997 James G. McLeod, ‘Multiple 2012 Stephen Davis, ‘Stroke in compression neuropathy’ Evolution — A Tale of Two Sclerosis in Australia’ 1980 W G Burke, ‘Myasthenia gravis: Strategies’ 1996 Mervyn J. Eadie, ‘Concerning a clinical review’ 2011 David Burke, ‘The Primacy of a very noble lady of a most the Spinal Cord in the Control curious shape’ 1979 D Dastur, ‘Aspects of cuprogenic disorder in Wilson’s of Movement’ 1995 Jerome B. Posner, disease in India: Pathology ‘Neurological paraneoplastic 2010 Ed Byrne, ’Crossing the and pathogenesis of chronic syndromes: a review of Crevasses, New Major myelopathy in atlanto-axial diagnosis and prospects for Challenges for Clinical dislocation, wither operative or therapy’ Neuroscience’ peri-operative haematomyelia 2009 A/Prof Richard Stark, 1994 William Landau or other cord complications’ ‘Headaches and Homicides’ 1993 Anita Harding, ‘Mitochondrial 1978 R Hooper, ‘Graeme Robertson 2008 Prof John Pollard, genes and neurological and the golden age of ‘Mechanisms and Management disease’ neurology’ in Inflammatory Neuropathy’
28 Awards
The Leonard Cox Award Dr. Leonard Cox was a leader in the early development of neurology in Australia and was elected the first President of the Australian Association of Neurologists in 1950. He excelled as a clinician, researcher and educator and has been described as a great innovator, a leader in his profession, beloved of his students yet a man of modesty, generosity and tolerance. The Leonard Cox Award was established in 2000 at the ANZAN Golden Jubilee meeting in Melbourne to recognise an investigator who has contributed significantly to neuroscience research at an early stage of their career. The Scientific Program Committee of ANZAN makes the award based on the quality of the applicant’s body of scientific work. It is open to all members of ANZAN who have been awarded their FRACP within the previous 10 years.
Leonard Cox Award Presenters
2021 Piero Perucca, ‘The Genetics 2015 Merrilee Needham, ‘Trying 2007 Dr David Williams, ‘The of Focal Epilepsies: From to understand Inclusion Body tau of PSP: untangling Research to Clinical Practice’ Myositis: The importance of the diagnosis of atypical collaboration’ Parkinsonism’ 2020 Rebekah Ahmed, ‘Physiological changes 2014 Simon Lewis, ‘The many 2006 Dr Lata Vadlamudi, ‘Genetics in Neurodegenerative (masked) faces of Parkinson’s’ of Epilepsy: The Testimony Dementias’ of Twins’ 2013 Bruce Campbell, 2019 Tomas Kalincik, ‘Multiple ‘Individualizing Ischemic 2005 Not Awarded Sclerosis: Navigating Stroke Management Using 2004 Peter Batchelor, ‘Neural Treatment Choice’ Advanced Imaging’ regeneration after spinal 2018 Parvathi Menon, ‘ALS 2012 Not Awarded cord injury’ Pathogenesis: Site of Disease 2011 Steve Vucic, ‘Novel insights 2003 Carolyn Sue, ‘Expanding the Onset and Mechanisms into the Pathophysiological umbrella of mitochondrial Underlying Disease Spread’ Mechanisms of ALS’ disease’ 2017 Michelle Farrar, ‘Findings 2010 Arun Krishnan, ‘Novel Clinical 2002 Matthew Kiernan, in Nerve Neverland: A Paradigms for Neuropathy ‘Demyelinating neuropathies’ neuromuscular adventure’ Identification and Treatment’ 2001 Simon Koblar, ‘Wiring The 2016 Dr Kishore Kumar, ‘Use of 2009 Robert Henderson, ‘A Tale of Nervous System — A Matter Next Generation Sequencing Three Cities’ of Guidance’ to Shed Light on Movements Disorders and Mitochondrial 2008 Dr Mark Parsons, ‘Guiding 2000 Trevor Kilpatrick, ‘LIF and Disease’ Stroke therapy with advanced Life for Academic Neurology’ brain imaging’
29 Awards
W. Ian McDonald Lecture Ian McDonald was an outstanding academic neurologist, a true friend of many in this Association, a mentor and supporter of trainees and someone who will be greatly missed by all neurologists in Australia and New Zealand. He changed the study of multiple sclerosis from one of ad hoc clinical observations into a highly productive discipline based on first class clinical science. Following his untimely death in 2008, the W. Ian McDonald lecture was introduced by ANZAN to honour his enormous contribution to world neurology and to reflect the great warmth felt towards him by so many members. It is awarded each year to a distinguished neurologist, particularly one from the field of multiple sclerosis.
W. Ian McDonald Lecture Presenters
2021 Ernie Willoughby, ‘MS Redux: 2015 Sean Pittock, ‘NMO Spectrum 2011 Michael Hanna, Looking back over 48 years’ Disorders’ Translational Research in Muscle Channelopathies — 2019 Jan Hillert, ‘Patient value of 2014 Michael Pender, ‘The role Genetics, Disease Mechanisms multiple sclerosis research – of Epstein-Barr virus in the and Treatment Trials’ from genes to quality of care’ pathogenesis of multiple sclerosis’ 2010 Alastair Compston, ‘Limiting 2018 Gavin Giovanonni, ‘Trialling and repairing the damage in new drugs and approaches 2013 Alberto Ascherio, ‘Can we Multiple Sclerosis’ in progressive multiple prevent multiple sclerosis?’ sclerosis’’ 2009 David Miller, ‘Perspectives 2012 Satoshi Kuwabara, of Multiple Sclerosis over 30 2017 Xavier Montalban, ‘Multiple ‘Neuromyelitis Optica: Years’ Sclerosis: A 30‑Year Tale’ Concepts in Evolution’ 2008 Inaugural lecture: Prof 2016 Mary Reilly, ‘Emerging Andrew Lees (UK), ‘The Therapies in Hereditary Parkinson sphinx’ Neuropathies’
James Lance Oration
James Lance Oration Presenters
2021 Ingrid Scheffer, ‘The promise 2019 David Burke of precision medicine for the epilepsies’
Young Investigator Awards
Jim McLeod Advanced Trainee Award For work presented at the ANZAN ASM and performed during the clinical years of Advanced Training.
Jim Lance Young Investigator Award For work presented at the ANZAN ASM and performed during pursuance of higher degree.
30 Program Information
Welcome Reception and ANZAN Electronic Posters Council Reception for Advanced Electronic Posters will be displayed for the Trainees duration of the virtual meeting and can be The Welcome Reception and ANZAN Council viewed at any time. They will be grouped Reception for Advanced Trainees will be held according to the category they were submitted 15:00 – 16:30 Australian Eastern Standard Time under, and featured on relevant days. (AEST) in Sydney, Melbourne, Brisbane, Adelaide, Launceston, Newcastle and Wellington. Wednesday, 19th May Poster Viewing Session 1
Attendance Certificate Proudly sponsored by Career achievements and Young Investigators, Your certificate of attendance will be emailed to COVID and non COVID neuroinfectious disease all registrants after the ASM. and Emergency Neurology.
CME Points Thursday, 20th May Poster Viewing Session 2 The ANZAN ASM may attract RACP Continuing Professional Development (CPD) points at the Proudly sponsored by rate of credit point per hour under Category 2: Functional Neurology and Clinical differentiation, Group Learning Activities. More information is and Cross speciality forefront updates. available at www.racp.edu.au/mycpd. Friday, 21st May Poster Viewing Session 3 Mobile App Proudly sponsored by Proudly sponsored by Cross speciality forefront updates. CrowdCompass is again the official ANZAN ASM 2021 smartphone app. Available to you at no cost as an ANZAN ASM delegate, CrowdCompass puts our event in the palm of your hand! You can use the app to look at the program, see profiles of all Sponsors and their staff, keep up with real time alerts and news during the event, find the people you’re looking for and exchange contact details and much more.
31 Daily Program and Abstracts Wednesday 19 May 2021
09:15 – 09:30 Meeting Opening Pamela McCombe
ANZAN Medal Announcement
09:30 – 12:10 Cutting Edge Updates for Neurologists Chairs: Kaitlyn Parratt and Jeanette Lechner Scott Symposium sponsored by Roche
09:30 Keynote Talk Nicholas Davies (UK) Neurology and COVID
10:00 COVID Q&A time Nicholas Davies (UK)
10:15 Concussion and CTE Lay Kun Kho
10:35 MS Therapies and complications Deborah Mason
10:55 Cancer Therapy Neurology Update Judy Spies
11:15 New in Neuromuscular Merrilee Needham
11:35 Cerebral venous sinus thrombosis from vaccine-induced Timothy Kleinig immune thrombotic thrombocytopenia (VITT)
11:50 Q&A time All Speakers
12:10 – 13:00 Lunch Break Poster Viewing Session 1 Sponsored by Novartis
13:00 – 15:00 Conceptions and Conquerings Chair: Lynette Kiers Symposium sponsored by Merck
13:00 Jim Lance Oration Ingrid Scheffer The promise of precision medicine for the epilepsies
13:30 – 15:00 Breakout Session 1 | Immunology Chairs: Jennifer Massey and Zara Ioannides
13:30 Neurological manifestations of coronavirus disease 2019: a Jonathon Fanning comprehensive review
13:37 Synaptic Antibodies and Spinal Predominant Demyelination: a John Parratt form of Multiple Sclerosis
13:44 Autoimmune encephalitis antibody biomarkers: frequency, age Amy Kunchok and sex associations
13:51 Pregnancy-related relapse in natalizumab, fingolimod and Wei Yeh dimethyl fumarate-treated women with multiple sclerosis
13:58 Psoriasis in multiple sclerosis: an Australian prevalence study Varitsara Mangkorntongsakul
33 14:05 Comparison of multiple disease modifying therapies in multiple Ibrahima Diouf sclerosis with marginal structural models
14:12 Q&A time All Speakers
13:30 – 15:00 Breakout Session 2 | Immunology Chairs: Ruth Leadbetter and Kevin O’Connor
13:30 Immunotherapy responsive neuropathic pain associated with Darshi Ramanathan LGI1 and CASPR2 antibodies
13:37 Peripheral Monocyte Populations in the Autoimmune Robb Wesselingh Encephalitis Post-acute Phase
13:44 Disease reactivation after cessation of disease-modifying Izanne Roos therapy in relapsing-remitting multiple sclerosis
13:51 Predicting Infection Risk in Multiple Sclerosis Patients Treated Nabil Seery with Ocrelizumab: A Retrospective Cohort Study
13:58 Real-world experience with Ocrelizumab in the MSBase Anneke Van der Walt Registry – Australian RRMS cohort
14:05 Worsening longitudinal reaction time trajectories using the Daniel Merlo MSReactor computerised battery predicts confirmed EDSS progression
14:12 Q&A time All Speakers
13:30 – 15:00 Breakout Session 3 | Neurodegeneration Chairs: Carly Oboudiyat and Campbell Le Heron
13:30 Volumetric and connectivity profile of regional thalamic Sicong Tu abnormality in amyotrophic lateral sclerosis
13:37 Progression of clinical features in Lewy body dementia can be Elie Matar detected over six months
13:44 Visualising the topographic pattern of tau deposition in Lucy Vivash patients with Progressive Supranuclear Palsy using PI2620‑PET
13:51 Gut microbiota and nutritional profiles of Parkinson’s Michal Lubomski disease patients
13:58 Genetic epidemiology of Motor Neuron Disease: Royal Brisbane Po Sheng Yang and Women’s Hospital Cohort
14:05 Neurophobia: A study of Australian medical students and junior Alex Yeung doctors
14:12 Q&A time All Speakers
13:30 – 15:00 Breakout Session 4 | Epilepsy and Miscellaneous Chairs: Zebunessa Rahman and Andrew Neal
13:30 Optimising selection for epilepsy surgery Anthony Khoo
13:37 Differentiating status epilepticus from prolonged psychogenic Tracie Tan non-epileptic seizures – can peripheral cell ratios help?
13:44 Real-time capture of patient-reported outcomes using a digital Wendyl D’Souza platform – a pilot study
13:51 Relationships between Cognitive Impairment and Clinical Wendy Wang Features of Idiopathic Intracranial Hypertension
13:58 Nitrous oxide induced myelo-neuropathy Grace Swart
14:05 Development of a bedside motion capture system: a pilot study Paul Kopanidis
14:12 Q&A time All Speakers
34 13:30 – 15:00 Breakout Session 5 | Neuromuscular and Stroke Chairs: Belinda Cruse and Antonia Carroll
13:30 Andersen-Tawil Syndrome: Multi-System Deep Phenotyping of Vinojini Vivekanandan a large UK cohort
13:37 A Cross-Sectional Study of Patients with Chronic Nicholas Crump Inflammatory Demyelinating Polyneuropathy (CIDP): Identifying Ultrasonographic Features for Diagnosis and Prognosis
13:44 Posturography as a biomarker of IVIG efficacy in CIDP patients Matthew Silsby
13:51 Effect of Inter-hospital transfer in patients undergoing Leon Edwards endovascular thrombectomy in the early and late time window
13:58 Adjunctive intraarterial thrombolysis in endovascular clot William Diprose retrieval: a systematic review and meta-analysis
14:05 High Sensitivity Troponin in Acute Ischaemic Stroke Study (TACIS) Andrew Hannaford
14:12 Q&A time All Speakers
13:30 – 15:00 Breakout Session 6 | Infectious Diseases Chairs: Zoe Woodward and Susan Tomlinson
13:30 Brain aging and cardiovascular risk factors in chronic HIV: A David Jakabek longitudinal MRI study
13:37 COVID-19 disease outcomes in a UK Myasthenia Centre during Paul Kopanidis the first year of the pandemic
13:44 Cognitive deficits are associated with anosmia but not anxio- Bruce Brew depressive symptoms in COVID-19
13:51 COVID:19 The Epicentre of Neurological Events in the Human Nicholas Parsons Brain
13:58 MR spectroscopy and dynamic contrast-enhanced perfusion Xin Zhang studies in two SARS-CoV-2 infection patients with neurological complications and no other MR abnormalities
14:05 Case-control study of risk factors for stroke among critically-ill Jonathon Fanning patients with SARS-CoV-2: an analysis of the COVID-19 Critical Care Consortium (CCCC) global registry
14:12 Q&A time All Speakers
15:00 End of Breakout Sessions
15:00 Ian McDonald Lecture Chair: Deborah Mason MS Redux: Looking back over 48 years Ernie Willoughby
15:00 Meeting Close Day 1
15:00 – 16:30 Welcome Reception and ANZAN Council Reception for Advanced Trainees
35 Wednesday 19th May 2021
Cutting Edge Updates for Neurologists Chairs: Kaitlyn Parratt and Jeanette Lechner Scott
Symposium sponsored by Roche
09:30 | Keynote Talk | Cutting Edge Updates for Neurologists Neurology and COVID
Nicholas Davies
10:15 | Cutting Edge Updates for Neurologists Concussion and CTE
Lay Kun Kho
This presentation gives an overview of chronic traumatic encephalopathy. It discusses the history, epidemiology, symptomatology, diagnosis and pathophysiology of this condition as we currently understand it. It also touches briefly on current management and research issues.
10:35 | Cutting Edge Updates for Neurologists MS Therapies and complications
Deborah Mason
10:55 | Cutting Edge Updates for Neurologists Cancer Therapy Neurology Update
Judy Spies
11:15 | Cutting Edge Updates for Neurologists New in Neuromuscular
Merrilee Needham
The world of neuromuscular neurology is an exciting one. Whilst there continue to be improvements to our diagnostic armamentarium, the most exciting recent developments are in therapeutics, with disease-specific therapies beginning to complement supportive care measures.
36 11:35 | Cutting Edge Updates for Neurologists Cerebral venous sinus thrombosis from vaccine-induced immune thrombotic thrombocytopenia (VITT)
Timothy Kleinig
In recent weeks data has accumulated linking adenovirus-vector COVID-19 vaccines to otherwise rare thrombotic events, in particular cerebral venous sinus thrombosis. Although this is still a rapidly evolving story, a causal link now seems certain. Preliminary epidemiological data suggests this condition occurs exclusively after the first vaccine, with a time-lag of between 4-20 days and with a predilection for younger females. Studies strongly implicate a heparin-induced thrombocytopenia- like mechanism involving platelet activating antibodies targeting platelet factor-4. The condition has been termed vaccine-induced immune thrombotic thrombocytopenia (VITT). Diagnostic and treatment strategies are discussed.
Conceptions and Conquerings Chair: Lynette Kiers
Symposium sponsored by Merck
09:45 | Jim Lance Oration | Conceptions and Conquerings The promise of precision medicine for the epilepsies
Ingrid Scheffer
Breakout Session 1 | Immunology Chairs: Jennifer Massey and Zara Ioannides
13:30 | Breakout Session 1 | Immunology Neurological manifestations of coronavirus disease 2019: a comprehensive review
Jonathon Fanning2, 1, Samuel Huth1, Sung-Min Cho3, Chiara Robba4, David Highton5, Denise Battaglini4, 6, Judith Bellapart-Rubio1, 7, Jacky Suen8, Gianluigi Li Bassi8, 9, Fabio Taccone10, Rakesh Arora11, Glenn Whitman12, John Fraser1, 8
1. Faculty of Medicine, The University of Queensland, Brisbane, QLD, Australia 2. Department of Neurology, Gold Coast University Hospital, Southport, QLD, Australia 3. Neurosciences Critical Care Division, Departments of Neurology, Neurosurgery and Anesthesiology and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, MA, USA 4. San Martino Policlinico Hospital, IRCCS for Oncology and Neuroscience, University of Genoa, Genoa, Italy 5. Department of Anaesthesia and Perioperative Services, The Princess Alexandra Hospital, Woolloongabba, QLD, Australia 6. Department of Medicine, University of Barcelona, Barcelona, Spain 7. Adult Intensive Care Services, Royal Brisbane and Women’s Hospital, Brisbane, QLD, Australia 8. Critical Care Research Group, The Prince Charles Hospital, Chermside, QLD, Australia 9. Institut d’Investigacions Biomediques August Pi I Sunyer, Barcelona, Spain 10. Department of Intensive Care, Hôpital Érasme, Brussels, Belgium 11. Cardiac Sciences Program, St. Boniface General Hospital Research Centre, Winnipeg, Manitoba, Canada 12. Cardiac Intensive Care Services, Johns Hopkins Hospital and University, Baltimore, MA, USA
37 Background | There is growing evidence that SARS-Cov-2 infection is associated with severe neurological complications. Understanding the nature and prevalence of these neurologic manifestations is essential for identifying higher-risk patients and projecting demand for ongoing resource utilisation. This review and meta-analysis report the neurologic manifestations identified in hospitalised COVID-19 patients and provide a preliminary estimate of disease prevalence.
Methods | MEDLINE, Embase and Scopus were searched for studies reporting the occurrence of neurological complications in hospitalised COVID-19 patients.
Results | A total of 2207 unique entries were identified and screened, among which 14 cohort studies and 53 case reports were included, reporting on a total of 8,577 patients. Central nervous system manifestations included ischemic stroke (n=226), delirium (n=79), intracranial haemorrhage (ICH, n=57), meningoencephalitis (n=13), seizures (n=3), and acute demyelinating encephalitis (n=2). Peripheral nervous system manifestations included Guillain-Barrè Syndrome (n=21) and other peripheral neuropathies (n=3). The pooled period prevalence of ischemic stroke from identified studies was 1.3% [95%CI: 0.9% – 1.8%, 102/7715] in all hospitalised COVID-19 patients, and 2.8% [95%CI: 1.0% – 4.6%, 9/318] among COVID-19 patients admitted to ICU. The pooled prevalence of ICH was estimated at 0.4% [95%CI: 0%-0.8%, 6/1006].
Conclusions | The COVID-19 pandemic exerts a substantial neurologic burden which may have residual effects on patients and healthcare systems for years. Low quality evidence impedes the ability to accurately predict the magnitude of this burden. Robust studies with standardised screening and case definitions are required to improve understanding of this disease and optimise treatment of individuals at higher risk for neurologic sequelae.
13:37 | Breakout Session 1 | Immunology Synaptic Antibodies and Spinal Predominant Demyelination: a form of Multiple Sclerosis
John DE Parratt1
1. Royal North Shore Hospital, St Leonards, NSW, Australia
Objective | To describe the clinical, radiological and pathological features of a novel demyelinating disease.
Methods | 105 patients with multiple sclerosis (MS) were examined for phenotypic differences radiologically, and their sera were tested for novel autoantibodies using a solid phase, indirect, immunofluorescence method. The characteristics of patients that had a specific autoantibody in their sera, were compared to a cohort of 163 conventional MS patients (CMS).
Results | Three of 105 (2.8%) MS patients had a serum autoantibody directed against vesicles of the Purkinje cell layer, synaptic molecules in the molecular layers of the cerebellum and hippocampus and the surface of choroid plexus epithelium. AQP4 and MOG antibodies were absent. Oligoclonal bands were absent in one patient. None of the other MS patients, 64 healthy controls or 78 other neurological controls exhibited this antibody.
Patients with synaptic antibodies had recurrent transverse myelitis, occasional optic neuritis and short, swollen cord lesions affecting the grey matter of the cord, in acute phase. Spinal cord demyelination was much more common in these patients (2.8 cord to 1 brain lesion) compared to CMS (1 cord to 5.8 brain lesions). Antibody positive patients suffered disease reactivation on Fingolimod (n=1) or responded poorly to Natalizumab (n=1). The disease was controlled with plasma exchange and Rituximab. The autoantibody bound to choroid plexus cells in culture from all three patients.
Discussion | Synaptic antibodies and spinal predominant demyelination (SAPD) can be diagnosed with a new serological test and responds to treatment directed at humoral immunity.
38 13:44 | Breakout Session 1 | Immunology Autoimmune encephalitis antibody biomarkers: frequency, age and sex associations
Amy Kunchok1, Vanda Lennon2, Christopher Klein3, 2, Eoin Flanagan3, 2, Divyanshu Dubey3, 2, Anastasia Zekeridou3, 2, Andrew McKeon3, 2, Sean J PIttock3, 2
1. Neurology, Cleveland Clinic, Cleveland, Ohio, United States of America 2. Neuroimmunology Laboratory, Mayo Clinic, Rochester, Minnesota, United States of America 3. Neurology, Mayo Clinic, Rochester, Minnesota, United States of America
Objective | To determine the frequency, age and sex associations for autoimmune encephalitis antibody biomarkers (AE-Abs).
Methods | There were 42032 patients tested in the Mayo Clinic Neuroimmunology Laboratory between January 2018-December 2019 for AE-Abs in serum and/or cerebrospinal fluid (CSF) including; NMDA- R-IgG, AMPA-R-IgG, GABAB-R-IgG, CASPR2-IgG, LGI1-IgG, GAD65-IgG, CRMP5-IgG, amphiphysin-IgG, PCA1/2/Tr-IgGs, ANNA1/2/3-IgGs, GFAP-α-IgG, mGluR1-IgG, DPPX-IgG, MOG-IgG1, were examined to determine frequency of antibody positivity. Age and sex associations were examined using multivariable logistic regression.
Results | Adult serum analysis (22,472 patients; 56% female) revealed 814 (4%) were positive: NMDA- R-IgG (25%) > GAD65-IgG (22%) > LGI1-IgG (21%) > others. Of children (5,649; 50% female), 250 (4%) were positive: NMDA-R-IgG (53%) > MOG-IgG1 (32%) > GAD65-IgG (7%) > others.
Adult CSF analysis (18,745 patients; 54% female) revealed 709 (4%) were positive; NMDA-R-IgG (45%) > GAD65-IgG (19%) > LGI1-IgG (13%) > others. Of children (5136; 50% female), 276 (5%) were positive: NMDA-R-IgG (90%) > GAD65-IgG (7%) > others.
Age <20 years was associated with NMDA-R-IgG and MOG-IgG1 (OR=8.11 and 7.73 respectively, p<0.001). Age >65 years was associated with GABAB-R-IgG, LGI1-IgG, CASPR2-IgG and ANNA1-IgG (OR=7.33, 14.98, 3.67, 14.53, p<0.001). Women accounted for 60% of NMDA-R-IgG (CSF) and 78% of GAD65-IgG (CSF/serum) cohorts (OR=1.32, p=0.002, OR=2.78, p<0.001, respectively). Men accounted for 62% of the LGI1-IgG cohort (OR=1.87, p <0.001). Age and sex interacted for NMDA-R-IgG, particularly in females <20 years (OR=7.72, p <0.001).
Conclusion | The most frequently detected were NMDA-R-IgG, LGI1-IgG, GAD65-IgG and MOG- IgG1. Age and sex associations may suggest paraneoplastic, endocrinological or aging influences on neurological autoimmunity.
13:51 | Breakout Session 1 | Immunology Pregnancy-related relapse in natalizumab, fingolimod and dimethyl fumarate-treated women with multiple sclerosis
Wei Z Yeh1, 2, Putu A Widyastuti1, Anneke Van der Walt1, 2, Jim Stankovich1, Eva K Havrdova3, Dana Horakova3, Karolina Vodehnalova3, Serkan Ozakbas4, Sara Eichau5, Pierre Duquette6, Tomas Kalincik8, 7, Francesco Patti9, Cavit Boz10, Murat Terzi11, Bassem Yamout12, Jeannette Lechner-Scott13, Patrizia Sola14, Olga Skibina2, Michael Barnett15, Marco Onofrj16, Maria J Sá17, Pamela McCombe19, 18, Pierre Grammond20, Radek Ampapa21, Francois Grand’Maison22, Roberto Bergamaschi23, Daniele L.A. Spitaleri24, Vincent Van Pesch25, Elisabetta Cartechini26, Suzanne Hodgkinson27, Aysun Soysal28, Albert Saiz29, Melissa Gresle1, 2, Tomas Uher3, Davide Maimone30, Recai Turkoglu31, Raymond MM Hupperts32, Maria Pia Amato33, 34, Franco Granella35, Celia Oreja-Guevara36, Ayse Altintas37, Richard Macdonell38, Tamara Castillo- Trivino39, Helmut Butzkueven1, 2, Raed Alroughani40, Vilija G Jokubaitis1, 2, MSBase Registry41
1. Department of Neuroscience, Central Clinical School, Monash University, Melbourne, VIC, Australia 2. Multiple Sclerosis and Neuroimmunology Unit, Department of Neurology, Alfred Health, Melbourne, VIC, Australia 3. Department of Neurology and Center of Clinical Neuroscience, First Faculty of Medicine, Charles University and General University Hospital, Prague, Czech Republic 4. Dokuz Eylul University, Turkey 5. Hospital Universitario Virgen Macarena, Spain 6. CHUM – Hopital Notre Dame, Canada
39 7. Melbourne MS Centre, Royal Melbourne Hospital, Melbourne, VIC, Australia 8. CORe, Department of Medicine, University of Melbourne, Melbourne, VIC, Australia 9. Department of Medical and Surgical Sciences and Advanced Technologies; GF Ingrassia, University of Catania – AOU Policlinico-San Marco, University of Catania, Italy 10. KTU Medical Faculty Farabi Hospital, Turkey 11. Mayis University, Medical Faculty, Turkey 12. American University of Beirut, Faculty of Medicine, Nehme and Therese Multiple Sclerosis Center, Beirut, Lebanon 13. John Hunter Hospital, New Lambton Heights, NSW, Australia 14. Neurology Unit, Azienda Ospedaliero-Universitaria of Modena, Modena, Italy 15. Brain and Mind Centre, University of Sydney, Sydney, NSW, Australia 16. Univ G.d’Annunzio Chieti-Pescara, Italy 17. Department of Neurology, São João Universitary Hospital Center, Porto, Portugal 18. Royal Brisbane and Women’s Hospital, Herston, QLD, Australia 19. St Andrews Place, Spring Hill, QLD, Australia 20. Centre de réadaptation déficience physique Chaudière-Appalache, Canada 21. Nemocnice Jihlava, Czech Republic 22. Neuro Rive-Sud, Canada 23. IRCCS Mondino Foundation, Pavia, Italy 24. AORN San Giuseppe Moscati Avellino, Italy 25. Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Belgium 26. Ospedale Generale Provinciale Macerata, Italy 27. Liverpool Hospital, Liverpool, NSW, Australia 28. Bakirkoy Education and Research Hospital for Psychiatric and Neurological Diseases, Turkey 29. Service of Neurology, Hospital Clinic, Institut d’Investigacions Biomediques August Pi i Sunyer (IDIBAPS), and Institut de Neurociències, Universitat de Barcelona, Barcelona, Spain 30. Centro Sclerosi Multipla, UOC Neurologia, ARNAS Garibaldi, Catania, Italy 31. Haydarpasa Numune Training and Research Hospital, Turkey 32. Maaslandziekenhuis, Netherlands 33. Department NEUROFARBA, University of Florence, Italy 34. IRCCS Fondazione Don Carlo Gnocchi, Florence, Italy 35. University of Parma, Italy 36. Department of Neurology, Hospital Clínico San Carlos, Departamento de Medicina, Facultad de Medicina, Universidad Complutense de Madrid (UCM) and IdISSC, Madrid, Spain 37. Department of Neurology, Koc University School of Medicine, Turkey 38. Department of Neurology, Austin Health, Heidelberg, VIC, Australia 39. Department of Neurology, Hospital Universitario Donostia, San Sebastian, Spain 40. Amiri Hospital, Kuwait 41. MSBase Neuro-immunology Registry, Melbourne, VIC, Australia
Objective | To investigate pregnancy-related disease activity in a contemporary multiple sclerosis (MS) cohort.
Methods | Data were obtained from the MSBase Registry. Term/preterm pregnancies conceived from 2011-2019 were included (modern cohort). Annualised relapse rates (ARR) were calculated before, during and after pregnancy. Predictors of intrapartum and early postpartum (1st 3 months) relapse were determined by clustered logistic and Cox regression analyses, respectively.
Results | We included 1640 pregnancies from 1452 women. Disease-modifying therapy (DMT) used in the one-year preconception included natalizumab (n=219), fingolimod (n=147), dimethyl fumarate (DMF; n=57) and low-efficacy therapies (n=845). Preconception ARR by DMT class used before conception were: natalizumab, 0.29 (95% CI 0.22-0.37); fingolimod, 0.37 (0.28-0.49); DMF, 0.24 (0.13- 0.41); low-efficacy, 0.29 (0.25-0.33); and none, 0.24 (0.19-0.31). Among women who used fingolimod or natalizumab, ARR increased during pregnancy. Intrapartum ARR decreased in preconception DMF, low- efficacy or no DMT groups. ARR spiked after delivery across all DMT groups. Natalizumab continuation into pregnancy reduced the odds of relapse during pregnancy (OR 0.76 per month [0.60-0.95], p=0.017). DMT re-initiation with natalizumab protected against postpartum relapse (HR 0.11 [0.04- 0.32], p<0.0001). Breastfeeding women were less likely to relapse (HR 0.61 [0.41-0.91], p=0.016).
Conclusion | Women with MS prescribed natalizumab or fingolimod preconception had higher rates of intrapartum and postpartum relapse. In women considered to be at high relapse risk, use of natalizumab before pregnancy and continued up to 32-34 weeks gestation, with early re-initiation after delivery is an effective option to minimise relapse risks. Strategies of DMT use have to be balanced against potential foetal/neonatal complications.
40 13:58 | Breakout Session 1 | Immunology Psoriasis in multiple sclerosis: an Australian prevalence study
Varitsara Mangkorntongsakul1, Olivia A Charlton2, Kevin Phan3, Ariadna Fontes4, John Parratt1, 4, Geoff Herkes1, 4, Saxon D Smith5, 7, 6
1. The University of Sydney, Sydney, NSW, Australia 2. Department of Dermatology, Royal North Shore Hospital, St Leonards, NSW, Australia 3. Department of Dermatology, Liverpool Hospital, Liverpool, NSW, Australia 4. Department of Neurology, Royal North Shore Hospital, St Leonards, NSW, Australia 5. The Dermatology and Skin Cancer Centre, Gosford, NSW, Australia 6. Sydney Adventist Hospital Clinical School, Sydney Medical School, The University of Sydney, Sydney, NSW, Australia 7. Department of Dermatology, Sydney Adventist Hospital, Wahroonga, NSW, Australia
Background | Multiple Sclerosis (MS) is an immune-mediated, demyelinating disease of the central nervous system(1). Although severe psoriasis and psoriasiform dermatitis have been noted in MS patients, the prevalence of psoriasis in these populations is uncertain and has not been explored in the Australian population.
Objectives | A pilot study to estimate the prevalence of psoriasis in MS cohorts in the Australian population.
Methods | A survey was conducted on 82 MS patients aged 18 and above who attended MS clinics in 2018.
Results | Data was recorded for 82 patients. The mean age was 48 years for the entire cohort and 48.0 years(SD ±11.30)for patients diagnosed with psoriasis. The observed prevalence of psoriasis in MS patients was 12.19% however, the true prevalence is likely to be much higher as various symptoms of psoriasis were reported by a much larger proportion of MS patients. Of the 72 cases without psoriasis, various skin symptoms that were reported were intermittent irritation (for at least 6 months) or erythematous rash (35.4%), seasonal skin changes (39.0%) and thickened scaly skin behind ears and scalps (18.3%). Moreover,18.3% had flaky, peeling or scaly skin while 24.4% experienced dandruff;17.1% reported nail changes, and 13.4% reported a family history of psoriasis. The study also showed that combined psoriasis and eczema was relatively common at 3.7%.
Conclusions | In this pilot study there is a high prevalence of psoriasis in patients with MS suggesting an immunopathological association between the two diseases and indicates that further studies should be done to elucidate common mechanisms,and the nature of this phenotype.
1. Montalban X, Tintore M, Swanton J, Barkhof F, Fazekas F, Filippi M, et al. MRI criteria for MS in patients with clinically isolated syndromes. Neurology. 2010;74(5):427-34.
14:05 | Breakout Session 1 | Immunology Comparison of multiple disease modifying therapies in multiple sclerosis with marginal structural models
Ibrahima Diouf1, Charles B Malpas1,2, Sifat Sharmin1,2, Olga Skibina3,4,5, Katherine Buzzard4, Jeannette Lechner- Scott6,7, Michael Barnett8, Suzanne Hodgkinson9, Mark Slee10, Ernest Butler11, Pamela McCombe12,13, Anneke van der Walt14,4, Helmut Butzkueven15, Steve Vucic16, Richard Macdonell15, Cameron Shaw17, Tomas Kalincik1,2
1. CORe, Department of Medicine, University of Melbourne, Melbourne, VIC, Australia 2. MS Centre, Department of Neurology, Royal Melbourne Hospital, Melbourne, VIC, Australia 3. Department of Neurology, Box Hill Hospital, Melbourne, VIC, Australia 4. Monash University, Melbourne, VIC, Australia 5. The Alfred Hospital, Melbourne, Australia 6. School of Medicine and Public Health, University Newcastle, Newcastle, NSW, Australia 7. Department of Neurology, John Hunter Hospital, Hunter New England Health, Newcastle, NSW, Australia 8. Brain and Mind Centre, Sydney, Australia; 6Liverpool Hospital, Sydney, NSW, Australia 9. Liverpool Hospital, Sydney, NSW, Australia 10. Flinders University, Adelaide, SA, Australia 11. Monash Medical Centre, Melbourne, VIC, Australia 12. University of Queensland, Brisbane, QLD, Australia 13. Royal Brisbane and Women’s Hospital, Brisbane, QLD, Australia
41 14. Department of Neurology, The Alfred Hospital, Melbourne, VIC, Australia 15. Austin Health, Melbourne, VIC, Australia 16. Westmead Hospital, Sydney, NSW, Australia 17. Geelong Hospital, Geelong, VIC, Australia
Background | Because of methodological challenges comparisons of multiple treatments in multiple sclerosis cohorts have been limited to pairwise and triple comparisons.
Objectives | Extend marginal structural models (MSM) to allow simultaneous comparisons of multiple MS treatments.
Methods | We selected patients from the MSBase registry with Clinically Isolated Syndrome and Relapsing-Remitting MS followed for ≥1 year, with ≥3 visits, ≥1 visit per year and exposed to a MS therapy. MSMs were used to compare cumulative hazards of 6-month confirmed worsening and improvement of disability, and the incidence of relapses between treatments. MSMs were continuously re-adjusted for patient age, sex, pregnancy, date from first symptom, prior relapse history and MRI activity. We used MSMs to compare the Average Treatment Effect (ATE), the effect a treatment would have had if the entire study population had been treated with this treatment vs. another treatment. We also estimated the Average Treatment Effect Among the Treated (ATT): comparison an observed effect of a treatment with a counterfactual (not observed) effect of another treatment in the same study population.
Results | Among 23687 patients, we compared ATE of glatiramer acetate (reference), interferon b, natalizumab, fingolimod, dimethyl fumarate, and teriflunomide. In ATE, a reduction of relapse frequency was more prominent on natalizumab, followed by fingolimod (47% and 24% respectively, reference: glatiramer acetate) when compared with the other treatments. The ATT models confirmed these observations.
Conclusions | Compared to other DMTs natalizumab and fingolimod were associated with superior reduction in relapse frequency than glatiramer acetate, interferon beta, teriflunomide and dimethyl fumarate.
Breakout Session 2 | Immunology Chairs: Ruth Leadbetter and Kevin O’Connor
13:30 | Breakout Session 2 | Immunology Immunotherapy responsive neuropathic pain associated with LGI1 and CASPR2 antibodies
Sudarshini Ramanathan2, 1, Alexander Davies2, Christopher Uy2, Mandy Tseng2, Sofija Paneva2, Sophia Michael2, James Varley2, Sophie Binks2, Andreas Themistocleous2, Yaacov Anziska2, Ana Candalija2, Anushka Soni2, Monika Hofer2, Fabienne Brilot1, Russell C Dale1, John Dawes2, Simon Rinaldi2, David Bennett2, Sarosh R Irani2
1. Faculty of Medicine and Health, Sydney Medical School, University of Sydney, Concord, NSW, Australia 2. University of Oxford, Oxford
Objective | We evaluated pain in leucine-rich glioma inactivated1(LGI1) and contactin-associated protein2(CASPR2)-antibody positive(Ab+) patients, to evaluate clinical associations and pathophysiology of treatable pain syndromes.
Methods | 108 LGI-Ab+, 33 CASPR2-Ab+, and 6 LGI1/CASPR-Ab+patients were phenotyped. Pain questionnaires were undertaken to identify neuropathic pain using the Douleur Neuropathique(DN4), patient reported outcome measurement information system(PROMIS), and quality of life(EQ5D). Skin biopsies, and serum binding to cell-based assays, sensory neuronal cocultures, and dorsal root ganglion(DRG) cultures were undertaken.
Results | 39/147 patients described pain, including 17/33 CASPR2-Ab+(52%), 20/108 LGI1-Ab+(19%), and 2 LGI/CASPR2-Ab+patients. Questionnaires completed in 23/39(59%) revealed comparable DN4 scores(p=0.319) with 58% of LGI1-Ab+ and 67% of CASPR2-Ab+patients having neuropathic pain.
42 Patients rated >50% response in 8/30(27%) analgesia trials, versus 20/40(55%) immunotherapy trials(p=0.045). PROMIS ratings were similar between LGI1-Ab+ and CASPR2-Ab+patients at nadir(p=0.662), but showed more improvement following immunotherapy in LGI1-Ab+(p=0.008) than CASPR2-Ab+patients(p=0.125). At follow-up(median 57 months) CASPR2-Ab+patients showed more impairment in mobility(p=0.014), daily activities(p=0.019), and anxiety/depression(p=0.043); and lower overall health(p=0.019) on the EQ5D compared to LGI1-Ab+patients. Intraepidermal nerve fibre density was reduced in 2 LGI1-Ab+ and 1 CASPR2-Ab+patients. Serum immunoglobulin G(IgG) from 6/16 CASPR2-Ab+patients bound to sensory neuronal cocultures compared to 0/14 LGI1- Ab+patients(p=0.019) and 0/12 healthy controls. Serum IgG from 10/16 CASPR2-Ab+patients bound to DRG cultures compared to 1/14 LGI1-Ab+patients(p=0.0024) and 1/12 healthy controls.
Conclusion | Neuropathic pain may be present in both LGI-Ab+ and CASPR2-Ab+patients, and is immunotherapy responsive. Serum IgG from CASPR2-Ab+patients more frequently bound sensory neurons and dorsal root ganglia, suggesting pathophysiological differences which may underlie the more severe pain in these patients.
13:37 | Breakout Session 2 | Immunology Peripheral Monocyte Populations in the Autoimmune Encephalitis Post-acute Phase
Robb Wesselingh1, 2, Sarah Griffith1, 2, James Broadley1, Katherine Buzzard3, 4, David Tarlinton1, Helmut Butzkueven1, 2, Terence J O’Brien1, 2, Mastura Monif1, 2, 3
1. Neurosciences, Monash University, Melbourne, VIC, Australia 2. Neurosciences, Alfred Health, Melbourne, VIC, Australia 3. Neurosciences, Melbourne Health, Melbourne, VIC, Australia 4. Neurosciences, Eastern Health, Melbourne, VIC, Australia
Objectives | Patients with Autoimmune encephalitis (AIE) have a high prevalence of chronic (>3 months post acute illness) morbidity despite appropriate immunotherapy in the acute phase. This post acute phase is often characterized by cognitive impairment, drug-resistant epilepsy and functional impairment. It remains unclear if this morbidity is residual injury from the acute event or driven by ongoing immune activity. We aimed to explore signatures of persistent inflammatory activity in the peripheral circulation of patients with post-acute AIE.
Methods | Patients with previous acute AIE (at least 3 months post symptom onset) were recruited from 4 health networks. We collected 30 mls of blood and separated out monocytes using a negative-selection antibody cocktail with gradient separation method. Monocytes were then stained with fluorochrome tagged antibodies to CD14, CD16, CD11b, CD80 and HLADR and the expression of these cell-surface markers was characterized using flow cytometry.
Results | We recruited 31 patients in the post-acute phase of AIE and 22 healthy controls. On analysis of monocytes there were increased monocyte sub-populations of CD14+CD16+ cells in AIE patients compared with healthy controls (8.74% vs 4.9%, p < 0.05) . This population of monocytes is thought to play an important role in antigen processing and presentation. Expansion of this population may be driving ongoing autoimmunity and requires further characterization.
Conclusion | We have identified perturbations in the normal ratios of monocyte sub-populations in patients in the post-acute phase of AIE that requires further characterization.
13:44 | Breakout Session 2 | Immunology Disease reactivation after cessation of disease-modifying therapy in relapsing-remitting multiple sclerosis
Izanne Roos1, 2, Charles B Malpas1, 2, Emmanuelle Leray3, Katherine Buzzard2, 4, Olga Skibina5, 4, Jeannette Lechner- Scott6, 7, Pamela McCombe8, Mark Slee9, Ernest Butler10, Richard Macdonell11, Anneke van der Walt12, 5, Suzanne Hodgkinson13, Michael Barnett14, Steve Vucic15, Sandra Vukusic16, Helmut Butzkueven12, 5, Tomas Kalincik1, 2
43 1. CORe Unit, Department of Medicine, University of Melbourne, Melbourne, VIC, Australia 2. Department of Neurology, Royal Melbourne Hospital, Melbourne, VIC, Australia 3. Rennes University, Rennes, France 4. Department of Neurology, Box Hill Hospital, Monash University, Melbourne, VIC, Australia 5. Department of Neurology, The Alfred Hospital, Melbourne, VIC, Australia 6. School of Medicine and Public Health, University of Newcastle, Newcastle, NSW, Australia 7. Department of Neurology, John Hunter Hospital, Hunter New England Health, Newcastle, NSW, Australia 8. Department of Neurology, Royal Brisbane and Women’s Hospital, Brisbane, QLD, Australia 9. Flinders University, Adelaide, SA, Australia 10. Monash Medical Centre, Melbourne, VIC, Australia 11. Department of Neurology, Austin Health, Melbourne, VIC, Australia 12. Department of Neuroscience, Central Clinical School, Monash University, Melbourne, VIC, Australia 13. Liverpool Hospital, Sydney, NSW, Australia 14. Brain and Mind Centre, Sydney, NSW, Australia 15. Westmead Hospital, Sydney, NSW, Australia 16. Hospital Neurologique Pierre Wertheimer, Lyon, France
Objectives | To evaluate the rate of return of disease activity after cessation of multiple sclerosis (MS) disease-modifying therapy.
Methods | This was a retrospective cohort study from two large observational MS registries: MSBase and OFSEP. Patients with relapsing-remitting MS who had ceased a disease-modifying therapy and were followed up for the subsequent 12-months were included in the analysis. The primary study outcome was annualised relapse rate in the 12 months after disease-modifying therapy discontinuation stratified by patients who did, and did not, commence a subsequent therapy. The secondary endpoint was the predictors of first relapse and disability accumulation after treatment discontinuation.
Results | 18 029 eligible treatment discontinuation epochs were identified for seven therapies. Rates of relapse started to increase 2-months after natalizumab cessation. Commencement of a subsequent therapy within 2-4 months reduced the magnitude of disease reactivation. After discontinuation of fingolimod, rates of relapse increased overall, and stabilised faster in patients who started a new therapy within 1-2 months. Magnitude of disease reactivation for other therapies was low, but reduced further by commencement of another treatment 1-10 months after treatment discontinuation. Predictors of relapse were higher relapse rate in the year before cessation, female sex, younger age and higher EDSS. Commencement of a subsequent therapy reduced both the risk of relapse (HR 0.76, CI 0.72-0.81) and disability accumulation (0.73, 0.65-0.80).
Conclusion | Understanding the rate of disease reactivation after discontinuing different MS immunotherapies will help guide optimal wash-out times for therapeutic agents during treatment sequencing.
13:51 | Breakout Session 2 | Immunology Predicting Infection Risk in Multiple Sclerosis Patients Treated with Ocrelizumab: A Retrospective Cohort Study
Nabil Seery1, Sifat Sharmin2, 3, Vivien Li4, 1, Ai-Lan Nguyen2, 3, 1, Claire Meaton1, Roberts Atvars1, Nicola Taylor5, Kelsey Tunnell5, Joh Carey5, Mark P Marriott1, 6, Katherine A Buzzard1, 6, Izanne Ross2, 3, 1, Chris Dwyer4, 1, Josephine Baker1, Lisa Taylor1, Kymble Springs2, 7, Trevor J Kilpatrick4, 1, Tomas Kalincik2, 3, 1, Mastura Monif1, 8, 9
1. Melbourne MS Centre, Department of Neurology, The Royal Melbourne Hospital, Parkville, VIC, Australia 2. Department of Medicine, The University of Melbourne, Melbourne, VIC, Australia 3. Clinical Outcomes Research Unit, The University of Melbourne, Melbourne, VIC, Australia 4. Florey Institute of Neurosciences and Mental Health, The University of Melbourne, Parkville, VIC, Australia 5. Day Medical Centre, Royal Melbourne Hospital, Parkville, Melbourne, VIC, Australia 6. Department of Neuroscience, Eastern Health Clinical School, Box Hill, VIC, Australia 7. Department of Immunology, The Royal Melbourne Hospital, Melbourne, VIC, Australia 8. MS and Neuroimmunology Department, Alfred Health, Melbourne, VIC, Australia 9. Department of Neuroscience, Monash University, Melbourne, VIC, Australia
Objective | To examine factors determining risk of self-reported infections and antimicrobial use in patients receiving Ocrelizumab for MS.
44 Methods | Retrospective, observational cohort study conducted in Ocrelizumab-treated patients at the Royal Melbourne Hospital. The association of clinical and laboratory factors with self-reported infection rate and antimicrobial use were estimated using univariate and multivariable logistic regression models.
Results | 185 patients were included in the study, and 176 infections were reported in 89 patients (46.1%), and in 47 patients (25.3%) antimicrobial use was identified. In univariate analyses, a higher serum IgA was associated with reduced odds of infection (OR 0.44, 95% CI 0.25 – 0.76). In multivariable analyses, older age (OR 0.94, 95% CI 0.88 – 0.99), higher serum IgA (OR 0.37, 95% CI 0.17 – 0.80) and higher serum IgG (OR 0.81, 95% CI 0.67 – 0.99) were associated with reduced odds of infection. Older age (OR 0.85, 95% CI 0.75 – 0.96) and higher serum IgA (OR 0.23, 95% CI 0.07 – 0.79) were associated with reduced odds of antimicrobial use, whilst longer MS disease duration (OR 1.22, 95% CI 1.06 – 1.41) and higher EDSS (OR 1.99, 95% CI 1.02 – 3.86) were associated with increased odds of antimicrobial use.
Conclusions | Higher serum IgA, IgG and older age were associated with reduced odds of infection. Our findings highlight non-uniformity of infection risk in Ocrelizumab-treated MS patients, and substantiate the need to monitor immunoglobulin levels pre-treatment and whilst on therapy.
13:58 | Breakout Session 2 | Immunology Real-world experience with Ocrelizumab in the MSBase Registry – Australian RRMS cohort
Anneke Van der Walt1, Helmut Butzkueven1, 2, Tim Spelman2, Tomas Kalincik3, Katherine Buzzard4, Jeanette Lechner- Scott5, Suzanne Hodgkinson6, Ernest Butler7, Richard Macdonell8, Mark Slee9, MSBase Study Group2, Bruno Marcel10
1. Monash University, Melbourne, VIC, Australia 2. MSBase Foundation, Melbourne, VIC, Australia 3. University of Melbourne, Melbourne, VIC, Australia 4. Box Hill Hospital, Box Hill, VIC, Australia 5. University of Newcastle, Newcastle, NSW, Australia 6. Liverpool Hospital, Sydney, NSW, Australia 7. Monash Medical Centre, Melbourne, VIC, Australia 8. Austin Health, Melbourne, VIC, Australia 9. Flinders University, Adelaide, SA, Australia 10. Roche Products Pty Ltd, Sydney, NSW, Australia
Introduction | Ocrelizumab (OCR) is a humanised anti-CD20+ monoclonal antibody for the treatment of Multiple Sclerosis.
Objectives | In Australian MSBase clinics, we describe baseline characteristics of relapsing-remitting MS (RRMS) patients treated with OCR, treatment pathways and early clinical outcomes.
Methods | Secondary analysis using MSBase Registry data for RRMS patients with OCR initiation within 3 months of MSBase recorded visit. Descriptive statistics included demographics, disease course/duration, prior disease modifying therapies (DMT) and EDSS. Relapse data was described in patients with ≥6 months follow-up.
Results | As of 4 June 2020, MSBase included 624 eligible Australian RRMS patients newly treated with OCR. Median age at OCR initiation was 42.5 years. OCR was first line therapy in 18.9% of patients. Most frequent DMT’s in the 12 months prior to OCR were natalizumab (32.1%) and fingolimod (24.8%). Of 434 RRMS patients with ≥6 months follow-up, 392 remained relapse free (90.3%; 95% CI 81.6, 99.7) over a mean OCR exposure of 1.35 years. In this group, the annualized relapse rate (ARR) was 0.10 (95% CI 0.08-0.13), compared to an ARR of 0.83 in the 24 months pre-OCR start. Treatment discontinuation was recorded for 20 of these 434 patients In the overall RRMS cohort, treatment persistence at 12 and 24 months was 94.3% (95%CI: 90.9%-96.1%%) and 88.7% (95%CI 77.2%-94.0%), respectively.
Conclusion | Almost 20% of RRMS patients treated with OCR in Australian MSBase centres received OCR as a first line treatment. During OCR treatment, relapses and OCR discontinuations were rare.
45 14:05 | Breakout Session 2 | Immunology Worsening longitudinal reaction time trajectories using the MSReactor computerised battery predicts confirmed EDSS progression
Daniel Merlo1, Jim Stankovich1, Claire Bai2, Tomas Kalincik2, Melissa Gresle1, Jeannette Lechner-Scott3, Trevor Kilpatrick4, Michael Barnett5, Bruce Taylor6, David Darby4, Helmut Butzkueven1, Anneke van der Walt1
1. MSNI, Central Clinical School, Monash University, Melbourne, VIC, Australia 2. CORe, Department of Medicine at RMH, University of Melbourne, Melbourne, VIC, Australia 3. Department of Neurology, John Hunter Hospital, Newcastle, NSW, Australia 4. Florey Institute of Neuroscience and Mental Health, Melbourne, VIC, Australia 5. Brain and Mind Centre, Sydney, NSW, Australia 6. Department of Neurology, Royal Hobart Hospital, Hobart, TAS, Australia
Objectives | To identify and validate longitudinal reaction time trajectories in relapsing remitting multiple sclerosis using a computerised cognitive battery and latent class mixed modelling, and to assess the association between reaction time trajectories and disability progression.
Methods | Participants serially completed web-based computerised reaction time tasks measuring psychomotor speed, visual attention and working memory. Testing sessions were completed 6-monthly with the option of additional home based testing. Participants who completed at least three testing sessions over a minimum of 180 days were included in the analysis. Longitudinal reaction times were modelled using Latent Class Mixed Models to group individuals sharing similar latent characteristics. Models were tested for consistency using a cross-validation approach. Inter-class differences in the probability of reaction time worsening and the probability of 6-month confirmed disability progression were assessed using survival analysis.
Results | A total of 460 relapsing remitting multiple sclerosis patients were included. For each task of the MSReactor computerised cognitive battery, the optimal model comprised of 3 latent classes. All tasks could identify a group with high probability of reaction time slowing. The visual attention and working memory tasks could identify a group of participants who were 3.7 and 2.6 times more likely to experience a 6-month confirmed disability progression, respectively. Participants could be classified into predicted cognitive trajectories after just 5 tests with between 64% and 89% accuracy.
Conclusion | Latent class modelling of longitudinal cognitive data collected by the MSReactor battery identified a group of patients with worsening reaction times and increased risk of disability progression.
Breakout Session 3 | Neurodegeneration Chairs: Carly Oboudiyat and Campbell Le Heron
13:30 | Breakout Session 3 | Neurodegeneration Volumetric and connectivity profile of regional thalamic abnormality in amyotrophic lateral sclerosis
Sicong Tu1, Marion Sourty2, Fernando Calamante1, Manoj Saranathan3, Ricarda Menke4, Kevin Talbot4, Matthew Kiernan1, Martin Turner4
1. Brain and Mind Centre, University of Sydney, Sydney, NSW, Australia 2. Université de Strasbourg, Strasbourg, France 3. University of Arizona, Tucson, Arizona, United States 4. Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK
Neurodegeneration in ALS follows a diffuse pattern of cortical involvement1. We have previously highlighted that thalamic abnormality is a robust disease signature in ALS2, but the integrity of thalamic nuclei and their clinical association remains unclear. We employed a novel segmentation technique for thalamic nuclei and track-weighted functional connectivity (TW-sFC) to characterize volumetric and connectivity profiles of regional thalamic abnormality.
46 Methods | Forty ALS patients and 27 age-and-education matched controls were recruited. All patients underwent comprehensive clinical examination and 3T MRI scan (T1; DWI; rs-fMRI). Thalamic nuclei were robustly segmented from T1 images using the THOMAS pipeline3. Whole-brain white matter fibre tracking was performed using MRtrix and combined with resting-state fMRI to generate combined structural and functional connectivity maps (TW-sFC)4.
Results | Reduced thalamus volume was observed bilaterally in ALS compared to control (p values < 0.036). Bilateral volumetric reduction was consistently observed across all regions except for the anterior thalamus in ALS (p values < 0.05). Significant increased TW-sFC was observed in ALS in the right anterior thalamus (p =0.03) and right anterior ventral nuclei (p < 0.01). TW-sFC of the mediodorsal nuclei correlated with disease duration (p < 0.02) and disease progression rate (p < 0.03).
Conclusions | Regional thalamic abnormalities are present in ALS and hold a significant association with clinical features. Variability in thalamic connectivity demonstrated significant clinical associations with disease duration, progression rate, and upper motor dysfunction. The findings reinforce that diffusion and functional MR imaging modalities are promising markers of disease burden in ALS.
1. Brettschneider, J., Del Tredici, K., Toledo, J., et al. (2013). Stages of pTDP-43 pathology in amyotrophic lateral sclerosis. Ann Neurol, 74, 20-38. 2. Tu, S., Menke, R., Talbot, K., Kiernan, M., Turner, M. (2018). Regional thalamic MRI as a marker of widespread cortical pathology and progressive frontotemporal involvement in amyotrophic lateral sclerosis. JNNP, 89, 1250- 1258. 3. Su, J., Thomas, F., Kaso, W., et al. (2019). Thalamus Optimized Multi Atlas Segmentation (THOMAS): fast, fully automated segmentation of thalamic nuclei from structural MRI. Neuroimage, 194, 272-282. 4. Calamante, F., Smith, R., Liang, X., Zalesky, A., Connelly, A. (2017). Track-weighted dynamic functional connectivity (TW-dFC): a new method to study time-resolved functional connectivity. Brain Struct Funct, 222, 3761-3774.
13:37 | Breakout Session 3 | Neurodegeneration Progression of clinical features in Lewy body dementia can be detected over six months
Elie Matar1, 2, Simon R White3, John P Taylor4, Alan Thomas5, Ian G McKeith4, Joseph PM Kane6, A J Surendranathan2, Glenda M Halliday1, Simon JG Lewis1, John T O’Brien2
1. Brain and Mind Centre, University of Sydney, Camperdown, NSW, Australia 2. Department of Psychiatry, University of Cambridge, Cambridge, Cambridgeshire, United Kingdom 3. MRC Biostatistics Unit, University of Cambridge, Cambridge, United Kingdom. 4. Newcastle Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, UK 5. Campus for Ageing and Vitality, Newcastle University, Newcastle, United Kingdom 6. Centre for Public Health, Queen’s University Belfast, Belfast, United Kingdom
Objective | This study aimed to quantify the trajectory and magnitude of change of the key clinical features and corresponding symptom domains of Dementia with Lewy bodies (DLB) and Parkinson’s disease dementia (PDD) including global cognition, parkinsonism, recurrent visual hallucinations, cognitive fluctuations and sleep disturbance.
Methods | 116 patients with Lewy body dementia (DLB=72, PDD=44) underwent assessment at baseline, 3 and 6 months as part of a prospective multi-centre randomized control trial. Linear mixed models were constructed for core outcome measures using the Mini-Mental State Examination (MMSE), motor section of the Unified Parkinson’s disease rating scale (UPDRS-III), Dementia Cognitive Fluctuations Scale (DCFS) and the Neuropsychiatric Inventory (NPI).
Results | Within the timeframe of our study (6 months) we were able to identify a significant cognitive decline of 1.3 points on the MMSE (P=0.002) and significant worsening of motor parkinsonism with an increase in UPDRS-III score of 3.2 points (P=0.018). Fluctuation severity also increased using the DCFS with a 6 month change in score of 1.3 points (P=0.001). Uniquely, a signal for increased severity of sleep symptoms of 1.2 points (NPI-sleep) was also detectable (P=0.04). Significant changes in neuropsychiatric symptoms were not detected. There was no difference in rates of change of scores between DLB and PDD.
47 Conclusion | Clinically significant rates of change in core clinical features can be detected and quantified in Lewy body dementia over a relatively short period (6 months) using common clinical instruments, and thus may be useful as clinical endpoints for therapeutic trials of disease modifying and symptomatic agents.
13:44 | Breakout Session 3 | Neurodegeneration Visualising the topographic pattern of tau deposition in patients with Progressive Supranuclear Palsy using PI2620-PET
Lucy Vivash1, 2, Huiliang Tang2, Kelly Bertram1, 2, Terence J O’Brien1, 2
1. Monash University, Melbourne, VIC, Australia 2. Alfred Hospital, Melbourne, VIC, Australia
Objectives | The accumulation of tau in the basal ganglia and later cerebellum and frontal cortex is a key pathogenic mechanism in Progressive Supranuclear Palsy (PSP). The ability to detect tau in the living brain has the potential to revolutionise the diagnosis of PSP and other tauopathies, and to monitor the effectiveness of therapeutic interventions targeting tau-based mechanisms. This study investigates the use of a new tau-specific PET radiotracer, PI-2620, as a tool for visualising tau in the living brain.
Methods | Ten patients with PSP (age 62-75 years, 6 male) underwent a 60 minute dynamic PET scan with PI2620 (185MBq). The dynamic PET data was processed in to a single parametric image of binding potential (BP) using the simplified reference tissue model and the corpus callosum as the reference region.
Results | Visual inspection of the images showed clear uptake in the basal ganglia nuclei. BP was highest in pallidum (1.99), putamen (1.67), thalamus (1.63), substantia nigra (1.62), cerebellum (1.6) and caudate (1.55), compared to 1.2-1.5 across the cortical regions. Higher BP in the basal ganglia nuclei were associated with higher scores on the PSPRS (putamen, r=0.77, p=0.01).
Conclusion | PI2620-PET shows promising potential as a technique for specifically imaging and quantitating the topographic pattern of tau distribution in patients with PSP. Further studies are needed to evaluate its use as a diagnostic and treatment monitoring tool for PSP and other tauopathies.
13:51 | Breakout Session 3 | Neurodegeneration Gut microbiota and nutritional profiles of Parkinson’s disease patients
Michal Lubomski1, 2, Xiangnan Xu3, Andrew J Holmes4, Samuel Mueller5, Jean Yang3, Carolyn M Sue1, 2, Ryan L Davis2
1. Neurology Department, Royal North Shore Hospital, St Leonards, NSW, Australia 2. Department of Neurogenetics, Kolling Institute, University of Sydney and Royal North Shore Hospital, Sydney, NSW, Australia 3. School of Mathematics and Statistics. Sydney Precision Bioinformatics, University of Sydney, Camperdown, NSW, Australia 4. School of Life and Environmental Sciences, The Charles Perkins Centre, University of Sydney, Sydney, NSW, Australia 5. School of Mathematics and Statistics. Sydney Precision Bioinformatics., University of Sydney, Camperdown, NSW, Australia
Objectives | Imbalances in the composition of the gut microbiome (GM) from Parkinson’s disease (PD) patients have been reported previously. Collectively, the limited literature indicates a reduction in short-chain fatty-acid–producing bacteria that negatively influence colonic permeability and inflammation. We investigated GM composition in association with various clinical features and nutritional data in a large cross-sectional Australian PD cohort, to determine whether short-chain fatty-acid–producing bacteria representation in the GM was altered in association with clinical or nutritional differences between PD patients and controls.
Methods | Clinical outcome measures derived from PD-validated questionnaires and stool samples were collected from 103 PD patients and 81 spousal healthy controls (HCs). GM composition,
48 determined from 16S amplicon sequencing of the V3-V4 region of stool bacterial DNA, was compared between groups and with clinical outcome measures.
Results | We identified significant compositional differences in the GM profiles of PD patients compared to HCs, across order, family and genus taxonomic levels. Multiple taxa were associated with a variety of clinical PD characteristics. Predictive models using GM profiles were developed to identify PD and were improved by incorporating nutritional data.
Conclusions | We identified notable differences in microbial diversity and GM composition in PD patients compared to HCs that, along with nutritional data, enabled the development of predictive modelling to identify PD. These findings further support the GM as a potentially useful biomarker of PD pathophysiology.
13:58 | Breakout Session 3 | Neurodegeneration Genetic epidemiology of Motor Neuron Disease: Royal Brisbane and Women’s Hospital Cohort
Po Sheng Yang1, Robert Henderson1, Pamela McCombe1
1. Neurology, Royal Brisbane and Women’s Hospital, Brisbane, QLD, Australia
Introduction | Motor neuron disease (MND) can be sporadic or familial, with approximately 10% of patients being familial. A number of genetic variants have been found in patients with or without family history. We present the results of genetic testing of a cohort of MND patients diagnosed at Royal Brisbane and Women’s Hospital (RBWH).
Methods | The criteria for genetic testing for our cohort were consenting MND patients with or without family history. Patients were tested for C9ORF72 repeat expansions and also with a panel of MND genes. The frequency of genetic variants and their clinical features were analysed and compared.
Results | There were 47 patients with a genetic variant. The most common variants were in C9ORF72 (27 patients; 57%), SOD1 (12 patients; 26%) and TARDBP (4 patients; 9%). Other genes with variants were TBK1, SPAST, NEK1 and HSPB1 (one patient for each gene). The median age of onset was 50, 49 and 58 years for C9ORF72, SOD1 and TARDBP, respectively. Survival was 813, 850 and 1889 days for C9ORF72, SOD1 and TARDBP, respectively. The frequency of bulbar onset was 26%; 8% and 50% for C9ORF72, SOD1 and TARDBP, respectively. A family history of MND was present in 63%, 83% and 50% of C9ORF72, SOD1 and TARDP patients, respectively.
Conclusion | The frequencies of genetic variants in our cohort are similar to what is described in European populations. Identification of genetic variants will aid the understanding of the associated phenotypes and identifying patients for possible therapy.
14:05 | Breakout Session 3 | Neurodegeneration Neurophobia: A study of Australian medical students and junior doctors
Alex Yeung1, Leila Karimi2, Tissa Wijeratne1
1. Western Health, Melbourne, VIC, Australia 2. La Trobe University, Melbourne, VIC, Australia
Objectives | Neurological disorders are the leading cause disability in Australia and the world. Combating the perceived difficulty of neurology or “neurophobia” and improving physician education is a key component in addressing this problem. We aim to conduct the first study to identify whether neurophobia exists in medical students and junior doctors in an Australian population and try to identify factors that may contribute to this in this population.
49 Methods | A 24 question online validated survey was distributed via email broadcast to all medical students and junior doctors at a metropolitan tertiary care centre in Australia. Responses were collected over 6 weeks with weekly reminder emails for 4 weeks after the initial invitation email.
Results | 114 medical students and junior doctors participated in the study. Participants perceived neurology as the most difficult medical speciality compared to 10 other medical specialties (p=0.001). The top three factors contributing to this perceived difficulty were: a lack of understanding of neuroanatomy, lack of diagnostic certainty and lack of clinical exposure. 65% of the participants stated that they had too little planned teaching in neurology with only 36% of the participants having performed a neurology rotation during medical school.
Conclusion | The prevalence of neurophobia in this Australian cohort of medical students and junior doctors is consistent with previous findings from around the world. This concerning finding requires further examination into the contributing factors in order to created trials of targeted interventions in order to resolve this.
Breakout Session 4 | Epilepsy and Miscellaneous Chairs: Zebunessa Rahman and Andrew Neal
13:30 | Breakout Session 4 | Epilepsy and Miscellaneous Optimising selection for epilepsy surgery
Anthony Khoo2, 1, Jane de Tisi2, 1, Shahidul Mannan1, Aidan O’Keeffe3, Josemir W Sander2, 1, John S Duncan2, 1
1. National Hospital for Neurology & Neurosurgery, Queen Square, London, UK 2. Department of Clinical & Experimental Epilepsy, UCL Queen Square Institute of Neurology, London, UK 3. Department of Statistical Science, UCL, London, UK
Objective | To determine reasons for adult patients with drug-resistant focal epilepsy who undergo presurgical evaluation not proceeding with surgery and identify factors that influence this decision.
Methods | We analyzed demographic, imaging and electroclinical data on 617 consecutive patients brought to the Queen Square presurgical epilepsy MDT between January 2015 and December 2019. Multivariable logistic regression was performed to identify predictors of not proceeding with surgery, using comparative data from a prospectively-followed cohort of individuals who had epilepsy surgery at the same centre over an identical 5-year period.
Results | A definitive decision not to proceed with surgery was made in 315 (51%) cases. Common reasons behind this were an inability to localise the epileptogenic zone (n=104), multifocal epilepsy (n=74) and patients’ decisions not to proceed with intracranial EEG (n=50) or surgery (n=39). Learning disability (OR: 2.35; 95% CI 1.07-5.16), normal MRI (OR: 6.68; 95% CI 3.71-12.05), extratemporal epilepsy (OR: 2.93; 95% CI 1.82-4.71) and bilateral seizure onset zones (OR 3.05; 95% CI 1.41-6.61) were independent predictors of not having surgery. Probability of having surgery in those with normal MRI and extratemporal epilepsy was <10%. Those who did not proceed to surgery resided in more deprived socio-economic areas (median deprivation decile 40-50% vs 50-60%, p<0.05).
Conclusions | Although underutilized, epilepsy surgery is only appropriate for selected individuals with drug-resistant focal epilepsy. A predictive model based on demographic, imaging and electroclinical data can help determine those unlikely to be suitable for surgery and aid the decision to refer for more extensive or invasive evaluation.
13:37 | Breakout Session 4 | Epilepsy and Miscellaneous Differentiating status epilepticus from prolonged psychogenic non- epileptic seizures – can peripheral cell ratios help?
Tracie HL Tan1, 2, 3, Piero Perucca1, 2, 3, 4, 5, Patrick Kwan1, 2, 5, Terence J O’Brien1, 2, 3, 5, Mastura Monif1, 2, 3
50 1. Department of Neuroscience, Central Clinical School, Faculty of Medicine, Nursing and Health Science, Monash University, Melbourne, VIC, Australia 2. Department of Neurology, Alfred Hospital, Melbourne, VIC, Australia 3. Department of Neurology, Royal Melbourne Hospital, Melbourne, VIC, Australia 4. Comprehensive Epilepsy Program, Austin Hospital, Heidelberg, VIC, Australia 5. Department of Medicine, The University of Melbourne, Melbourne, VIC, Australia
Objectives | Differentiating status epilepticus (SE) from prolonged episodes of psychogenic non- epileptic seizures (PNES) is not always straight forward and treatment paradigms are dichotomous with potential for harm from misdiagnosis. This study aims to identify the utility of the neutrophil- to-lymphocyte ratio (NLR), neutrophil-to-monocyte ratio (NMR), monocyte-to-lymphocyte ratio (MLR) and platelet-to-lymphocyte ratio (PLR) in differentiating between SE and PNES.
Methods | Retrospective case control study in adults presenting to a tertiary hospital between April 2017 and December 2020. SE was defined as per ILAE criteria for time point 1. PNES events needed to meet the same time criterion. Patients were excluded if they had other factors that altered peripheral cell counts. After screening 1052 cases, 69 SE events from 56 patients and 38 prolonged PNES events from 22 patients were analysed.
Results | NLR, NMR, MLR and PLR were all significantly higher in SE compared with PNES with mean values of 6.91 vs 2.09, 11.03 vs 7.94, 0.59 vs 0.27 and 189.7 vs 102.3, respectively. Using receiver operating curves, cut off values for NLR, NMR, MLR and PLR of >3.175, >9.36, >0.435 and >129.5 respectively were identified, yielding sensitivities and specificities of 60.87% and 89.47%, 53.62% and 78.95%, 56.5% and 94.7%, 59.42% and 86.84% respectively. AUC ranged from 0.689 to 0.7931.
Conclusion | Patients with SE had significantly higher peripheral cell ratios than those with PNES. When the diagnosis is in doubt, elevated cell ratios can be used to increase diagnostic certainty. However, where cell ratios are not elevated, further investigations are required.
13:44 | Breakout Session 4 | Epilepsy and Miscellaneous Real-time capture of patient-reported outcomes using a digital platform – a pilot study
Wendyl D’Souza1, Nicholas Lawn2, Michelle Kiley3, Joe Frasca4, Emma Whitham5, Udaya Seneviratne6, Lisa Gillinder7, Patrick Carney8, Farhad Goodarzy9, Andi Partovi9
1. Medicine, St Vincent’s Hospital, The University of Melbourne, Fitzroy/Melbourne, VIC, Australia 2. Western Australian Adult Epilepsy Service, Sir Charles Gairdner Hospital,Perth, Australia, WA, Australia 3. Department of Neurology, Royal Adelaide Hospital, Adelaide, SA, Australia 4. Department of Neurology, Flinders Medical Centre, Adelaide, SA, Australia 5. Department of Neurology, Flinders Medical Centre, Adelaide, SA, Australia 6. Department of Neurology, Monash Medical Centre, Melbourne, Australia, Monash Medical Centre, Melbourne, VIC, Australia 7. Neurology, Mater Hospital, Brisbane, QLD, Australia 8. Neurology, Box Hill Hospital, Melbourne, VIC, Australia 9. Keylead Health, Melbourne, VIC, Australia
Objectives | To demonstrate proof of concept/feasibility of a novel digital platform using real-time capture of patient-reported outcomes for real-world research involving patients with epilepsy.
Methods | The phone app collects multi-faceted patient-reported outcomes including seizure frequency, medication side effects, mood, anxiety, quality of life and cognition along with voice and digital images. Patients are invited through a national consortium of 18 adult epilepsy centres in Australia. The patient-reported information potentially allows feedback to their treating specialists and tertiary centre in near real-time, along with deidentified aggregation across all participating centres for comparison. Currently, more than 40 patients are enrolled. We present the outcomes of one patient, with the longest-running data points. The new platform was developed by KeyLead Health TM, Melbourne Australia.
Results | The results report a single patient’s composite scores for mood, sleep, cognition seizures and medication side-effects from the first 1.5 months.
51 Conclusions | Our digital phone platform has the potential to facilitate the more effective and efficient capture of longitudinal data enhancing real-world research data integrity along with patient and specialist engagement.
Table: Patient reported outcomes for single patient captured using digital phone app
Date 4/1 7/1 10/1 15/1 20/1 1/2 15/2 18/2 Side effects 4.5 3.8 3.4 3.4 3.4 3.4 3.3 3.8 Memory 9 1 7 5 5 6 7 6 Seizures 3 0 0 1 1 1 2 0 Reaction time 7.7 7.1 12.4 7.0 7.7 7.7 7.9 6.7 Mood 17 16 15 15 12 15 18 16 Sleep 12 6 8 4 3 10 8 5
13:51 | Breakout Session 4 | Epilepsy and Miscellaneous Relationships between Cognitive Impairment and Clinical Features of Idiopathic Intracranial Hypertension
Wendy Wang1, Meaghan Clough2, Owen White1, Neil Shuey3, Anneke Van der Walt1, Joanne Fielding2
1. Alfred Health, Melbourne, VIC, Australia 2. Monash University, Melbourne, VIC, Australia 3. Royal Victorian Eye and Ear Hospital, Melbourne, VIC, Australia
Objective | To determine whether cognitive impairments in patients with Idiopathic Intracranial Hypertension (IIH) are correlated with changes in visual processing, weight, waist circumference, mood or headache and whether they change over time.
Methods | Twenty-two newly diagnosed IIH patients participated, with a subset assessed longitudinally at 3 and 6 months. Both conventional and novel ocular motor tests of cognition were included: Symbol Digit Modalities Test (SDMT), Stroop Colour and Word Test (SCWT), Digit Span, California Verbal Learning Test (CVLT), prosaccade (PS) task, antisaccade (AS) task, interleaved antisaccade-prosaccade (AS-PS) task. Patients also completed headache, mood and visual functioning questionnaires.
Results | IIH patients performed more poorly than controls on the SDMT (p<.001), SCWT (p=.021), Digit Span test (p<0.001) and CVLT (p=.004) at baseline, and generated a higher proportion of AS errors in both the AS (p<.001) and AS-PS tasks (p=.007). Further, IIH patients exhibited prolonged latencies on the cognitively complex AS-PS task (p=.034). While weight, waist circumference, headache and mood did not predict performance on any experimental measure, increased retinal nerve fibre layer (RNFL) was associated with AS error rate on both the block (F(3, 19)=3.22, B=0.30, p=0.022) and AS-PS task (F(3, 20)=2.65, B=0.363, p=0.013). Unlike ocular motor changes, impairments revealed on conventional tests of cognition persisted up to 6 months.
Conclusions | We found multi-domain cognitive impairments in IIH patients that were unrelated to clinical features. Marked ocular motor inhibitory control deficits were predicted by RNFL thickness but remained distinct from other cognitive changes, underscoring the significance of visual processing changes in IIH.
13:58 | Breakout Session 4 | Epilepsy and Miscellaneous Nitrous oxide induced myelo-neuropathy
Grace Swart1, Christopher Blair2, Zhong X Lu3, Solomon Yogendran4, Joanna Offord5, Emily Sutherland6, Stephanie Barnes7, Natalie Palavra6, Phillip Cremer6, Samuel Bolitho8, Gabor M Halmagyi1
1. Royal Prince Alfred Hospital, Camperdown, NSW, Australia 2. Liverpool Hospital, Sydney, NSW, Australia
52 3. Medicine, Department of Medicine, Monash University, Melbourne, VIC, Australia 4. Anaesthesia, Prince of Wales Hospital, Sydney, NSW, Australia 5. Neurology, Prince of Wales Hospital, Sydney, NSW, Australia 6. Royal North Shore Hospital, Sydney, NSW, Australia 7. Neurology, Concord Repatriation Hospital, Sydney, NSW, Australia 8. St Vincents Hospital, Sydney, NSW, Australia
Background | Nitrous oxide misuse is a recognized issue worldwide. It is cheap, legal and can be bought in bulk online. Prolonged misuse inactivates vitamin B12 causing a myelo-neuropathy.
Methods | Review of 20 patients with nitrous-oxide induced myelo-neuropathy from tertiary hospitals between 2016-2020
Results | Twenty patients had an average age of 25 years. Mean canister consumption was 150 per day for 9 months. At presentation paraesthesia and gait unsteadiness were common, and six patients were bedbound. Mean serum B12 was normal: 258 pmol/L(NR=140-750) as was active B12: 94 pmol/L(N>35). In contrast mean serum homocysteine was high: 51 umol/L(NR=5-15). Spinal MRI(n=19) showed characteristic dorsal column T2 hyperintensities. Nerve conduction studies(n=5) showed a lower limb predominant axonal sensorimotor neuropathy. Patients were treated with intramuscular vitamin B12, with variable functional recovery at discharge. Three of 6 patients who were bedbound at presentation were able to walk with an aid at discharge. Of 8 patients with follow-up, most had persistent paraesthesiae and/or sensory ataxia. Admission and discharge mobility scores were not significantly correlated with serum total and active B12 levels or cumulative nitrous oxide use. However, there was an inverse trend for decreased serum active B12 level with increased cumulative nitrous oxide use (Spearman’s rho -0.416, p=0.09).
Conclusion:Nitrous oxide misuse can cause severe but potentially reversible subacute myelo- neuropathy. Serum and active B12 can be normal, while elevated homocysteine and dorsal column high T2 signal on MRI imaging strongly support the diagnosis. Neurological deficits can improve with abstinence and B12 replacement, even in the most severely affected patients.
14:05 | Breakout Session 4 | Epilepsy and Miscellaneous Development of a bedside motion capture system: a pilot study
Paul Kopanidis1, Joe Lynch2, Md Asikuzzaman 3, Mark Pickering 3, Diana Perriman2, Wayne Spratford4, Christian Lueck2, 1
1. Neurology, The Canberra Hospital, Garran, ACT, Australia 2. Medical School, Australian National University, Canberra, ACT, Australia 3. School of Engineering and Information Technology, The University of New South Wales, Canberra, ACT, Australia 4. Research Institute for Sport and Exercise, University of Canberra, Canberra, ACT, Australia
Objectives | Pronator drift is one of many clinical signs that would benefit from detailed study, but this requires accurate measurement of movement in three dimensions. The Vicon system is currently considered to be the gold standard for measurement of limb kinetics in a movement analysis lab but it cannot be used at the bedside for many reasons. This study aimed to investigate a portable camera-based motion capture system (MCS) as a clinically-useful alternative.
Methods | The MCS used two commercially-available cameras arranged so as to permit stereoscopic calculation of depth (i.e. distance from the cameras), and therefore a 3-D representation of movement at the shoulder, elbow and wrist. Data were obtained simultaneously from both movement capture and Vicon systems while three normal subjects simulated four scenarios of the pronator drift test in each limb. Outputs from Vicon and MCS were analysed using Matlab to determine root mean square error (RMSE) in XYZ coordinates. A priori, an acceptable difference was considered to be an average RMSE of < 10 mm.
Results | Collectively, the studies generated 53,424 sets of data. The average RMSE in the XYZ axis was 14.9 mm (range 5.0-20.3 mm). Inaccuracy was greatest at the wrist during trials involving larger degrees of pronation.
Conclusion | The motion capture system was able to generate a 3-D trajectory of limb motion but further refinement is needed before it can be used for the purposes of clinical measurement.
53 Breakout Session 5 | Neuromuscular and Stroke Chairs: Belinda Cruse and Antonia Carroll
13:30 | Breakout Session 5 | Neuromuscular and Stroke Andersen-Tawil Syndrome: Multi-System Deep Phenotyping of a large UK cohort
Vinojini Vivekanandam1, Michael G Hanna1, Emma Matthews1
1. University College London, London, United Kingdom
Andersen-Tawil Syndrome (ATS) is a rare channelopathy caused by mutations in the KCNJ2 gene that encodes the ubiquitously expressed Kir2.1 potassium channel. In our paper, we describe key findings in a large UK cohort of 52 patients, pertinent to the diagnosis and management of ATS. We report a new point prevalence of 0.105 per 100 000 (increased from 0.08 per 100 000).
While ATS has historically been considered a triad of episodic weakness, cardiac arrhythmias and dysmorphic features, we show that there is considerable variability to this phenotype. Pure cardiac or muscle phenotypes may exist. The absence of dysmorphic features does not exclude the diagnosis. Similarly, a normal long exercise test was seen in five patients.
Importantly, we identify that the phenotype includes a significant risk of cardiac morbidity and mortality with 13% of our cohort requiring cardiac defibrillator or pacemaker insertion and an additional 23% reporting syncope. Syncope has been recently associated with an increased risk of life threatening arhythmic events in this cohort. Severe fixed myopathy was seen in a quarter of our cohort with 14% requiring a wheelchair or gait aid.
This is the largest multi-system study in ATS and provides key clinical insights to improve diagnosis, as well as management recommendations to address the potential for severe muscle weakness and cardiac morbidity and mortality.
13:37 | Breakout Session 5 | Neuromuscular and Stroke A Cross-Sectional Study of Patients with Chronic Inflammatory Demyelinating Polyneuropathy (CIDP): Identifying Ultrasonographic Features for Diagnosis and Prognosis
Nicholas Crump1, 2, Richard Macdonell1, Michael Cartwright3
1. Austin Health, Heidelberg, VIC, Australia 2. Departments of Medicine and Neurology, University of Melbourne, Austin Campus, Heidelberg, VIC, Australia 3. Department of Neurology, Wake Forest Baptist Medical Center, Winston-Salem, NC, USA
Objectives | Diagnosis and treatment monitoring in CIDP is primarily based on clinical parameters. High-frequency ultrasound of peripheral nerves can reflect CIDP pathophysiology and changes with treatment. This project investigated potential diagnostic and prognostic biomarkers utilizing neuromuscular ultrasound.
Methods | We conducted a standardized clinical and ultrasonographic assessment of 50 CIDP patients (25 at WFBMC, 25 at Austin), comparing to 25 healthy controls and 25 axonal neuropathy subjects. Our protocol included whole-length assessment of both median and ulnar nerves, with unilateral assessment of other nerves.
Results | 25 of 25 CIDP patients studied at WFBMC had an abnormality on ultrasound (as determined by focal nerve enlargement determined by increased cross-sectional area), with 23 of 25 subjects having >=4 enlarged segments. 23 of 25 Austin CIDP patients had at least one enlarged segment, and 20 of 25 had >=4 enlarged segments. 46 of 48 had at least one abnormality in either median or ulnar nerve.
54 Mild nerve enlargements were infrequently seen in healthy and disease controls. However, CIDP patients had clear difference in extent and pattern of enlargements, particularly with proximal upper limb enlargement. Specific markers differentiating CIDP patients will be presented.
We analyzed our data in line with previously published diagnostic scores. We will discuss these findings for typical vs. atypical CIDP subtypes, and clinical correlations.
Conclusions | This cross-sectional study of neuromuscular ultrasound in patients with CIDP suggests assessing bilateral whole-length median and ulnar nerves may be adequate for diagnosis, and differentiating potentially treatment responsive immune-mediated neuropathies from axonal neuropathies and healthy controls.
13:44 | Breakout Session 5 | Neuromuscular and Stroke Posturography as a biomarker of IVIG efficacy in CIDP patients
Matthew Silsby1, Con Yiannikas2, Karl Ng2, Matthew C Kiernan3, 4, Victor SC Fung1, Steve Vucic1
1. Neurology, Westmead Hospital, Westmead, NSW, Australia 2. Neurology, Royal North Shore Hospital, St Leonards, NSW, Australia 3. Neurology, Royal Prince Alfred Hospital, Sydney, NSW, Australia 4. Brain and Mind Centre, University of Sydney, Camperdown, NSW, Australia
Introduction | Poor balance is a common and debilitating feature in patients with Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP). Posturography is an objective method of assessing balance. Intravenous Immunoglobulin (IVIG) exerts clinical benefits in CIDP, including improving balance, although this is difficult to quantify. The present study used posturography as a biomarker for determining IVIG efficacy in CIDP.
Methods | Eighteen patients with CIDP established on IVIG were compared with healthy controls. Five conditions were used to assess balance: Feet 16cm apart with eyes open and closed, feet together with eyes open and closed (Romberg’s test), and tandem stance. Centre of pressure (COP) was sampled for 15 seconds at 100Hz using a Kistler force platform, and the total path travelled by the COP was calculated (sway path). Testing was performed on the day of IVIG, corresponding to a trough, and at the mid-point of treatment, corresponding to a peak.
Results | At baseline, there was a significant increase in the sway path in CIDP patients compared with healthy controls (average performance 1191 ± 104mm vs 724 ± 26mm, P<0.001). Treatment with IVIG resulted in a significant reduction in the sway path when assessing Romberg’s test (1759 ± 324mm vs 1081 ± 134mm, P=0.019) and tandem stance (1775 ± 290mm vs 1152 ± 113mm, P=0.027). In contrast, clinical markers of neuropathy were unchanged, and repeat assessments in healthy controls were stable.
Conclusion | The present study shows that objective assessment of balance using posturography may serve as a biomarker of IVIG efficacy in patients with CIDP.
13:51 | Breakout Session 5 | Neuromuscular and Stroke Effect of Inter-hospital transfer in patients undergoing endovascular thrombectomy in the early and late time window
Leon Edwards1, 2, 3, Christopher Blair1, 2, 3, Dennis Cordato1, 2, 3, Nathan Manning4, 5, 6, Andrew Cheung2, 4, 5, 3, Jason Wenderoth4, 5, 7, Cecilia Cappelen-Smith1, 2, 3
1. Neurology and Neurophysiology, Liverpool Hospital, Liverpool, NSW, Australia 2. Ingham Institute for Applied Medical Research, Ingham Institute, Liverpool, NSW, Australia 3. South Western Sydney Clinical school, University of New South Wales, Liverpool, NSW, Australia 4. Institute of neurological sciences, Prince of Wales Hospital, Randwick, NSW, Australia 5. Neurointervention, Liverpool Hospital, Liverpool, NSW, Australia 6. The Florey institute of neurosciences, Melbourne, VIC, Australia 7. Prince of Wales Clinical School, Prince of Wales, Randwick, NSW, Australia
55 Objective | Assess the effect of inter-hospital transfer and treatment time on mortality, reperfusion rates and workflow time metrics in patients undergoing endovascular thrombectomy (EVT) for acute ischaemic stroke (AIS) due to large vessel occlusion (LVO) in the anterior cerebral circulation (ACC).
Methods | Analysis of a prospective database of consecutive patients undergoing EVT for LVO presenting between January 2017-December 2018 at a single Australian comprehensive stroke centre (CSC). Patients presented directly or were transferred to the CSC from 21 sites across New South Wales and the Australian Capital Territory. A definition of ≤6 hours or >6 hours of stroke onset to treatment (groin puncture) was used for the early time window (ETW) and late time window (LTW) respectively.
Results | 154/213 (72%) patients were inter-hospital transfers.There was no significant difference in baseline characteristics including age, National-Institute-of-Health-Stroke-Scale-score, intravenous thrombolysis administration or procedure time between transferred and direct presenters (all p>0.05). Transferred patients within the ETW had worse 90-day functional outcome (35.6% vs 61.0%, Odds ratio [OR] 0.36, 95% confidence interval [CI] 0.17-0.75), higher mortality (25.3% vs 6.8%, OR 6.57, CI 1.48- 29.32) and longer stroke-onset to treatment time (180 vs 245 minutes, p<0.01). In the LTW transferred patients there was no significant difference in 90-day functional outcome, mortality or stroke-onset to treatment compared with the directly presenting patients (all p>0.05). Successful reperfusion rates and sICH were similar between cohorts (all p>0.05)
Conclusion | Inter-hospital transfer in the ETW but not LTW is associated with longer stroke-onset to treatment, worse 90-day functional outcome and higher mortality.
13:58 | Breakout Session 5 | Neuromuscular and Stroke Adjunctive intraarterial thrombolysis in endovascular clot retrieval: a systematic review and meta-analysis
William K Diprose1, Michael T. M. T.M Wang2, Kaustubha Ghate1, Stefan Brew3, James R Caldwell3, Ben McGuinness3, P. Alan Barber2
1. Department of Neurology, Auckland City Hospital, Auckland, New Zealand 2. Department of Medicine, University of Auckland, Auckland, New Zealand 3. Department of Radiology, Auckland City Hospital, Auckland, New Zealand
Objective | To evaluate the safety and efficacy of intra-arterial thrombolysis (IAT) as an adjunct to endovascular clot retrieval (ECR) in ischaemic stroke, we performed a systematic review and meta- analysis of the literature.
Methods | Searches were performed using Medline, Embase, and Cochrane databases for studies that compared ECR to ECR with adjunctive IAT (ECR+IAT). Safety outcomes included symptomatic intracerebral haemorrhage (sICH) and mortality at three months. Efficacy outcomes included successful reperfusion (Thrombolysis in Cerebral Infarction score of 2b to 3), and functional independence, defined as a modified Rankin Scale score of 0 to 2 at three months.
Results | Five studies were identified that compared combined ECR+IAT (IA alteplase or urokinase) to ECR-only, and were included in the random effects meta-analysis. There were 1693 ECR patients, including 269 patients treated with combined ECR+IAT and 1424 patients receiving ECR-only. Pooled analysis did not demonstrate any differences between ECR+IAT and ECR-only in rates of sICH (OR: 0.61, 95% CI: 0.20-1.85; P=0.78), mortality (OR: 0.77, 95% CI: 0.54-1.10; P=0.15), or successful reperfusion (OR: 1.05, 95% CI: 0.52-2.15; P=0.89). There was a higher rate of functional independence in patients treated with ECR+IAT, although this was not statistically significant (OR: 1.34, 95% CI: 1.00-1.80; P=0.053).
Conclusions | Adjunctive IAT appears to be safe. In specific situations, neurointerventionists may be justified in administering small doses of intraarterial alteplase or urokinase as rescue therapy during ECR.
56 14:05 | Breakout Session 5 | Neuromuscular and Stroke High Sensitivity Troponin in Acute Ischaemic Stroke Study (TACIS)
Andrew Hannaford1, Michael Hayes2, John Worthington3, Timothy Ang3, Nimalin Harinesan2
1. Neurology, Westmead Hospital, Sydney, NSW, Australia 2. Neurology, Concord Repatriation General Hospital, Sydney, NSW, Australia 3. Neurology, Royal Prince Alfred Hospital, Sydney, NSW, Australia
Objective | We designed a multi-centre prospective cohort study to explore the hypothesis that early acutely elevated high sensitivity troponin (hsT) is associated with cardioembolic stroke (CES)
Methods | Ischaemic strokes across three hospitals underwent hsTroponin testing and 2 blinded clinicians classified patients as CES, NCE (Non Cardioembolic) or ESUS by ESUS criteria. Characteristics included baseline NIHSS, renal function, hypertension, diabetes, smoking, ischaemic heart disease, past stroke and congestive cardiac failure. The odds of positive hsT for CES Vs NCE and ESUS Vs NCE were modelled with step-wise addition of patient characteristics.
Results | 194 ischaemic stroke cases were included, with a mean age of 71 years and a 57:43 male:female ratio. 65 had a positive hsTroponin, which was associated with older age, hypertension, cardiac failure, coronary disease, an eGFR < 60 and a higher NIHSS. Positive hsTroponin was associated with CES (OR, 2.06; 95% CI, 1.12-3.79; P = 0.02). This association persisted after adjusting for confounders, such as age, sex, atrial fibrillation, renal impairment, ischaemic heart disease and previous stroke (Adjusted OR, 4.07; 95% CI, 1.41–11.75; P=0.01). ESUS was negatively associated with an elevated hs troponin (OR, 0.45; 95% CI, 0.22–0.94; P=0.03). This was not significant when adjusting for other variables (P=0.09).
Conclusions | An elevated hs troponin after acute ischaemic stroke is independently associated with a cardioembolic mechanism. High sensitivity cardiac troponin was not significantly associated with ESUS after adjusting for confounders, suggesting that a cardio-embolic cause may not be the dominant mechanism in this group.
Breakout Session 6 | Infectious Diseases Chairs: Zoe Woodward and Susan Tomlinson
13:30 | Breakout Session 6 | Infectious Diseases Brain aging and cardiovascular risk factors in chronic HIV: A longitudinal MRI study
David Jakabek1, 2, 3, Caroline D Rae2, 3, Bruce J Brew1, 2, 3, 4, Lucette A Cysique1, 2, 5
1. Departments of Neurology and HIV Medicine, St Vincent’s Hospital, & Peter Duncan Neurosciences Unit, St Vincent’s Centre for Applied Medical Research, Sydney, NSW, Australia 2. Neuroscience Research Australia, Sydney, NSW, Australia 3. Faculty of Medicine, University of New South Wales, Sydney, NSW, Australia 4. Faculty of Medicine, University of Notre Dame, Sydney, NSW, Australia 5. UNSW Psychology, University of New South Wales, Sydney, NSW, Australia
Objectives | We aimed to examine the relative contributions of HIV infection, age, and cardiovascular risk factors to subcortical brain atrophy.
Methods | Virally suppressed HIV+ participants with low neuropsychological confounds (n = 75) and demographically matched HIV- controls (n = 31) completed baseline and 18-month follow-up MRI scans, neuropsychological evaluation, cardiovascular assessments, and laboratory tests. HIV+ participants were evaluated for HIV associated neurocognitive disorder (HAND). Subcortical volumes were extracted with Freesurfer. Volumetric and shape analyses were conducted using linear mixed- effect models incorporating interactions between age, time, and each of HIV status, HAND status, HIV disease factors, and cardiovascular markers.
57 Results | HIV+ participants had smaller volumes of most structures compared to HIV- participants. Premature aging was evident in the pallidum using volumetric (p = 0.032) and shape analyses. Accelerated aging was observed in the caudate volumes for the more severe HAND subgroup (p = 0.008) and was associated with longer HIV duration for putamen volumes (p = 0.04). Higher CD4 counts had a protective effect on hippocampal volumes in older participants (p = 0.04). Cardiovascular measures were associated with smaller volumes across time for most structures; only the putamen demonstrated accelerated atrophy over time in HIV+ participants with higher cardiovascular risk factors (p = 0.002).
Conclusion | The study demonstrates a three-hit model of subcortical injury in HIV+ individuals: HIV-driven atrophy in most subcortical structures; abnormal brain aging and HIV infection synergy in the caudate and pallidum; and cardiovascular-related injury linked to diffuse premature atrophy and emerging accelerated atrophy in the putamen.
13:37 | Breakout Session 6 | Infectious Diseases COVID-19 disease outcomes in a UK Myasthenia Centre during the first year of the pandemic
Paul Kopanidis1, Mary Quirke1, Camilla Buckley1, Isabel Leite2
1. John Radcliffe Hospital, Oxford, OXFORDSHIRE, United Kingdom 2. Nuffield Department of Neurosciences, Oxford, United Kingdom
Objectives | Describe the outcomes of patients treated at the Myasthenia Centre at Oxford University Trust who developed COVID-19 during the first year of the pandemic.
Methods | Retrospective audit of patients diagnosed with COVID-19 between 31st January 2020 – 31st January 2021. Outcomes of COVID-19 complications, including relapse of myasthenia gravis (MG), were analysed.
Results | The Myasthenia Centre treated 487 patients, including 370 with acetylcholine receptor (AChR) MG, 74 sero-negative MG, 20 MuSK MG and 23 Lambert-Eaton Myasthenic Syndrome (LEMS). COVID-19 was diagnosed in a total of twelve patients (2.5%) including ten AChR, one MuSK and one LEMS patient, with a mean age of 63.8 years (range 20 – 92 years). Five patients were asymptomatic of MG prior to the diagnosis of COVID-19. Treatments prior to diagnosis included pyridostigmine (8/12), prednisolone (7/12), azathioprine (3/12), mycophenolate (1/12) and rituximab (1/12). The majority (8/12) had at least one other co-morbid risk factor for severe COVID-19.
COVID-19 resulted in hospital admission in six patients, with three requiring intensive care treatment. One patient with AChR MG (with NHL and NMO treated with rituximab) died from COVID-19 without MG relapse. Two elderly patients developed moderate COVID-19 after a single dose BioNTech vaccination without MG relapse.
MG relapse occurred in four patients post COVID-19, with two requiring inpatient management including IVIG.
Conclusion | COVID-19 disease was associated with relapse of MG, with all patients in this group surviving. Further research is required to establish if COVID-19 precipitates MG relapse at a different rate compared to other infectious diseases.
13:44 | Breakout Session 6 | Infectious Diseases Cognitive deficits are associated with anosmia but not anxio- depressive symptoms in COVID-19
Lucette A Cysique1, 2, Yasmin Allen-Davidian3, 2, David R Darley4, 5, Anthony Byrne4, 5, Kay Wilhelm6, 5, Greg Dore7, 8, Gail Matthews7, 8, Bruce J Brew9, 5, 2
1. Psychology, The University of New South Wales, Sydney, NSW, Australia 2. Peter Duncan Neuroscience Unit, Sydney St. Vincent’s Applied Medical Research Centre, Darlinghurst, NSW, Australia
58 3. Psychology, Macquarie University, Sydney, NSW, Australia 4. Respiratory Medicine, Sydney St. Vincent’s Hospital, Darlinghurst, NSW, Australia 5. Medicine, The University of New South Wales, Sydney, NSW, Australia 6. Psychiatry, Sydney St. Vincent’s Hospital, Darlinghurst, NSW, Australia 7. Kirby Institute, The University of New South Wales, Sydney, NSW, Australia 8. Infectious Diseases, Sydney St. Vincent’s Hospital, Sydney, NSW, Australia 9. Neurology, Sydney St. Vincent’s Hospital, Darlinghurst, NSW, Australia
Objectives | To characterise cognitive performance and olfaction in recovered COVID-19 patients
Methods | Patients underwent cognitive, olfaction and mental health assessments 2 months after initial SARS-CoV-2 infection as part of the Sydney St. Vincent’s Hospital ADAPT study, a prospective cohort study. Cognition was assessed with the Cogstate computerised battery and expressed as a demographically-corrected composite z-score and clinically classified as impaired/borderline/ unimpaired. Anxio-depressive symptoms were assessed with the Depression in the Medical ill scale-10 (DMI-10), the Somatic and Psychological HEalth Report-34 (SPHERE) Psych sub-scale, and the Impact of Events Scale-Revised (IESR) and reduced into single Principal Component explaining 80% of the variance. Olfaction was assessed with the NIH Toolbox Odor Identification test and expressed as demographically-corrected T-scores, and impaired/unimpaired. Disease severity was classified as mild (40%), moderate (50%) or hospitalised (10%)
Results | 132 patients (mean age=46±15; 40% women, median education=16 years, 10% Non-English- Speaking Background-NESB) were included. 17% had impaired cognition, 10% had borderline deficits, 25% has impaired olfaction. 25% had clinically elevated symptoms on the DMI-10, 13% on the IESR, and 35% on the SPHERE. Regression analyses showed that anxio-depression was not associated with cognitive performance (unadjusted p=.43; adjusted for sex & NESB p=.98) nor impaired/unimpaired status (unadjusted p=.50; adjusted for sex & NESB p=.78). Cognitively impaired patients were more likely to have impaired olfaction (p<.009). Results were independent of disease severity.
Conclusions | Cognitive impairment is common and not related to psychological factors, may occur independent of disease severity and is associated with anosmia. These point to direct brain effects of COVID-19.
13:51 | Breakout Session 6 | Infectious Diseases COVID:19 The Epicentre of Neurological Events in the Human Brain
Nicholas Parsons1, Fiore D’Aprano2, Athanasia Outsikas3, Annie Parish3, Fidel Toomey4, Shailesh Advani5, Govinda Poudel6
1. Cognitive Neuroscience Unit, Deakin University, Melbourne, VIC, Australia 2. Department of Neurology, Royal Melbourne Hospital, Melbourne, VIC, Australia 3. School of Psychology, Deakin University, Melbourne, VIC, Australia 4. School of Medicine, Deakin University, Melbourne, VIC, Australia 5. National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, USA 6. Department of Health Sciences, Mary Mackillop Institute for Health Research, Melbourne, VIC, Australia
Neuropathology caused by COVID-19 has been widely reported, and the characterisation of the spatial distribution of these pathology remains critical to assess long and short-term neurological sequelae. We performed a systematic review of the literature to quantify the locations of small neurological events identified with magnetic resonance imaging (MRI) among COVID-19 patients. Neurological events were localised into the Desikan-Killiany grey and white matter atlases. A mathematical network diffusion model was then used to test whether the spatial distribution of neurological events could be explained via a linear spread through the structural connectome of the brain. The highest proportions (26%) of white matter events were observed within the bilateral corticospinal tracts. The highest proportions (~10%) of grey matter events were observed in areas including the bilateral superior temporal, precentral, and lateral occipital cortices. Sub-cortical events were most frequently identified in the Pallidum. The application of a mathematical network diffusion model suggested that the spatial pattern of the small neurological events in COVID-19 can be modelled with a linear diffusion of spread from epicentres in the bilateral cerebellum and basal ganglia (Pearson’s r=0.41, p<0.001, corrected). To our knowledge, this is the first study to systematically characterise the spatial distribution of small neurological events in COVID-19 patients and test whether the spatial distribution of these events can be explained by a linear diffusion spread model.
59 As such, initial sub-cortical events which manifest as altered consciousness could be expected to be followed by later cortical events manifesting as altered sensorimotor functioning.
13:58 | Breakout Session 6 | Infectious Diseases MR spectroscopy and dynamic contrast-enhanced perfusion studies in two SARS-CoV-2 infection patients with neurological complications and no other MR abnormalities
Xin Zhang1, 2, Joga Chaganti2, Bruce Brew2
1. Royal Prince Alfred Hospital, Camperdown, NSW, Australia 2. St Vincent’s Hospital Sydney, Darlinghurst, NSW, Australia
Objectives | To understand NeuroCovid pathogenesis by using MR spectroscopy and dynamic contrast perfusion in 2 SARS-COV-2 patients with neurological complications.
Methods | MR spectroscopy (MRS) and dynamic contrast-enhanced perfusion (k-trans), which is a sensitive marker of blood brain barrier (BBB) damage, were performed in 2 patients with respiratory viral PCR confirmed SARS-CoV-2 infection and 2 local controls. The MRS and k-trans results were also compared with published controls.
Results | Case 1 was a 73-year-old man with critical SARS-CoV-2 infection requiring 4 days of mechanical ventilation. He had persistent anosmia, anorexia and apathy; saccadic pursuit eye movements, action tremor in the left hand and a positive right-sided palmomental reflex. These changes normalised by day 30 of presentation. MRI brain was undertaken on day 37.
Case 2 was a 61-year-old man with critical SARS-CoV-2 infection requiring 43 days of mechanical ventilation. He was slow to wake post weaning of sedation and had residual mild cognitive impairment. MRI brain was undertaken on day 62 of presentation.
No abnormalities were detected on T1, T2, FLAIR, DWI, SWI MR sequences. However, for both patients there was diffuse increase in k-trans especially in the frontal cortex and increased glutamate- glutamine MRS signal intensities in the pons compared to controls.
Conclusions | The MRS and k-trans changes show excitotoxicity and BBB damage, in the absence of stroke or MR-defined white matter injury. They suggest a direct effect of SARS-CoV-2 on the brain. These MR techniques can offer insight into NeuroCovid pathogenesis when patients are no longer infectious.
1. Heye AK, Culling RD, Valdés Hernández MC, Thrippleton MJ, Wardlaw JM. Assessment of blood-brain barrier disruption using dynamic contrast-enhanced MRI. A systematic review. Neuroimage Clin. 2014;6:262-274. 2. Langer DL, Rakaric P, Kirilova A, Jaffray DA, Damyanovich AZ. Assessment of metabolite quantitation reproducibility in serial 3D-(1)H-MR spectroscopic imaging of human brain using stereotactic repositioning. Magn Reson Med. 2007;58(4):666-673. 3. Chang L, Munsaka SM, Kraft-Terry S, Ernst T. Magnetic resonance spectroscopy to assess neuroinflammation and neuropathic pain. J Neuroimmune Pharmacol. 2013;8(3):576-593.
14:05 | Breakout Session 6 | Infectious Diseases Case-control study of risk factors for stroke among critically-ill patients with SARS-CoV-2: An analysis of the COVID-19 Critical Care Consortium (CCCC) global registry
Jonathon P Fanning3, 2, 1, Adrian Barnett4, Glenn Whitman5, Rakesh Arora6, Lavien Premraj7, Denise Battaglini8, Sam Huth2, 1, Diego Bastos Porto9, Huimahn Choi10, Jacky Suen2, 1, Gianluigi Li Bassi2, 1, 11, John Fraser2, 1, 12, Matthew Griffee13, Chiara Robba8, Sung-Min Cho14
1. Critical Care Research Group, The Prince Charles Hospital, Chermside, QLD, Australia 2. Faculty of Medicine, The University of Queensland, Brisbane, QLD, Australia 3. Department of Neurology, Gold Coast University Hospital, Southport, QLD, Australia
60 4. Australian Centre for Health Services Innovation (AusHSI) and Centre for Healthcare Transformation, School of Public Health & Social Work, , Queensland University of Technology, Brisbane, QLD, Australia 5. Cardiac Intensive Care Services, Johns Hopkins Hospital and University, Baltimore, MA, USA 6. Cardiac Sciences Program, St. Boniface General Hospital Research Centre, Winnipeg, Manitoba, Canada 7. Griffith University, Gold Coast, QLD, Australia 8. San Martino Policlinico Hospital, IRCCS for Oncology and Neuroscience, University of Genoa, Genoa, Italy 9. Hospital Sao Camilo de Esteio, Tamandaré, Esteio, Brazil 10. University of Texas Health Sciences Center, Houston, Texas, USA 11. Institut d’Investigacions Biomediques August Pi I Sunyer, Barcelona, Spain 12. St. Andrew’s War Memorial Hospital, Brisbane, QLD, Australia 13. Department of Anesthesiology, University of Utah, Salt Lake City, Utah, USA 14. Neurosciences Critical Care Division, Departments of Neurology, Neurosurgery and Anesthesiology and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, MA, USA
Objective | COVID-19 has been identified as a risk factor for severe cerebrovascular complications, albeit mostly in small patient populations, limited to specific regions, and including all severities of disease. Utilising the largest database of critically-ill COVID-19 patients, we investigated risk factors for stroke in intensive care unit (ICU) COVID-19 patients.
Methods | Data for this matched case-control study were extracted from a large international registry of adult COVID-19 patients requiring ICU admission. Patients with imaging-confirmed cerebrovascular events identified following ICU admission were compared against five controls per case, matched for demographics, morphometrics, illness severity, and ICU days. Expert consensus determined key clinical and laboratory variables for risk assessment.
Results | From January 1-December 21 2020, 2,715 ICU patients were registered across >370 sites spanning 52 countries; acute stroke was identified during the ICU stay in 59(2.2%); 27(46%) haemorrhagic, 19(32%) ischaemic, 13(22%) unspecified. Stroke patients had higher SOFA and APACHE scores, more frequent hypertension and cardiovascular disease, and more often required mechanical ventilation, vasopressors, and ECMO. Diabetes, hypertension, smoking, and Caucasian ethnicity were identified as risk factors for ischaemic versus haemorrhagic stroke and being stroke-free. Ethnicity (Hispanic or black), higher PaO2, and extracorporeal membrane oxygenation (ECMO) were significant risk factors for haemorrhagic stroke.Anticoagulation had no association with either stroke subtype.
Conclusions | Severe illness and more aggressive management were major risk factors for acute stroke. Traditional vascular risk factors and Caucasian ethnicity were risk factors for ischaemic stroke, while Hispanic or black ethnicity, higher PaO2, and ECMO were significant risk factors for haemorrhagic stroke.
Ian McDonald Lecture Chair: Deborah Mason
15:00 | Ian McDonald Lecture | Conceptions and Conquerings MS Redux: Looking back over 48 years
Ernie Willoughby
A personal review of the substantial advances in diagnosis and management of multiple sclerosis since the mid-1970s, when CT and MR scanning were not available and there was no established disease-modifying therapy. Given the limited time available, the aspects covered are necessarily somewhat selective and limited, with more attention to diagnosis and MR scanning than to a review of available therapies. Distinction of MS from other CNS inflammatory disorders (especially NMOSD and other syndromes related to Aquaporin-4 and MOG antibodies) has been important, with more contentious issues being the place of MS variants (such as Marberg’s fulminant MS, progressive solitary sclerosis and myelocortical MS), views on the (still-unknown) cause, particularly the relevance of the extensively studied autoimmune animal model, EAE, and the pathological basis of the low- grade progressive phase of the disease. There have been stumbles along the way, and final comments will include thoughts on the path ahead.
61 Thursday 20 May 2021
09:30 – 12:00 Acute Neurology for Practice Chairs: Anna Ranta and Tom Wellings Symposium sponsored by Novartis
09:30 Hyperacute Stroke including ICH Bruce Campbell
09:50 Refractory and super-refractory status epilepticus Michelle Kiley
10:10 Continuous EEG (cEEG) in the ICU: why and why not? John Dunne
10:30 Movement Disorder Crises Victor Fung
10:50 Q&A time All Speakers
11:00 Stroke: Complex Case Discussions Ian Wilson, Bruce Campbell
12:00 – 13:00 Lunch Break Poster Viewing Session 2 Sponsored by Roche
13:00 – 15:00 The Young Achievers Chairs: Amy Brodtmann and Michael Dreyer Symposium sponsored by Biogen
13:00 Leonard Cox Award 2020 Rebekah Ahmed Physiological changes in Neurodegenerative Dementias
13:30 – 14:30 Young Investigator Award Presentations
13:30 Nerve excitability and motor unit number estimation: early Antonia Carroll biomarkers of nerve involvement in hereditary amyloidosis (ATTRv)
13:37 The gut microbiome in Parkinson’s disease: longitudinal Michal Lubomski insights into disease progression and the use of device- assisted therapies
13:44 Resolving Infectious Meningitis in Uganda with Metagenomics Prashanth and Host Transcriptomics Ramachandran
13:51 Prevalence of MRI signs of intracranial hypertension and Benson Chen their association with papilledema: A prospective study using ocular fundus photography
13:58 The Quest to Reduce Stroke Treatment Delays at A Peter Park Melbourne Metropolitan Primary Stroke Centre over the Last Two Decades
14:05 Cognitive impairment in late onset epilepsy Xin Zhang
14:12 Q&A time All Speakers
62 14:30 Leonard Cox Award 2021 Piero Perucca The Genetics of Focal Epilepsies: From Research to Clinical Practice
15:00 Meeting Close Day 2
15:00 – 16:30 Virtual Walk #mdok Share course and photos by App 5 km Walk of your choice ANZAN Phone App for live photo updates
63 Thursday 20 May 2021
Acute Neurology for Practice Chairs: Anna Ranta and Tom Wellings
Symposium sponsored by Novartis
09:30 | Acute Neurology for Practice Hyperacute Stroke including ICH
Bruce Campbell
This update lecture will cover the latest evidence on thrombolysis, thrombectomy and the interface between these two reperfusion strategies. Systems of care including pre-hospital triage and mobile stroke units, stroke telemedicine and imaging strategies will be discussed. Potential therapeutic strategies for intracerebral haemorrhage and stroke secondary prevention will be explored.
09:50 | Acute Neurology for Practice Refractory and super-refractory status epilepticus
Michelle Kiley
Refractory and super-refractory status epilepticus are conditions which carry high morbidity and mortality and for which is there is virtually no class I or II evidence to guide management. Many guidelines are therefore based on expert opinion. In this presentation the goals of management, including the suppression of clinical and electrographic seizure activity, treating the cause of status epilepticus, subjecting the patient to minimal anaesthesia, thus minimising side effects and complications, and successfully weaning the patient off sedation, will be discussed.
10:10 | Acute Neurology for Practice Continuous EEG (cEEG) in the ICU: why and why not?
John Dunne
EEG can direct patient care like no other investigation, so is cEEG even better? Many technical challenges need to be overcome for EEG in the ICU, but to be useful no shortcuts are possible. This includes using a full 10-20 electrode array. Limited montages have inadequate sensitivity for spikes and seizures.
Abnormal movements and posturing are very common in the critically ill, and they are usually not seizure-related. The exclusion of seizures is perhaps EEG’s most useful role in the ICU, since this prevents inappropriate treatment, and for this cEEG is usually not required.
EEG knowledge is evolving. Different EEG patterns form a continuum of non-seizure to seizure activity, and over-interpretation of these patterns may lead to inappropriate advice and treatment. In particular, cEEG commonly shows various periodic patterns, sometimes stimulus-evoked, that are 64 often misinterpreted as seizures. To make EEG helpful rather than harmful, EEG interpretation needs to be made within the clinical context by an expert reporter. Careful training and competency in EEG reading is essential, but unfortunately this is not always the case. cEEG is no substitute for accurate clinical assessment. Recording (and watching/listening to!) video- audio with cEEG is also essential to identify artefacts mimicking seizures and stimulus-evoked changes. cEEG is resource-intensive and time-consuming. Whilst computer-assisted approaches, including machine learning, can assist with cEEG review, they lack sensitivity and specificity. An expert EEG reporter must review the raw EEG. cEEG commonly detects epileptic seizures in acutely ill patients. Seizures can be associated with poorer prognosis, but is this seizure activity a therapeutic target? Is missing non-convulsive seizures (however defined) in the critically ill important? We still do not know whether seizures are worsening the various illnesses or represent a diagnostic or prognostic marker, simply reflecting outcome. Specific EEG findings combined with the clinical context determine the approach. For example, in subarachnoid haemorrhage electrographic seizures (and slowing) are a marker of vasospasm and ischaemia. In intracranial haemorrhage, seizures are a marker further bleeding/swelling. After cardiac arrest, despite differing EEG definitions, seizure activity almost invariably predicts poor outcome. Epileptic seizure treatment does not alter outcome in these patients. Counter-intuitively, this may even apply with acute seizure presentations, since seizures are a symptom, and aetiology mainly determines prognosis. cEEG increases costs and length of hospital stay, but as yet has not been clearly shown to improve outcomes in the critically ill, including in a recent randomised trial comparing cEEG with routine EEGs.
A 30-60 minute EEG, repeated if necessary, answers most questions in the ICU. With respect to seizures, most are detected within the first 30-60 minutes of EEG, and if unclear, a prolonged 1-4 hour EEG clarifies the situation for most patients. cEEG can be a valuable tool, but is expensive, requires adequate resourcing, is vulnerable to overuse, and the wrong hands can be harmful. EEG quality rather than quantity, combined with accurate clinical assessment, is far more important.
10:30 | Acute Neurology for Practice Movement Disorder Crises
Victor Fung
11:00 | Acute Neurology for Practice Stroke: Complex Case Discussions
Ian Wilson, Bruce Campbell
65 The Young Achievers Chairs: Amy Brodtmann and Michael Dreyer
Symposium sponsored by Biogen
13:00 | Leonard Cox Award 2020 | The Young Achievers Physiological changes in Neurodegenerative Dementias
Rebekah Ahmed
Increasingly, it is recognised that the effects of neurodegenerative dementia syndromes extend beyond that of cognitive and motor systems to involve key physiological systems within the body including eating and metabolism, the autonomic nervous system, and sleep. Changes in these physiological systems are present across a number of conditions including frontotemporal dementia, amyotrophic lateral sclerosis. Key neural structures mediating physiological change across these conditions include neuroendocrine and hypothalamic pathways, reward pathways, motor systems and the autonomic nervous system. The key changes in physiological processing in dementia will be reviewed and the overlap across the progressive neurodegenerative brain conditions, which may provide novel insights into the human neural correlates of physiological functioning. Given the increasing evidence that physiological changes may arise early in the neurodegenerative process, these changes may provide potential biomarkers to aid in early diagnosis and potential treatment targets.
Young Investigator Award Presentations
13:30 | Young Investigator Award Presentation Nerve excitability and motor unit number estimation: early biomarkers of nerve involvement in hereditary amyloidosis (ATTRv)
Antonia Carroll1, 2, 3, 4, Cindy Lin1, Susanna Park1, Neil Simon5, Mary Reilly2, Steve Vucic3, Matthew Kiernan1
1. Brain and Mind Research Centre, Camperdown, NSW, Australia 2. MRC Centre for Neuromuscular diseases, National Hospital of Neurology and Neurosurgery, London, UK 3. Westmead Hospital, University of Sydney, Sydney, NSW, Australia 4. St Vincent’s Hospital, University of NSW, Sydney, NSW, Australia 5. Northern Clinical School, University of Sydney, Sydney, NSW, Australia
Objective | Gene silencing treatments for hereditary transthyretin amyloidosis (ATTRv) have recently been developed with dramatic improvements observed in patient outcomes. However, the optimal time to initiate treatment is not yet known. The aim of this study is to explore the pathophysiological progression of neuropathic features of ATTRv using nerve excitability and motor unit number estimation.
Methods | We prospectively recruited 14 symptomatic patients and 7 asymptomatic carriers and with varied TTR mutations and compared these to 21 healthy controls. Nerve excitability properties of ulnar motor and sensory axons, and ulnar-ADM motor unit number estimation was collected.
Results | “Fanning in” of threshold electrotonus was observed in the motor axons of symptomatic ATTRv patients, suggestive of membrane depolarisation. Motor unit number estimation demonstrated a significant reduction in mean unit number between symptomatic and asymptomatic ATTRv
66 patients (p =0.04), with declines seen according to FAP stage and PND score. Significantly increased hyperpolarising current/threshold gradients were seen in sensory axons between symptomatic ATTRv patients and healthy controls (p=0.002), suggesting that upregulation of inwardly rectifying conductance may underlie sensory symptoms and neuropathic pain in ATTRv amyloidosis.
Conclusions | These findings suggest that ulnar nerve excitability and motor unit number estimation could be used as a tool to identify early nerve disease in ATTRv and monitor progression.
13:37 | Young Investigator Award Presentation The gut microbiome in Parkinson’s disease: longitudinal insights into disease progression and the use of device-assisted therapies
Michal Lubomski1, 2, Xiangnan Xu3, Andrew J Holmes4, Samuel Mueller3, Jean Yang3, Carolyn M Sue1, 2, Ryan L Davis2
1. Neurology Department, Royal North Shore Hospital, St Leonards, NSW, Australia 2. Department of Neurogenetics, Kolling Institute, University of Sydney and Royal North Shore Hospital, Sydney, NSW, Australia 3. School of Mathematics and Statistics. Sydney Precision Bioinformatics, University of Sydney, Camperdown, NSW, Australia 4. School of Life and Environmental Sciences, The Charles Perkins Centre, University of Sydney, Sydney, NSW, Australia
Objectives | Previous studies have reported altered gut microbiome (GM) composition in association with motor and non-motor symptoms in Parkinson’s disease (PD). Only a few prior studies considered the influences of PD medications, namely oral therapies, on the GM. We investigated the temporal stability of GM profiles from PD patients initiating device-assisted therapies (DAT) and in a separate cohort characterise GM influences on PD progression.
Methods | Clinical data from validated questionnaires and stool samples from 74 PD patients and 74 healthy controls (HCs) were longitudinally evaluated, at t=0, 6 and 12 months. PD patients were sub-stratified as faster or slower progressors, inferred from levodopa equivalence dose and motor severity measures. Additionally, 19 PD patients receiving Deep Brain Stimulation or levodopa-carbidopa intestinal gel were longitudinally evaluated at t=0, 6 and 12 months post-therapy initiation.
Results | Persistent underrepresentation of short-chain fatty-acid-producing bacteria, Clostridium_ XVIII, Butyricicoccus and Fusicatenibacter was apparent in PD patients compared to HCs. No persisting GM profiles were recognised between faster and slower progressing patients, although predictive modelling supported the use of GM profiles to assist in defining PD progression. Our previous findings of acute GM changes in response to DAT initiation were not sustained at 6 and 12 months, although differing microbiota profiles persisted following DAT initiation.
Conclusions | We present the largest longitudinal GM study in PD patients showing persistently altered GM profiles indicative of underrepresentation of short-chain fatty-acid-producing bacteria. DAT’s were found to exert acute variable influence on the GM that didn’t persist over time.
13:44 | Young Investigator Award Presentation Resolving Infectious Meningitis in Uganda with Metagenomics and Host Transcriptomics
Prashanth Ramachandran1, 2, Akshaya Ramesh1, Fiona Creswell3, Annie Wapniarski 1, Carson Quinn1, M Rutakingirwa 4, Ananta Bandigwala5, E Kagimu 4, KT Kandole 4, Kelsey Zorn 6, L Tugume 4, J Kasibante 4, K Ssebambulidde 4, M Okirwoth 4, Nathan Bahr5, Abdu Musubire 4, Amy Lyden 6, Paula Serpa 7, Gloria Castaneda 7, Saharai Caldera7, Chaz Langelier 7, Emily Crawford 7, David Boulware 5, David Meya 5, Michael Wilson 1
1. Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, California, United States 2. University of Melbourne, Melbourne , VIC, Australia 3. Clinical Research Department, London School of Hygiene and Tropical Medicine, London, WC1E 7HT, United Kingdom 4. Infectious Diseases Institute, Makerere University, Kampala, Uganda 5. University of Minnesota, Minneapolis, Minnesota, United States
67 6. University of California San Francisco, San Francisco, CA, United States 7. Chan Zuckerburg Biohub, San Francisco, California, United States
Objectives | Tuberculous meningitis(TBM) is a common cause of meningitis in sub-Saharan Africa. CSF PCR with GeneXpert RIF/MTB Ultra is only 70% sensitive for detection of definite/probable TBM. Many infections can mimic TBM. Metagenomic next generation sequencing(mNGS) can detect the whole diversity of infectious microbes, but can be insensitive to TB in CSF. We assessed whether leveraging CSF mNGS to identify infections combined with a machine learning classifier(MLC), based on host transcriptomic data generated by mNGS, could enhance diagnostic accuracy for TBM.
Methods | Prospectively enrolled 347 HIV-infected Ugandan adults with subacute meningitis: RNA/ DNA libraries were made from CSF and deep sequenced. Non-human sequences were interrogated to identify pathogens. A host transcriptomic MLC was developed from human RNA transcripts using 70 cases. The MLC and mNGS reporting thresholds were then tested on 108 blinded cases within the cohort.
Results | mNGS was 75% concordant(27/36) for detecting TB in definite TBM cases and 59% concordant(30/51) in definite/probable TBM combined. 3 TB and 3 non-TB pathogens were detected in the probable TBM group. In the possible TBM/indeterminant groups, mNGS identified 3 cases of TBM and 17 other pathogens. The combined mNGS and host-MLC displayed 83.3%(5/6) sensitivity, 86.8%(59/68) specificity, with an area under the ROC curve of 0.83(p=0.009).
Conclusion | mNGS identified an array of infectious TBM mimics, including many treatable and vaccine preventable pathogens. mNGS was 75% concordant with definite TBM. We further enhanced the sensitivity of the CSF mNGS assay by developing the first CSF-based host MLC to discriminate between TBM and its mimics
13:51 | Young Investigator Award Presentation Prevalence of MRI signs of intracranial hypertension and their association with papilledema: A prospective study using ocular fundus photography
Benson S. Chen1, 3, 2, Benjamin I. Meyer1, Amit M. Saindane1, Beau B. Bruce1, Nancy J. Newman1, Valérie Biousse1
1. Emory University School of Medicine, Atlanta, GA, United States 2. Department of Clinical Neurosciences, University of Cambridge, Cambridge, United Kingdom 3. Cambridge Eye Unit, Addenbrooke’s Hospital, Cambridge, United Kingdom
Objectives | MRI signs of intracranial hypertension (MRI-IH) are classically associated with idiopathic intracranial hypertension (IIH), but also detected in asymptomatic individuals without papilledema. The objective of this study was to determine the prevalence of MRI-IH in consecutive outpatients undergoing brain MRI for any clinical indication, and explore their association with papilledema.
Methods | Prospective cross-sectional observational study of outpatients undergoing brain MRI, with ocular fundus photographs taken concurrently. Radiologic studies were analyzed for MRI-IH. Univariate analysis with Fisher’s exact test or t-test was performed.
Results | Of 296 patients included, the most common indication for MRI was surveillance of a brain neoplasm (27.7%). Investigation of headaches (8.8%) or disorders of raised ICP (1.4%) were uncommon. At least one MRI-IH was present in 49% of patients [empty sella (33.1%), enlarged Meckel’s cave (15.9%), increased peri-optic CSF (10.8%), optic nerve tortuosity (7.8%), scleral flattening (0.7%), cephaloceles (1.4%)]. Bilateral transverse venous sinus stenosis (TVSS) was present in 3.0% of 198 patients. Five patients (1.7%) had papilledema. Compared to patients without papilledema, those with papilledema had significantly higher BMI and prior history of IIH, and increased prevalence of empty sella, optic nerve tortuosity, and TVSS on MRI. The prevalence of papilledema increased from 2.8% among patients with at least one MRI-IH to 40% among patients with four or more MRI-IH.
Conclusion | MRI-IH are common in patients undergoing brain MRI, but rarely associated with papilledema. The management of patients with incidentally detected MRI-IH likely does not require systematic lumbar puncture unless concerning symptoms or papilledema are present.
68 13:58 | Young Investigator Award Presentation The Quest to Reduce Stroke Treatment Delays at A Melbourne Metropolitan Primary Stroke Centre over the Last Two Decades
Peter SW Park1, 2, Tanya Frost1, Peter SY Tan1, Joseph Wong1, Alun Pope2, Helen M Dewey1, 2, Philip MC Choi1, 2
1. Department of Neurosciences, Box Hill Hospital, Eastern Health, Box Hill, VIC, Australia 2. Eastern Health Clinical School, Faculty of Medicine, Nursing and Health Sciences, Box Hill, VIC, Australia
Objectives | To examine door-to-needle time (DNT) trends and the impact of continuous quality improvement initiatives since thrombolysis became standard of care in Australian metropolitan setting for eligible acute ischaemic stroke patients.
Methods | Single-centre retrospective cohort study of consecutive patients treated with thrombolysis at high volume primary stroke centre from January 2003 to December 2019. Primary outcomes are DNT, and annual number of ‘Code Stroke’ activations and stroke admissions. Regression modelling for potential factors associated with DNT.
Results | 1,250 patients were treated with thrombolysis over 17 years; 54% were male with a median age of 76 (interquartile ranges [IQR], 66–83). Median DNT fluctuated between 70 to 93 minutes (IQR, 55–95 to 82–120) from 2003 to 2012, reaching 60 minutes in 2013 and nadir of 47 minutes in 2014. Median DNT then decreased from 58 minutes in 2015 to 51 minutes in 2019 with progressive tightening of IQR (46–78 to 40–62). Number of patients treated within 60 minutes of hospital arrival was less than 30% between 2003-2012. This rose to an average of 63% during 2015–2018 and 71% in 2019. From 2015 to 2019, per annum number of ‘Code Stroke’ activations increased from 940 to 1300 while stroke admissions plateaued at 750. ‘Direct-to-CT’ protocol and acute stroke presence were two modifiable workflow factors independently associated with faster DNT (P<0.001).
Conclusion | Targeted quality improvement initiatives are key to reducing treatment delays in the Australian metropolitan setting. Relative stagnation in DNT improvement is concerning and needs further investigation.
14:05 | Young Investigator Award Presentation Cognitive impairment in late onset epilepsy
Xin Zhang1, Armin Nikpour1, Rebekah Ahmed1
1. Royal Prince Alfred Hospital, Camperdown, NSW, Australia
Objective | Late onset, unprovoked epilepsy patients with cognitive impairment can have complex pathophysiology.1 Our objective was to study the characteristics and contributors of cognitive impairment in this group; and how patients with dementia could be differentiated from late onset epilepsy patients.
Methods | Twenty-six patients with epilepsy, onset after 50 years of age, with new cognitive complaints and 26 patients with clinically diagnosed Alzheimer’s Disease (AD) were recruited. These participants had comprehensive neuropsychological and neuroimaging assessments. A subset of 17 participants from the Epilepsy group underwent longitudinal neuropsychological assessment.
Results | In the Epilepsy group, the neuropsychological profile of cognitive impairment was consistent with the foci and severity of seizure activity in 46% of participants; subcortical microvascular change in 15%; mood disturbance in 15%; medication in 15%; alcohol in 4% and AD in 4%. Compared with the Epilepsy group, the AD group had a lower Addenbrookes Cognitive Examination III (ACE-III) score (79.3±10.8 versus 87.5±6.5, p=0.01); specifically in the attention, memory and visuospatial subdomains (p=0.004, p=0.002 and p=0.02) but not fluency and language subdomains p( >0.05); and lower scores on additional assessments of naming, visuospatial and executive function (p≤ 0.001). The AD group had more abnormal metabolism in the temporal, parietal and occipital lobes than the Epilepsy group (p=0.02, p=0.006 and p=0.005).
69 Conclusion | Patients with late onset epilepsy and cognitive complaints rarely have dementia diagnosed at their first neuropsychological assessment and tend to have milder cognitive impairment than patients with AD. The two groups can be differentiated by their neuropsychological and FDG-PET profiles.
1. Sen A, Capelli V, Husain M. Cognition and dementia in older patients with epilepsy. Brain. 2018;141(6):1592-1608.
14:30 | Leonard Cox Award 2021 | The Young Achievers The Genetics of Focal Epilepsies: From Research to Clinical Practice
Piero Perucca
Focal epilepsies have traditionally been regarded as largely ‘acquired’ disorders due to environmental factors, such as stroke, head trauma or brain infections. In reality, accumulating data point to important genetic contributions to this group of epilepsies. In this lecture, I present the different lines of evidence supporting the major role of genetics in the pathogenesis of focal epilepsies and discuss how genetic discoveries are making their way in the routine management of these patients.
70 Friday 21 May 2021
09:30 – 12:30 The Borderlands Chairs: Rob Henderson and Alex Lehn Symposium sponsored by Bristol Myers Squibb
09:30 Putting the Bio into Psycho and Management Pearls Mark Edwards (UK)
10:00 Q&A time Mark Edwards
10:10 Psychogenic non epileptic seizures Hanka Laue Gizzi
10:25 Functional Visual Disorders Neil Shuey
10:40 Clinical Differentiation Interactive Video Session Andrew Bleasel, Barry Snow, Victor Fung
11:20 Q&A All Speakers
11:30 Mervyn Eadie Award Mark Cook Seizures and Cycles: Patterns and Prediction
12:00 Clinical Pearls in Neuroinfectious Diseases Joshua Davis
12:20 Q&A time Joshua Davis
12:30 – 13:30 Lunch Break Poster Viewing Session 3 Sponsored by Eisai
13:00 – 15:00 Pupils, Pressure and Pandemics Chairs: Pamela McCombe and Jim Burrow Symposium sponsored by Teva
13:30 EG Robertson Lecture Christian Lueck The Optic Chiasm: Studies in Architecture and Neurology
14:00 Raised intracerebral pressure for Neurologists Ben Jonker
14:20 Q&A time Ben Jonker
14:30 – 15:00 Best Case Reports 2020 – 2021
14:30 1908 Spinal cord presentation of biopsy-proven PET-positive Grace Swart giant cell arteritis
14:33 1963 Pembrolizumab induced Lambert-Eaton Myasthenic Jasmine Ashhurst Syndrome
14:36 1997 Hemi-cord infarction following vertebral artery Alanna Rottler dissection in a patient with congenital hypoplastic vertebral artery: A case report
14:39 2017 Ganglioside antibodies, meningoencephalitis and Xin Zhang longitudinally extensive transverse myelitis: GAME-LETM
71 14:42 2030 A persistent false familiarity: recollective confabulation Xin Zhang presenting in a patient describing a life of ‘total instant recall’
14:45 2036 An Unexpected Cause of Ophthalmoplegia and Areflexia: Jessica Qiu The Pupils Have It
14:48 2062 Intraventricular migration of intraocular silicone oil Leon Edwards
14:51 2091 Clinical and neurophysiological improvement in James Triplett Hereditary sensory and autonomic neuropathy type I (HSAN-1) following high dose serine therapy
14:54 2076 Embouchure dystonia complicating a case of focal Ariadna Fontes-Villalba cerebral vasculitis
14:57 2031 Fulminant ADEM mimicking a glial tumour Jessica Qiu
15:00 Emerging Causes of Brain Infections Tom Solomon (UK)
15:20 – 16:00 Awards and Meeting Close
72 Friday 21 May 2021
The Borderlands Chairs: Rob Henderson and Alex Lehn
Symposium sponsored by Bristol Myers Squibb
09:30 | The Borderlands Putting the Bio into Psycho and Management Pearls
Mark Edwards
10:10 | The Borderlands Psychogenic non epileptic seizures
Hanka Laue Gizzi
Psychogenic non-epileptic seizures (PNES) are a common Functional Neurological Disorder (FND) subtype. The average delay from first symptom to diagnosis is over 7 years, often leading to unnecessary antiepileptic medication trials, hospital admissions and delayed psychiatric treatment. Neurologists play a crucial role in establishing an accurate diagnosis early, communicating the diagnosis to the patient and supporting the transition into psychiatric care. This talk will concentrate on the diagnostic process and the tools available.
10:25 | The Borderlands Functional Visual Disorders
Neil Shuey
Functional visual loss is demonstrated when patients have vision loss that is not consistent with an organic disorder, and represents an example of the broader categories of Functional Neurological Disorder and Conversion Disorder. This condition is often difficult to diagnose and manage, particularly when it occurs in the context of organic disease (eg Idiopathic Intracranial Hypertension, other ophthalmic or neurological disorders). Immediate clues that can prove helpful include the “Sunglasses sign” (the wearing of sunglasses indoors, in the absence of an ocular disease to account for this) which has a high specificity for non-organic vision loss. History taking may reveal other functional neurological or systemic symptoms, and sometimes a long trail of negative investigations, questionable diagnoses, and patients or relatives disputing test results or medical judgments.
Examination should ensure that “pitfall” organic diagnoses are not missed, and then include tests for functional vision loss. These fall into 3 categories:
1. Behavior observed that is not compatible with blindness a. Eg Easy entrance into consulting room 2. Failing to succeed at tasks that are independent of vision
73 a. Eg Proprioceptive tasks 3. Non-anatomical or non-physiological patterns of vision loss a. Eg Tubular visual fields
It is also important to demonstrate that vision is better than claimed, for example the patient with an organic constricted visual field is unable to accurately make a saccade into the blind field area, whereas in functional field constriction this may be normal.
Strategies for dealing with functional vision loss include the important step of direct communication with other health care providers (especially mental health professionals) in order to prevent unnecessary investigations and procedures, and hopefully increase the chances of a positive outcome.
Functional eye movement disorders can also be difficult to diagnose. These include voluntary nystagmus (which is often mistaken for oscillopsia or ocular flutter), convergence spasm, and pseudoptosis.
10:40 | The Borderlands Clinical Differentiation Interactive Video Session
Andrew Bleasel, Barry Snow, Victor Fung
11:30 | Mervyn Eadie Award | The Borderlands Seizures and Cycles: Patterns and Prediction
Mark Cook
Remarkable insights into the patterns underlying seizure activity have been provided by data acquired in recent years from long-term intracranial monitoring devices. The recognition of marked disparities in the ability of patients to accurately identify seizures has very significant implications for management, assessment of new therapies, and safety. Intracranial recordings from devices intended to predict seizures demonstrated not only that this was possible, but as well that striking patterns could be recognised that provided new approaches to the problem of seizure prediction. Our work with wearables and minimally invasive implantable seizure detection systems has confirmed that these cycles are not simply features of drug-resistant epilepsies, but found in the majority of patients with epilepsy, who showed circadian (24-hour) modulation of their seizure rates, as well as strong circaseptan (weekly) rhythms, and that cycles longer than 3 weeks were also highly prevalent. The causes of multi-scale variation in seizure rates are yet to be comprehensively explored, but are likely to include a range of environmental and endogenous factors. Detecting multi-scale oscillations in seizure rate may provide new approaches to treatment decisions and the interpretation of drug trials. Knowledge of these cycles can also be used to develop patient specific forecasting algorithms. Capturing accurate information about seizure frequency is clearly critical, but difficult to achieve in practice. We have subsequently developed a minimally invasive seizure detection system, currently being trialled in a first-in-man study. The use of these devices, and the implications of cycle recognition for clinical practice and epilepsy management will change the way we currently manage epilepsy.
12:00 | The Borderlands Clinical Pearls in Neuroinfectious Diseases
Joshua Davis
I will present and discuss ten “pearls” that relate to clinical practice, infectious diseases and neurology. This will include general infections of interest to practicing neurologists rather than only CNS infections. For each of the ten areas discussed, I will emphasise issues that arise from clinical practice and may not be found in textbooks, and touch on recent relevant research findings as well as highlighting current evidence gaps.
74 Pupils, Pressure and Pandemics Chairs: Pamela McCombe and Jim Burrow
Symposium sponsored by Teva
13:30 | EG Robertson Lecture | Pupils, Pressure and Pandemics The Optic Chiasm: Studies in Architecture and Neurology
Christian Lueck
14:00 | Pupils, Pressure and Pandemics Raised intracerebral pressure for Neurologists
Ben Jonker
14:30 | Pupils, Pressure and Pandemics Best Case Reports 2020 – 2021
14:30 | 1908 Spinal cord presentation of biopsy-proven PET-positive giant cell arteritis
Grace Swart
See Poster Abstracts, page 84
14:33 | 1963 Pembrolizumab induced Lambert-Eaton Myasthenic Syndrome
Jasmine Ashhurst
See Poster Abstracts, page 99
14:36 | 1997 Hemi-cord infarction following vertebral artery dissection in a patient with congenital hypoplastic vertebral artery: A case report
Alanna Rottler
See Poster Abstracts, page 110
75 14:39 | 2017 Ganglioside antibodies, meningoencephalitis and longitudinally extensive transverse myelitis: GAME-LETM
Xin Zhang
See Poster Abstracts, page 116
14:42 | 2030 A persistent false familiarity: recollective confabulation presenting in a patient describing a life of ‘total instant recall’
Xin Zhang
See Poster Abstracts, page 122
14:45 | 2036 An Unexpected Cause of Ophthalmoplegia and Areflexia: The Pupils Have It
Jessica Qiu
See Poster Abstracts, page 125
14:48 | 2062 Intraventricular migration of intraocular silicone oil
Leon Edwards
See Poster Abstracts, page 131
14:51 | 2091 Clinical and neurophysiological improvement in Hereditary sensory and autonomic neuropathy type I (HSAN-1) following high dose serine therapy
James Triplett
See Poster Abstracts, page 143
14:54 | 2076 Embouchure dystonia complicating a case of focal cerebral vasculitis
Ariadna Fontes-Villalba
See Poster Abstracts, page 135
14:57 | 2031 Fulminant ADEM mimicking a glial tumour
Jessica Qiu
See Poster Abstracts, page 122
76 15:00 | Pupils, Pressure and Pandemics Emerging Causes of Brain Infections
Tom Solomon
Over the last 18 months, the threat of emerging infections has become a startling and devastating reality for the whole world. In Liverpool we have been studying emerging infections, particularly those that infect the brain, for the last 25 years. In this brief talk we review some of the most important recent findings from the group’s work in three areas. From the Brain Infections UK Programme, we review the latest data on viral meningitis. This is now numerically more important than bacterial disease, as a consequence of vaccines against bacteria, and can have important long-term effects. From the Brain Infections Global Programme, we consider recent work on neurological disease caused by the arboviruses Zika and Chikungunya in Brazil. The former is associated with peripheral nervous system disease, especially Guillain-Barre syndrome, whilst the latter is linked to central nervous system disease, especially myelitis. From the National Institute for Health Research (NIHR) Health Protection Research Unit on Emerging and Zoonotic Infections we review recent data from the ISARIC-4C study, which shows the importance of neurological disease in Covid-19. We share unpublished individual patient data from the Global Covid-Neuro Network comparing the two most important neurological complications in Covid-19, encephalopathy and stroke. Finally, we report our early findings on vaccine-induced thrombosis and thrombocytopenia following Covid-19 vaccines, just published in the New England Journal of Medicine.
77 Poster Presentations
Sponsored by
1888 A unique New Zealand case of Generalised Cutaneous dysesthesia and review of the literature
Eileen J Mc Manus1, Peter PW Wright 1, Amanda AO Oakley1
1. WDHB, Hamilton, Waikato, New Zealand
We describe a rare case of distressing generalized cutaneous dysaesthesia in a patient with hypertrophic lichen planus in which neurological evaluation was normal. The dysaesthesia markedly improved with alcohol and clonazepam. We speculate that the response to alcohol and clonazepam suggests a role for GABAergic neuromodulation. We also include a review of the existing literature regarding cutaneous dysaesthesia. Most existing therapies for cutaneous dysaesthesia are symptomatic, with our case supporting a role for pharmacological modulators of GABAergic neurotransmission. Our report highlights that early recognition and treatment of cutaneous dysaesthesia is necessary to avoid negative repercussions on function and quality of life.
1889 Prevalence of hyperammonemia in patients on Valproic Acid therapy in tertiary care hospital of Karachi
Iqra Ali1
1. Ziauddin Hospital, Karachi, Pakistan
Objective | To determine the frequency of hyperammonemia in patients on valproic acid therapy in a tertiary care hospital of Karachi.
Subject and Methods | 158 patients who fulfilled the inclusion criteria were included in the study. A written informed consent was taken from patients before enrolling them into study. Blood samples were collected in sterilized syringes to test for valproic acid levels (predose) if on sodium valproate therapy, platelet count, total bilirubin, direct billirubin, alanine transaminase and gamma glutamyl transferase under the supervision of consultant Neurologist having more than 5 years of experience. Demographic data including name, age, sex and study variable i.e. hyperammonemia was noted into the predesigned proforma attached. Confounding variables and biasness was controlled by strictly following inclusion and exclusion criteria.
Results | Mean age of patients was 55.53±18.26 (18-80) years. Mean serum ammonia level was 60.56±44.59 (15-240) (mg/day). Mean dose of valproic acid was 1000±125 (mg/day). Out of total 77(48.7%) patients were male and 81(51.3%) were female. In this study 67(57.6%) patients were diabetics and 91(57.6%) patients had hypertension. Hyperammonemia was diagnosed in 81 patients (51.3%) patients on VPA therapy.
Conclusion | The use of valproic acid in patients was associated with a dose-dependent increase in blood concentrations of ammonia. Combination treatment with liver enzyme-inducing antiepileptic drugs and antipsychotic drugs increases the risk of VPA-induced hyperammonemia. Most of the patients with VPA-induced hyperammonemia were asymptomatic; however, if patients taking VPA
78 present with symptoms such as nausea, fatigue, somnolence, ataxia, and altered mentation, the blood ammonia level should be measured.
1. Sattar Y, Merotto B, Dedousis A, Aadil M, Zil-E-Ali A. Valproic Acid-Induced Hyperammonemia with Encephalopathy (VIHE): A Case Report. J Med Res Innov.2018;2(1):e000108. 2. Baddour E, Tewksbury A, Stauner N. Valproic acid–induced hyperammonemia: Incidence, clinical significance, and treatment management. Ment Health Psychiatry.2018;8(2):73-7. 3. Aiyer R, Seide M, Stern RG. Valproic acid induced hyperammonemia in a long time treated patient. Case Rep Psychiatry.2016;2016:6242314. 4. Cattaneo CI, Ressico F, Valsesia R, D’Innella P, Ballabio M, Fornaro M. Sudden valproate-induced hyperammonemia managed with L-carnitine in a medically healthy bipolar patient: essential review of the literature and case report. Medicine (Baltimore).2017;96(39):e8117 5. Tesen H, Katsuki A, Hori H, Atake K, Yoshimura R, Nakamura J. Plasma ammonia levels in patients treated with valproic acid. Clin Neurosci.2017;7(1):42-7. 6. Maldonado C, Guevara N, Silveira A, Fagiolino P, Vázquez M. L-Carnitine supplementation to reverse hyperammonemia in a patient undergoing chronic valproic acid treatment: a case report. Br J Med Med Res.2017;45(3):1268-72 7. Bene J, HAdziev K, Melegh B. Role of carnitine and its derivations in the development and management of type 2 debates. Nutr Diabetes. 2018 Mar 7;8(1):8 8. Longo N, Frigeni M, Pasquali M. Carnitine transport and fatty acid oxidation. BiochimBiophysActa Mol CellRes.2016;1863(10):2422-35. 9. Marx W, Teleni L, Opie RS .Efficiancy and effectivenessof carnitine supplementation for cancer related fatigue: a systematic literature review and Met-analysis. Nutrients.2017;9(11):1224 10. Song X, Qu H, Yang Z ,Rong Jet . Efficacy safety of L-cranitine treatment for chronic heart failure: a Met-analysis of randomized controlled trials. Biomed Res Int.2017:1-11 11. Baddour E, Tewksbury A, Stauner N. Valproic acid–induced hyperammonemia: Incidence, clinical significance, and treatment management. Ment Health Clin.2018;8(2):73-7.
1892 A case of isolated musculocutaneous nerve injury following skydiving simulation
Oshi Swarup1, Belinda Cruse1, Daniela Leupold1, John King1
1. Department of Neurology, Royal Melbourne Hospital, Parkville, Melbourne, VIC, Australia
Background | Isolated musculocutaneous nerve injuries are rare, and mostly iatrogenic or traumatic.
Case Presentation | We present a case of isolated musculocutaneous neurapraxia in an otherwise well young woman following uncomplicated simulated skydiving.
Management and Outcome | While the injury was quite debilitating, complete neurological recovery occurred within two months without any intervention.
Discussion | This case illustrates a rare pattern of neurological injury, caused by a recreational activity growing in popularity. The pattern of injury mimics that of an upper trunk brachial plexopathy or C5/6 radiculopathy. Increased awareness of the injury avoids misdiagnosis and affords the opportunity for prevention.
1893 Neurological manifestations in Rheumatological disease: a case series I
Eileen J Mc Manus1, Douglas White1, Alan Doube1, Jan Schepel1, Matthew Phillips1, Kamal Solanki1
1. WDHB, Hamilton, Waikato, New Zealand
Rheumatology encompasses a broad range of multisystemic, autoimmune and inflammatory disorders. Neurological manifestations of these diseases are not uncommon. Neurological findings may predate rheumatological findings or may emerge months to years post initial diagnosis. Rheumatological diseases presenting as neurological syndromes can cause diagnostic challenges. We present a
79 range of rheumatological cases with unusual neurological presentations that demonstrate this point including; C2-C3 facet arthropathy in Diffuse Scleroderma, Granulomatosis with polyangiitis manifesting with craniofacial involvement, pseudo vasculitis associated cerebrovascular events, SAPHO syndrome with a thoracic syrinx, Neuro- Bechet’s vasculitis with tumour-like CNS lesions, Platybasia in Paget’s disease and others. Familiarity with the neurological manifestations of rheumatologic diseases is important for both rapid diagnosis and appropriate intervention.
1894 Extended Interval Dosing Natalizumab infusion and impact on neuropsychological deficits in Relapsing-Remitting Multiple Sclerosis
Eileen J Mc Manus1, Karen M Clark1, Jamie A.B Macniven1, Jan Schepel1
1. WDHB, Hamilton, Waikato, New Zealand
Background | Cognitive impairment and neuropsychiatric symptoms are frequently reported in Relapsing-Remitting Multiple Sclerosis (RRMS). Natalizumab (NTZ) is a humanised monoclonal antibody to α4β1 subunit of VLA4 on leucocytes. It is usually administered on a 4-weekly schedule. However, Extended Interval Dosing (EID) at 6-8 weekly intervals has been proven non-inferior regarding relapse risk, with the benefit of a lower risk of Progressive Multifocal Leukoencephalopathy (PML). The impact of EID Natalizumab on neuropsychological deficits in RRMS has not been studied.
Objective | To determine if 6 weekly NTZ demonstrates an improvement in neuropsychological parameters in RRMS patients.
Method | We performed a retrospective analysis of 34 RRMS patients treated between August 2015- 2017. Patients underwent baseline neuropsychological testing before commencing NTZ EID. A second evaluation was performed, on average 28 months after commencing treatment.
Results | Z scores at the initial assessment showed baseline cognitive impairment in multiple domains in particular; Attention and Abstraction, Executive functioning, Short Term Memory. 14/20 Z-scores showed an improvement post-NTZ and 5/14 reached statistical significance; namely Trails A (visual attention/processing speed), Line-orientation (visual-spatial), Picture-naming (word finding), Digital-Span (attention, executive function and memory) and Story-recall (memory). HADS data remained unchanged. Correlation matrix showed no association between HADS scores, the time between assessments and the changes in Z scores.
Conclusion | This data suggests the efficacy of NTX EID in improving cognitive impairment in RRMS. A prospective observational study is warranted.
1895 Metabolic syndrome in a New Zealand Glioblastoma cohort 2005- 2020: A retrospective analysis and review of the literature
Eileen J Mc Manus1, Christopher Frampton1, 2, Alvin Tan1, Matthew Phillips1
1. WDHB, Hamilton, Waikato, New Zealand 2. Department of Medicine, University of Otago, Christchurch, New Zealand
Background | Glioblastoma (GBM) is an aggressive form of glioma. Even with standard-of-care Stupp protocol (surgery, radiotherapy, and temozolomide), median overall survival is only 10-12 months in population-based studies. Metabolic reprogramming is a hallmark of glioblastoma, with energy metabolism aberrantly geared towards aerobic fermentation. The prevalence of metabolic syndrome is 16% in the general NZ population and 32% in the Maori population.
Objectives | We aimed to determine 1) if metabolic syndrome was more prevalent in our GBM cohort compared to general NZ population 2) if metabolic syndrome was associated with worse overall survival in GBM 3) if ethnicity influenced survival outcomes.
80 Methods | We performed a retrospective analysis of 170 patients diagnosed and treated for GBM between 2005-2020 in one institution. Clinical and biochemical data relating to metabolic syndrome were collected. Overall survival was determined from the date of initial a surgical diagnosis to the date of death or data acquisition.
Results | 18.2% of GBM patients met the criteria for metabolic syndrome, 27.7% of Maori and 16.1% of European New Zealanders. Patients with metabolic syndrome had statistically significant worse overall survival compared to those patients without metabolic syndrome regardless of treatment [mean 9.7 vs 18.4 months] p= 0.016 (p≤0.05). Power was too low to comment on the prevalence of metabolic syndrome or ethnicity.
Conclusion | Our study demonstrates that metabolic syndrome is associated with statistically significant poorer outcome in GBM patients. Consequently, this data will provide a control group for our current prospective study investigating the antineoplastic effects of metabolic therapies in GBM.
1898 Multiple Cranial Neuropathies In A Patient With Syphilitic Meningitis
Melissa Chu1, Shejil Kumar1, Jonathan Sturm1, 2
1. Gosford Hospital, Gosford, NSW, Australia 2. Neurology, University of Newcastle, Newcastle, NSW, Australia
Syphilis is increasing in prevalence in the community [1, 2]. Neurosyphilis has protean manifestations making recognition, diagnosis and early initiation of treatment challenging. We present a case of early syphilitic meningitis in a 37-year-old female presenting with multiple cranial neuropathies (V, VI, VII, VIII and XII) developing over the course of two weeks. This began with a sensation of disequilibrium and unsteady gait, and progressed to difficulty closing both eyes, right lip numbness, bilateral hearing impairment (right, followed by left), and dysarthria. She did not report headache, meningism, features of primary syphilis infection or risk factors for sexually transmitted infections (STIs). Examination confirmed the presence of right-sided trigeminal, bilateral abducens, facial, vestibulocochlear and hypoglossal nerve palsies. Cerebrospinal fluid (CSF) examination was inflammatory (protein 1.28 g/L, glucose 3.8mmol/L) with predominant lymphocytosis (76%, WCC 441 x 106/L). Magnetic resonance imaging (MRI) demonstrated post-contrast enhancement of the trigeminal nerve at the pons, as well as facial and vestibulocochlear nerves at the geniculate ganglion with no leptomeningeal enhancement. Our patient was diagnosed with neurosyphilis on serum and CSF serological testing (Serum Treponema pallidum particle agglutination assay (TPPA) positive, chemiluminescent microparticle immunoassay (CMIA) IgG and IgM positive, rapid plasma reagen 1:32. CSF TPPA positive, Venereal Disease Research Laboratory test titre of 1:8). She was treated with intravenous benzylpenicillin with rapid improvement in her cranial neuropathies. This is the most extensive cranial neuropathy reported with syphilitic infections to date. Neurosyphilis should be considered as a differential in patients presenting with multiple cranial neuropathies.
1. Kojima N, Klausner J. An Update on the Global Epidemiology of Syphilis. Curr Epidemiol Rep 2018;5(1):24-38. 2. Ghanem K, Ram S, Rice P. The Modern Epidemic of Syphilis. N Engl J Med 2020;382(24):2379-80.
1899 MR-based intramuscular fat fraction assessment in hereditary sensory neuropathy type 1
Stephanie L Barnes1, Michael V Chan2, Stephen Morris2, Garth A Nicholson1, 3, 4
1. Department of Neurology, Concord Repatriation General Hospital, Concord, NSW, Australia 2. Department of Radiology, Concord Repatriation General Hospital, Concord, NSW, Australia 3. ANZAC Research Institute, Concord, NSW, Australia 4. University of Sydney, Sydney, NSW, Australia
Objectives | Hereditary sensory neuropathy type 1 (HSN1) is a rare progressive neuropathy characterised by profound sensory dysfunction, often accompanied by significant weakness. Muscle
81 magnetic resonance (MR) imaging with determination of intramuscular fat accumulation has been proposed as a marker of progression in this condition and we aimed to investigate this further.
Methods | Calf-level muscle MR images were acquired longitudinally over three years in patients with genetically confirmed HSN1 due toSPTLC1 and SPTLC2 mutations. These patients were part of a larger trial of L-serine supplementation as a candidate therapy and MR images were acquired at baseline and while on treatment. Individual muscles and muscle groups were manually segmented at two cross-sectional levels in the proximal calf. Intramuscular fat accumulation and muscle atrophy were assessed.
Results | Detailed MR analysis was performed in a preliminary series of three patients. We demonstrated an average annual change in MR-based intramuscular calf fat fraction of 3.2%, 1.0% and 3.7% at a cross-sectional level 130mm below the tibial plateau and 2.0%, 0.6% and 1.2% at a cross- sectional level 25% of the tibial plateau-medial malleolus distance. The degree of muscle atrophy did not significantly change. There appeared to be ongoing progression of disease during this short duration of L-serine supplementation.
Conclusion | MR-based intramuscular calf fat fraction can be used to monitor progression in HSN1 and has potential utility in clinical trials. Technical limitations to this technique may be overcome using volumetric imaging with automated muscle segmentation in the future. Further investigation of L-serine supplementation is required.
1902 Rare late onset neutropenia in a patient with multiple sclerosis treated with ocrelizumab and review of literature
Babar Malik1, Shoaib Dal1, Robert McGrath1
1. Department of Neurology, Wollongong Hospital, Wollongong, NSW, Australia
Introduction | Ocrelizumab is a recombinant humanised monoclonal antibody that selectively depletes CD20 expressing B lymphocytes. Ocrelizumab was approved in Australia for use in relapsing- remitting and primary-progressive multiple sclerosis in July 2017. Rare incidences of late onset neutropenia with ocrelizumab have been reported (3 cases worldwide, none in Australia).
Case | A 40-year-old man of Lebanese descent with multiple sclerosis, diagnosed 11 years ago and stabilized with 600 mg 6-monthly ocrelizumab since March 2018, presented in November 2019 with lethargy and myalgia, two weeks after his fourth cycle of ocrelizumab. Clinical examination was unremarkable. Full blood count showed white cell count of 2.35x109/L and absolute neutrophil count of 0.1x109/L with normal lymphocyte count, peripheral blood film, haemoglobin and platelet count. Serum iron studies, B12, folate, C-reactive protein, thyroid function were normal and so was the chest x-ray. The urine microscopy showed sterile pyuria. Electrolytes, liver function and renal function were normal. The nasopharyngeal viral swabs were negative. MRI brain-cervical spine showed stable old plaques and no enhancement with contrast. He had no other co-morbid condition and was not taking any other prescribed or over-the-counter medications. The neutrophil count improved to 7.68x109/L in 48 hours after Filgrastim 400 mcg subcutaneously. Ocrelizumab was ceased.
Conclusion | Late onset severe neutropenia is rarely reported with ocrelizumab. In our case, the neutropenia occurred after 1.5 years of ocrelizumab use and the mechanism remains unclear. The case raises the issue of ocrelizumab re-exposure vs cessation in these patients and highlights the importance of monitoring of serial blood count.
82 1905 The indications and utility of electroencephalogram, an audit of hospital practices
Nicholas F Halliwell1, Lucy Zhang1, Stephan Golja1, Ellie Skacel1, Renee Pope1, Callan A Coventry1, Chinthuran Thilagarajan1, Georgie CJ Dixson1, Anna Schutz1
1. Neurology, Gosford Hospital, Gosford, NSW, Australia
Objectives | The MBS taskforce recommends discouraging use of electroencephalogram (EEG) investigations for low yield indications without neurological specialty input. This study aimed to examine the indications and utility of electroencephalogram within a hospital setting.
Methods | A retrospective audit was undertaken over a 3 month period across two hospitals of adult inpatient EEGs ordered by services other than neurology.
Results | Data was collected on 236 EEG encounters. 11% of EEGs performed had a definitive diagnosis of seizures on discharge, of these EEGs the report documented; a normal EEG in 7%, epileptiform activity in 19%, and non-specific slowing in 74%. 17% of Adult EEGs were performed for low yield indications. None of these EEGs resulted in change of management nor a diagnosis of seizures. An additional 14% were performed as part of a “falls work up,” none of the EEGs for this indication resulted in a diagnosis of seizure on the discharge summary.
Conclusion | This audit supports previous findings that EEGs have a low sensitivity and can not be exclusively used to attain a diagnosis. Low yield indications were common within this audit and the EEG was not clinically significant in this group. EEG should not be used to rule out seizures when the clinical suspicion for seizures is near zero and this audit identified an additional low yield category within the hospital setting as part of a “falls work up.” This study supports the conservative use of EEG in line with the MBS funding taskforce protocol.
1906 Under PRESsure – An unusual trigger of posterior reversible encephalopathy
James O Thomas1, David B Williams1, Christopher Grainge1
1. John Hunter Hospital, New Lambton Heights, NSW, Australia
A 68-year-old female, and experienced recreational diver, presented via a regional hospital to our neurology service with acute onset visual disturbance following a 30-metre open water dive.
She had no significant past medical history, specifically no history of hypertension and was on no regular medications. She was an ex-smother with a 20 pack year history
On arrival she was hypertensive to 190/100mmHg but otherwise systemically stable. She had intact visual acuity and visual fields but had difficulty distinguishing objects from complex backgrounds and described vivid visual distortions.
MRI performed acutely demonstrated multifocal areas of T2 hyperintensity within her posterior parietal and occipital lobes and a provisional diagnosis of posterior reversible encephalopathy syndrome (PRES) was made.
Further questioning revealed that the patient was diving with a 70/30% nitrogen/oxygen mixture for which she had not received appropriate training, and was diving at depths close to the recommended limits for this mixture. Diving mixtures containing higher concentrations of oxygen are used to reduce the risk of nitrogen narcosis and decompression sickness but can be associated with CNS toxicity thought to be due to significantly increased PaO2 and associated cerebral vasoconstriction.
83 The patient was managed with intensive blood pressure control and made a significant recovery within seven days. We postulate that her PRES may have been triggered by excessive cerebral vasoconstriction from hyperoxaemia in a patient with impaired vasoregulatory reserve.
1. Wingelaar TT, van Ooij P-JAM and van Hulst RA (2017) Oxygen Toxicity and Special Operations Forces Diving: Hidden and Dangerous. Front. Psychol. 8:1263. doi: 10.3389/fpsyg.2017.01263 2. Matsuo R, Kamouchi M, Arakawa S, Furuta Y, Kanazawa Y, Kitazono T. Magnetic resonance imaging in breath- hold divers with cerebral decompression sickness. Case Rep Neurol. 2014;6(1):23-27. Published 2014 Jan 24. doi:10.1159/000357169
1908 Spinal cord presentation of biopsy-proven PET-positive giant cell arteritis
Grace Swart1, Sapna Balgobind1, Charles Chan1, Michael Fulham2, Sean Riminton1, Emma Mitchell3, Stephen Reddel1
1. Neurology, Concord Repatriation Hospital, Sydney, NSW, Australia 2. Royal Prince Alfred Hospital, Camperdown, NSW, Australia 3. Rheumatology, Hermitage Medical Centre, Port Macquarie, NSW, Australia
Background/Objectives | We report a spinal cord syndrome in a biopsy-proven and PET-positive giant cell arteritis (GCA) – a rare manifestation of GCA.
Methods/Results | A 63-year-old man presented with sudden onset weakness of his legs followed a week later by decreased pain and temperature sensation in his right trunk and leg. Two months prior a rheumatologist had diagnosed him with polymyalgia rheumatica/giant cell arteritis and commenced a weaning prednisone course due to proximal limb-girdle pain, morning stiffness and headaches and ESR=30. An initial temporal artery biopsy had been negative.
Neurologic examination revealed lower limb hypertonia, mild bilateral pyramidal weakness, hyperreflexia and positive right Babinski. A right-sided T8 sensory level to pain/temperature was present. Admission ESR was 17 and C-reactive protein was 9. An MRI whole spine was unremarkable, however somatosensory evoked potentials revealed central conduction delay below the cervical cord.
Vasculitis with partial anterior spinal cord infarction was suspected and intravenous methylprednisolone commenced. PET-CT revealed vertebral, femoral and popliteal artery hypermetabolism. Duplex ultrasonography of the remaining right temporal artery revealed a hypoechoic halo consistent with arteritis. Directed temporal artery biopsy was diagnostic of giant cell arteritis. The patient was commenced on cyclophosphamide given major organ involvement and resistance to prednisone. On follow up two months later his symptoms remained stable.
Conclusion | GCA can extremely rarely present with spinal cord involvement, which is associated with high mortality and therapeutic challenges. False-negative biopsies can occur and investigations including PET/CT, temporal artery ultrasonography and even repeat biopsy can be helpful in confirming the diagnosis.
1916 Presence of VGCC antibodies with possible late onset multiple acyl- CoA dehydrogenase deficiency
Po Sheng Yang1, Viral Upadhyay1, Thomas Robertson2, Robert Boots3, Robert Henderson1
1. Neurology, Royal Brisbane and Women’s Hospital, Brisbane, QLD, Australia 2. Pathology of Queensland, Royal Brisbane and Women’s Hospital, Brisbane, QLD, Australia 3. Thoracic Medicine, Royal Brisbane and Women’s Hospital, Brisbane, QLD, Australia
Introduction | Voltage-gated calcium channel (VGCC) antibodies are considered specific for Lambert- Eaton myasthenic syndrome (LEMS). However, VGCC antibodies have been reported in other groups of patients without LEMS.
84 Multiple acyl-CoA dehydrogenase deficiency (MADD) is a type of mitochondrial myopathy which can present with a late onset form.
We report a case of a patient with positive VGCC antibodies concurrently with a diagnosis of possible late onset MADD.
Methods | Clinical information and results of investigations were obtained.
Results | A 75 year old woman presented with a gradual onset of proximal weakness associated with dyspnoea.
There was no relevant past medical history and family history was unremarkable.
Apart from mild proximal weakness, rest of the neurological examination was unremarkable. Deep tendon reflexes were normal.
Acylcarnitine profile on multiple occasions showed a pattern consistent with MADD. Muscle biopsy showed mild mitochondrial changes.
VGCC antibodies were detected on 2 separate occasions (86 and 100 pM; Ref Range <30pM). CMAP amplitudes and repetitive stimulation was normal with no facilitation nor decrement found. Position emission tomography was unremarkable.
The patient’s symptoms were thought to be secondary to MADD, therefore was treated with riboflavin, Q10 and carnitine and described significantly improved proximal strength and function.
Conclusion | It has been generally considered that the VGCC Ab has a high sensitivity and specificity for LEMS. However, in a study of 100 neuromuscular patients with elevated VGCC antibodies, only 6 patients were diagnosed with LEMS. This case illustrates the importance of applying appropriate clinical judgement with results of investigations.
1917 Ischaemic stroke as the only manifestation of anti-neutrophilic cytoplasmic autoantibody associated vasculitis
John Tran1, Joshua Abasszade2, Yew Li (Michelle) Dang1, Doug Crompton1
1. Northern Health, Melbourne, VIC, Australia 2. Monash Health, Melbourne, VIC, Australia
The diagnosis of anti-neutrophilic cytoplasmic autoantibodies (ANCA) associated vasculitis (AAV) in first episode strokes is more challenging compared with consecutive strokes, especially in patients lacking other clinical features of AAV. Here, we present the case of a 71-year-old female with positive myeloperoxidase (MPO) ANCA and negative proteinase 3 (PR3) ANCA. Our patient presented with a one-week history of pyramidal weakness in both upper and lower limbs, hyperreflexia and clonus. Brain MRI demonstrated widespread bi-hemispheric cortical and deep white matter acute infarcts. Investigations revealed eosinophilia on full blood examination and positive MPO-ANCA antibody. Consistent with features of stroke secondary to AAV, the deep penetrating vessels were predominantly affected resulting in a multifocal distribution of infarcts in the white matter. MPO-ANCA positive vasculitis diseases are more commonly associated with renal, pulmonary and cutaneous manifestations, however our patient did not have other systemic manifestations of AAV, and her presentation was solely limited to the CNS. This case highlights the challenges of diagnosing primary CNS vasculitis, especially an atypical MPO-ANCA positive disease that fails to have the classical clinical signs and course.
1918 Predictive Value of Signs and Symptoms in Code Strokes for Diagnosis of Ischaemic Stroke or TIA
Mei Yan Ngun1, Mark Tacey1, Douglas Crompton2
85 1. Austin Hospital, Heidelberg, VIC, Australia 2. Northern Health, Epping, VIC, Australia
Objectives | This study aimed to determine the history features, signs and symptoms identified during a code stroke that correlate with the diagnosis of ischemic stroke or transient ischemic attack (TIA). We investigated the rate of stroke mimics and aimed to develop a clinical prediction model.
Methods | Consecutive code stroke presentations to a primary stroke centre were recruited. Patient characteristics, medical history, signs or symptoms on activation of code stroke were collected from the medical record. Diagnosis of ischemic stroke was determined by radiographic evidence of infarction. Univariate analysis and multivariable logistic regression analysis were used to determine the features that predict ischemic stroke/TIA versus mimic.
Results | Among 493 code strokes, 64.5% were mimics. The most commonly diagnosed mimics were migraine, peripheral vertigo and seizure. Upper limb sensory change (OR 3.27 [95% CI, 1.75-6.11]), hemiplegia (OR 2.70 [95% CI, 1.65-4.43]), dysphasia (OR 2.62 [95% CI, 1.56-4.40]) and history of atrial fibrillation (OR 2.01 [95% CI, 1.14-3.54]) or hypertension (OR 1.77 [95% CI, 1.10-2.83]) are highly predictive of stroke/TIA. Headache (OR 0.40 [95% CI, 0.23-0.69]) is predictive of a mimic. Dizziness and vertigo were more common in stroke mimics. C-statistic for the study models ranged from 0.70 to 0.76.
Conclusion | Objective signs such as unilateral motor weakness and dysphasia are highly predictive of ischemic stroke/TIA whereas symptoms of headache and dizziness are suggestive of stroke mimic. Stroke mimic rate is influenced by local prevalence and threshold for code stroke activation. Incorporating positive and negative predictive features may improve future stroke prediction tools.
1921 Long-Term Reduction of Relapse Rate and 48-Week Confirmed Disability Progression After 6.5 Years of Ocrelizumab Treatment in Patients With RMS
Gavin Giovannoni1, John Parratt2, Ludwig Kappos3, Jerome De Seze4, Stephen Hauser5, James Overell6, Harold Koendgen6, Hans-Martin Schneble6, Kalpesh Prajapati7, Qing Wang6, Jerry S Wolinsky8
1. Centre for Neuroscience, Surgery and Trauma, Queen Mary University of London, London, UK 2. Neurology, North Shore Private Hospital, St Leonards, NSW, Australia 3. University Hospital Basel, University of Basel, Basel, Switzerland 4. University Hospital of Strasbourg, Strasburg, France 5. University of California, San Francisco, San Francisco, CA, USA 6. F. Hoffmann-La Roche Ltd, Basel, Switzerland 7. GCE Solutions Inc., Amsterdam, Netherlands 8. McGovern Medical School, The University of Texas Health Science Center at Houston (UTHealth), Houston, TX, USA
Objectives | The efficacy and safety of ocrelizumab (OCR) in relapsing multiple sclerosis (RMS) were demonstrated in the 96-week controlled double-blind period (DBP) of Phase III OPERA I/ (NCT01247324) and OPERA II/(NCT01412333) trials. We aim to assess the efficacy of switching from interferon (IFN)-β-1a or maintaining OCR therapy on disease activity and confirmed disability progression (CDP) after 4.5-year follow-up, in OPERA I and II open-label extensions (OLE).
Methods | Patients were randomized to receive OCR or IFN-β-1a, and on entering OLE period either continued OCR (OCR-OCR) or switched to OCR (IFN-OCR). Adjusted annualized relapse rate (ARR), time-to-onset of 48-week CDP (CDP48) and time-to-48-week confirmed EDSS score ≥6.0 (time-to- require-a-walking-aid) were analyzed until Week 336.
Results | 79.2% of OLE patients completed Year 4.5 (Y4.5). Adjusted ARR decreased from pre-switch year to OLE Y4.5 in IFN-OCR (0.2 vs 0.06), and maintained low in OCR-OCR (0.12 vs 0.04). Rates were lower in OCR-OCR vs IFN-OCR for CDP48 (DBP-end: 4.1% vs 8.5%; p<0.001, OLE Y4.5: 16.0% vs 20.3%; p=0.05) and in requiring walking-aid (DBP-end: 0.8% vs 3.1%; p=0.001, OLE Y4.5: 5.1% vs 8.3%; p=0.024). Over the DBP and OLE periods, risks were lower in OCR-OCR vs IFN-OCR for CDP48 by 28% (95%CI 0.56–0.93; p=0.01) and in requiring walking-aid by 46% (95%CI 0.35–0.83; p=0.004). Safety profiles in DBP and OLE were consistent.
86 Conclusions | Switching IFN-β-1a to ocrelizumab at OLE start was associated with rapid reduction in ARR, maintained throughout the 4.5-year follow-up period. Patients initiating ocrelizumab 2 years earlier accrued significant benefits on CDP48 and time-to-require-a-walking-aid.
1922 Serum Ig Levels and Risk of Serious Infections by Baseline Ig Quartile in the Pivotol Phase III Trials and Open-Label Extensions of Ocrelizumab in Multiple Sclerosis
Amit Bar-Or1, Robert Bemel2, Martin S Weber3, Richard W Hughes4, Chien-Ju Lin5, Jianmei Wang5, Annette Sauter4, Harold Koendgen4, Licinio Craveiro4, Stephen Hauser6, Tobias Derfuss7, Neil Shuey8
1. Department of Neurology and Center for Neuroinflammation and Experimental Therapeutics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA 2. Mellen Center for MS, Cleveland Clinic, Cleveland, OH, USA 3. Universitätsmedizin Göttingen, Göttingen, Germany 4. F. Hoffmann-La Roche Ltd, Basel, Switzerland 5. Roche Products Ltd, Welwyn Garden City, UK 6. University of California, San Francisco, San Francisco, CA, USA 7. University Hospital Basel, University of Basel, Basel, Switzerland 8. St Vincent’s Hospital, Melbourne, Fiztroy, VIC, Australia
Objectives | Reduction in serum immunoglobulin (Ig) levels was observed over 6-years of ocrelizumab treatment, with Ig levels remaining above the lower limit of normal-LLN for the majority of patients. An apparent association between decreased Ig levels and serious infections-SIs was previously reported. We aim to characterise the temporal evolution of Ig levels and risk of SIs by baseline Ig quartile in OPERA-I/(NCT01247324), OPERA-II/(NCT01412333) and ORATORIO/(NCT01194570) trials of ocrelizumab in multiple sclerosis, and their open-label extensions.
Methods | Serum Ig levels were measured at least every 24 weeks. Baseline IgG/IgM/IgA quartiles (g/L) were determined for OPERA and ORATORIO; Q1 represents patients with lowest baseline levels. LLNs for IgG/IgM/IgA were defined as 5.65/0.40/0.70 g/L, respectively. Periods of Ig Results | Over 6-years of treatment, mean change in IgG levels, g/L(% change) per year by baseline quartile were: Q1 –0.24(–2.9%); Q2, –0.32(–3.2%); Q3, –0.40(–3.6%); Q4, –0.40(–3.0%) in OPERA, and Q1, –0.23(–2.9%); Q2, –0.28(–2.9%); Q3 –0.35(–3.1%); Q4, –0.51(–3.8%) in ORATORIO. SI rates per 100 patient-years (95% CI) were: Q1, 1.63(0.95–2.61); Q2, 1.55(0.90–2.48); Q3, 1.51(0.86–2.45); Q4, 1.11(0.57– 1.94) in OPERA, and Q1, 4.04(2.66–5.87); Q2, 3.81(2.46–5.62); Q3, 5.39(3.76–7.50); Q4, 2.17(1.22–3.59) in ORATORIO. Conclusions | Patients with lower baseline IgG levels demonstrated less absolute IgG decline versus patients with higher baseline IgG levels. Similar percentage decline was observed across quartiles. Overall, rates of SIs per baseline IgG quartiles were similar and remain low. 1923 Eosinophilic Granulomatosis with Polyangiitis presenting with simultaneous central and peripheral nervous system involvement Julia Lim1, Yi Chao Foong1, Abhishek Malhotra1 1. Barwon Health, Geelong, VIC, Australia Objective | Peripheral nervous system involvement in eosinophilic granulomatosis with polyangiitis (EGPA) is well described. However, central nervous system involvement is uncommon. We describe a case of simultaneous central and peripheral nervous system involvement in EGPA. The diagnosis was confirmed on histopathology. A high index of suspicion is needed to initiate prompt treatment for this potentially life-threatening condition. 87 Report | 85-year-old male presented with subacute generalised lower limb weakness on the background of known eosinophilic asthma treated with interleukin-5 inhibitor (mepolizumab) and prednisolone. During his admission, he developed transient aphasia, followed by mononeuritis multiplex involving the left median and femoral nerves and purpuric rash over few days. Laboratory investigations showed no peripheral eosinophilia. ANCA revealed a borderline elevated myeloperoxidase antigen (MPO) of 21 U/mL. MRI brain revealed multiple small foci of diffusion restriction within the basal ganglia bilaterally, as well as paranasal sinusitis. MRA/CT cerebral angiogram was unremarkable. Prolonged telemetry and TOE did not show any central embolic cause. Left lateral gastrocnemius muscle biopsy revealed fibrinoid necrosis associated with adjacent eosinophils. Induction with intravenous cyclophosphamide was commenced along with high dose corticosteroids. He has been neurologically stable since. Conclusion | Simultaneous peripheral nervous system involvement with multiterritory stroke should heighten the suspicion for systemic vasculitis. Cerebral arterial imaging may be normal in small to medium vessel vasculitis such as EGPA. .Pre-existing mepolizumab therapy may make diagnosis more challenging by normalising pathology results. Histopathology can be of value to confirm diagnosis. 1. Keogh KA, Specks U. Churg-Strauss syndrome: update on clinical, laboratory and therapeutic aspects. Sarcoidosis Vasc Diffuse Lung Dis. 2006 Mar;23(1):3-12. 2. André R, Cottin V, Saraux JL, et al. Central nervous system involvement in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): Report of 26 patients and review of the literature. Autoimmun Rev. 2017;16(9):963-969. 1924 A Case of Chronic Inflammatory Demyelinating Polyradiculoneuropathy Presenting As Unilateral Lumbosacral Plexopathy Julia Lim1, Abhishek Malhotra1 1. Barwon Health, Geelong, VIC, Australia Objective | There are uncommon variants of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) including focal variants. Focal CIDP is rare but should be considered in the differential diagnosis of chronic progressive neuropathy affecting a single limb. Diagnosis can be challenging however electrophysiological study and nerve/plexus MRI can be helpful. We report a case presenting with monomelic right lower limb weakness and sensory deficit. This case adds to the limited literature regarding focal CIDP particularly with lower limb involvement. Report | 66-year-old male presented with chronic, progressive right lower limb weakness and sensory deficits in keeping with a lower right lumbosacral plexopathy with predominantly sciatic nerve involvement clinically and on electrophysiological studies. Whilst the distal motor latency and conduction velocities were within normal limits, there was marked asymmetry of right tibial and peroneal nerves with minimum F wave latency significantly prolonged on the right (greater than 120% upper limit of normal compared to the opposite side) raised possibility of proximal demyelination. Contrast enhanced MRI revealed a diffused thickening of the right lumbosacral plexus and proximal right sciatic nerve up. A lumbosacral plexus biopsy would have been helpful but was not undertaken due to patient’s preference. No alternative cause was detectable upon extensive investigation. Patient was initiated on intravenous immunoglobulin for probable focal CIPD and has remained stable over a short period of follow up. Conclusion | Even though lower limb involvement has rarely been described in literature, focal CIDP should be considered as a differential diagnosis of patients with focal neuropathies including unilateral lumbosacral plexopathy. 1. Mathey EK, Park SB, Hughes RAC, et al. Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype. J Neurol Neurosurg Psychiatry 2015;86:973–85. 2. Thomas PK, Claus D, Jaspert A, et al. Focal upper limb demyelinating neuropathy. Brain 1996;119:765–74. 3. El-Wahsh S, Cappelen-Smith C, Spies J. Chronic inflammatory demyelinating polyradiculoneuropathy presenting as predominantly sciatic monomelic neuropathy. BMJ Neurology Open 2020;2:e000045. 88 1925 Ofatumumab Reduces Disability Progression Independent of Relapse Activity in Newly Diagnosed, Treatment- Naïve Patients with Relapsing Multiple Sclerosis Suzanne Hodgkinson1, Ludwig Kappos2, Xavier Montalban3, Patricia Coyle4, Jacqueline Nicholas5, Sven Meuth6, Bingbing Li7, Krishnan Ramanathan8, Wendy Su7, Roman Willi8, Dieter A Haring8, Stephen Hauser9 1. Department of Neurology, Liverpool Hospital, Ingham Institute, University of NSW, Sydney, NSW, Australia 2. Neurologic Clinic and Policlinic and Research Centre for Clinical Neuroimmunology and Neuroscience, Departments of Medicine, Clinical Research, Biomedicine and Biomedical Engineering, University Hospital and University of Basel, Basel, Switzerland 3. Centre d’Esclerosi Multiple de Catalunya (Cemcat), Hospital Universitario Vall d’Hebron, Barcelona, Spain 4. Department of Neurology, Stony Brook University, Stony Brook, NY, USA 5. OhioHealth Multiple Sclerosis Center, Columbus, Ohio, USA 6. Department of Neurology, Medical Faculty, Heinrich-Heine-University, Dusseldorf, Germany 7. Novartis Pharmaceuticals Corporation, East Hanover, New Jersey, USA 8. Novartis Pharma AG, Basel, Switzerland 9. Department of Neurology, UCSF Weill Institute for Neurosciences, University of California San Francisco, San Francisco, California, USA Objectives | Ofatumumab significantly reduced PIRA in the ASCLEPIOS I/II trials1. This analysis tests the effect of ofatumumab on confirmed disability PIRA against variability in body weight in the ASCLEPIOS population and evaluates the relevance of PIRA in newly-diagnosed, treatment-naïve RMS patients. Methods | In the pooled ASCLEPIOS population, the effect of ofatumumab versus teriflunomide on PIRA and its robustness to variability in body weight by quartile was evaluated. Risk of 3-month/6- month PIRA was defined as 1.0-point increase if baseline EDSS score <6 or 0.5-point increase if baseline EDSS score 6 and was analyzed using Cox-regression models. The proportion of PIRA events (3m/6mPIRA) among the confirmed disability worsening events (3m/6mCDW) was quantified in 2 subsets of newly-diagnosed, treatment-naïve patients: A, without confirmed relapses on-study; B, without confirmed relapses prior to a 3m/6mPIRA. Results | Ofatumumab (versus teriflunomide) delayed PIRA in overall RMS population, irrespective of body weight; the risk of 6mPIRA by body weight quartiles was reduced with ofatumumab in both subsets (A: Q1- Q4: 31%- 37%; B: 36%-47%). In newly-diagnosed treatment-naïve patients, >50% of the CDW events were PIRA; ofatumumab (versus teriflunomide) reduced the risk of 6mPIRA by 56% (p=0.049) in Subset-A and by 59% (p=0.023) in Subset-B, with similar results for 3mPIRA. Conclusions | Ofatumumab demonstrated superior efficacy versus teriflunomide on PIRA in RMS patients, independent of body weight. In newly-diagnosed treatment-naïve RMS patients, a high proportion of CDW events were PIRA; ofatumumab markedly reduced the risk of PIRA in early RMS patients versus teriflunomide. 1. Kappos et al 2020, Presented at EAN 2020, 02034, European Journal of Neurology, 27 (Suppl. 1), 47 1932 An adult case of acute cerebellitis as a manifestation of Mycoplasma Pneumoniae infection Amanda Ji1, Michael Roizman1, Andrew Swayne1 1. Centre for Neurosciences, Mater Hospital, Brisbane, QLD, Australia Introduction | Acute cerebellitis is exceptionally rare in adults often preceded by systemic viral or bacterial infections. We present a case of acute cerebellitis following otherwise asymptomatic Mycoplasma Pneumoniae infection. Case descriptions | A 40-year-old man presented with a three-day history of acute onset of dizziness, vomiting and headache which was initially diagnosed and managed as vestibular neuritis. He was discharged and four days later represented with slurred speech and difficulty walking. On examination, 89 he was alert, had an ataxic gait, dysarthria and bilateral hypermetric saccades. CSF studies showed an elevated leukocyte count of 152X106/L with 100% mononuclear cells and a mildly elevated protein level of 0.7g/L with normal glucose. Other CSF analysis including a multiplex PCR panel for a variety of viral, bacterial and fungal pathogens, cytology, flow cytometry, antineuronal and encephalitis antibody tests were negative. MRI showed features consistent with acute cerebellitis including subtle diffuse cerebellar hyperintense signal on T2 and FLAIR sequences. Serological testing showed the presence of Mycoplasma Pneumoniae IgM and IgG. A diagnosis of acute cerebellitis was made, and the patient received supportive care and made a complete neurological recovery after 10 days. A repeat MRI one month later showed imaging resolution and he remained asymptomatic at clinic follow-up. Conclusion | This case describes an adult with cerebellitis most likely secondary to Mycoplasma Pneumoniae infection with other causes excluded on extensive investigation. Although rare, acute cerebellitis should be considered as a part of the differential diagnosis of acute vertigo particularly in the presence of cerebellar signs. 1933 Prognostic Value of Serum NfL for Subclinical Disease Activity and Worsening in Patients with RMS: Results from the Phase 3 ASCLEPIOS I and II Trials John Parratt1, 2, Tjalf Ziemssen3, Douglas L Arnold4, 5, Enrique Alvarez6, Anne H Cross7, Roman Willi8, Bingbing Li9, Petra Kukkaro8, Harald Kropshofer8, Krishnan Ramanathan8, Martin Merschhemke8, Wendy Su9, Dieter A Haering8, Stephen L Hauser10, Ludwig Kappos11, Jens Kuhle11 1. Department of Neurology, Royal North Shore Hospital, Sydney, NSW, Australia 2. Northern Clinical School, University of Sydney, Sydney, NSW, Australia 3. Department of Neurology, University Clinic Carl-Gustav Carus, Dresden, Germany 4. Brain Imaging Centre, Montreal Neurological Institute and Hospital, McGill University, Montreal, QC, Canada 5. NeuroRx Research, Montreal, QC, Canada 6. University of Colorado School of Medicine, Aurora, CO, USA 7. Department of Neurology, Division of Neuroimmunology, Washington University School of Medicine, Saint Louis, MO, USA 8. Novartis Pharma AG, Basel, Switzerland 9. Novartis Pharmaceuticals Corporation, East Hanover, New Jersey, USA 10. Department of Neurology, UCSF Weill Institute for Neurosciences, University of California San Francisco, San Francisco, California, USA 11. Neurologic Clinic and Policlinic and Research Centre for Clinical Neuroimmunology and Neuroscience, Departments of Medicine, Clinical Research, Biomedicine and Biomedical Engineering, University Hospital and University of Basel, Basel, Switzerland Objectives | To confirm prognostic value of baseline sNfL for brain lesion formation and volume change (VC) on MRI, and investigate the relationship of sNfL with regional brain VC. Methods | A pre-planned pooled analysis of patients (N=1882) stratified by median baseline sNfL (9.3 pg/ml) to assess prognostic value of sNfL on: new/enlarging T2 (neT2) annual rate in Year 1/2, and annual percentage VC in WB, cortical grey matter (cGM), white matter (WM), and thalamus. Pearson correlation coefficients between baseline sNfL and regional brain VC were analyzed. Results | High baseline sNfL was prognostic of increased on-study neT2 lesion formation in Year 1 (adjusted mean annual rate: ofatumumab 1.48 vs 0.57, +158%, p<0.001; teriflunomide 5.49 vs 3.26, +69%, p<0.001) and 2 (ofatumumab 0.09 vs 0.06, +65%, p=0.124; teriflunomide 4.53 vs 3.12, +46%, p=0.003), and was prognostic of higher annual percentage VC in WB (ofatumumab -0.32% vs - 0.23%, p=0.045 and teriflunomide -0.43% vs - 0.29%, p=0.001), WM (-0.30% vs - 0.18%, p=0.049 and - 0.37% vs - 0.17%, p=0.002), and thalamus (-0.49% vs - 0.29%, p=0.092 and -0.94% vs - 0.53%, p=0.001), but not cGM VC (- 0.36% vs -0.32%, p=0.514 and -0.51% vs - 0.48%, p=0.641). Baseline sNfL was correlated with thalamic (ofatumumab r=-0.374, p<0.0001 and teriflunomide r=- 0.308, p<0.0001) and WM (r=- 0.230, p<0.0001 and r=- 0.207, p<0.0001) VC, and less with cGM VC (r=-0.026, p=0.4679 and r=- 0.070, p=0.0523). Conclusions | Baseline sNfL levels are prognostic of on-study lesion formation and WB, WM, and thalamus VC, but not cGM. 90 1934 Characteristics and Outcome of COVID-19 in Patients with Relapsing Multiple Sclerosis Receiving Ofatumumab Kate Martel1, Robert A Walker1, Anne H Cross2, Silvia Delgado3, Mario Habek4, Maria Davydovskaya5, Natalia Totolyan6, Ratnakar Pingili7, Linda Mancione7, Roseanne Sullivan7, Martin Zalesak8, Wendy Su7, Krishnan Ramanathan8, Xavier Montalban9, Kevin Winthrop10 1. Novartis Pharmaceuticals Australia Pty Ltd, Macquarie Park, NSW, Australia 2. Washington University School of Medicine, Saint Louis, MO, USA 3. University of Miami Miller School of Medicine, Miami, FL, USA 4. School of Medicine, University Hospital Center Zagreb, University of Zagreb, Zagreb, Croatia 5. Moscow state Public Healthcare InsCity Clinical Hospital 24, Moscow, Russia 6. National Medical University, St. Petersburg, Russia 7. Novartis Pharmaceuticals Corporation, East Hanover, New Jersey, USA 8. Novartis Pharma AG, Basel, Switzerland 9. Centre d’Esclerosi Multiple de Catalunya (Cemcat), Hospital Universitario Vall d’Hebron, Barcelona, Spain 10. School of Public Health, Oregon Health & Science University, Portland, OR, USA Objective | Report characteristics of COVID-19 infections in people with multiple sclerosis (pwMS) receiving ofatumumab. Methods | Demographics, COVID-19 seriousness category, ofatumumab treatment duration, action taken with ofatumumab, interventions and COVID-19 outcomes were recorded for pwMS in the open- label extension study ALITHIOS or in the post-marketing setting. Results | At 28 September 2020, 12/1623 pwMS (5/12 females; 9/12 white) in the ALITHIOS study were reported to have laboratory-confirmed SARS-CoV-2 infection. Mean age was 37.8 years (median 44 years, range 25–51 years), disease duration 3 to 23 years, and EDSS score 0–5.5. Ofatumumab exposure range was 8.5–13.8 months (n=6 who received ofatumumab only in ALITHIOS) and 17.4–44.2 months (n=6 who continued ofatumumab from prior trials). One had COVID-19 seriousness grade 3 – a 39 year old white male with bilateral pneumonia requiring hospitalization who recovered with normal follow- up chest X-ray. The remaining 11 cases were non-serious grades 1 or 2: seven reported as completely recovered, one recovering, two as ongoing and one asymptomatic with SARS-CoV-2 IgM and IgG positive. Six patients were treated with anti-infectives (three received both antivirals and antibacterials and three received antibacterials). Ofatumumab treatment was unchanged in seven and interrupted in four (resumed in three; information not available for one) patients; action unknown in one. Conclusions | We report 12 cases (11 non-serious; one serious hospitalized for bilateral pneumonia) of laboratory-confirmed SARS- CoV-2 infection in pwMS treated with ofatumumab. More surveillance data are needed to determine the risks associated with COVID-19 in pwMS treated with ofatumumab. 1935 An Atypical Presentation of Transient Epileptic Amnesia Lasting Six Hours Benjamin D Wood1, Amanda Ji1, Michael Roizman1, Andrew Swayne1 1. Mater Centre for Neurosciences, Brisbane, QLD, Australia Background | Transient epileptic amnesia (TEA) is a clinical presentation of focal epilepsy from temporal origin characterised by transient episodes of anterograde amnesia without other aspects of cognitive impairment. It is a rare diagnosis which requires evidence of epilepsy, such as epileptiform abnormalities on electroencephalogram (EEG). We present a case of a 76-year-old female with a prolonged episode of TEA. Case Description | A previously well 76-year-old female presented to a tertiary hospital emergency department following sudden onset anterograde amnesia. She was disoriented to time and had repetitive questioning, however routine physical and neurological examination revealed no abnormalities. Initial investigations including CT Head and routine bloods did not find a reversible cause for her symptoms. She subsequently fully recovered after six hours. MRI of her brain showed 91 chronic small vessel changes with no hippocampal abnormality. Routine EEG revealed right temporal sharp waves maximal at T8, indicating an underlying temporal lobe epilepsy. A diagnosis of TEA was made, and the patient was commenced on Levetiracetam. The patient has not had further amnestic events since. Conclusion | This case demonstrates an atypical presentation of TEA. Typically, episodes of TEA last less than 1hr and occur on waking. Our case presented deceptively similarly to Transient Global Amnesia, a disorder with usually one episode of anterograde amnesia 2-24hrs long, characterized by repetitive questioning without impairment of other cognitive functions. However routine EEG workup showed evidence of temporal lobe epilepsy, re-clarifying the diagnosis as TEA. This case demonstrates the importance of EEG workup in presentations of transient amnesia. 1938 Outcome of endovascular thrombectomy for ultra-long aeromedical transfers: The experience of one “hub” and eleven “spoke” sites covering more than 1.8 million km2 Seppy Pakrah1, Karen Huang1, Haylee Berrill1, Meng Tan1, Peter Bailey1, Arman Sabet1, Laetitia Devilliers2, Hal Rice2, Darshan Shah1 1. Department of Neurology , Gold Coast University Hospital, Southport, QLD, Australia 2. Department of Interventional Neuroradiology, Gold Coast University Hospital, Southport, QLD, Australia Background | Local endovascular services for acute stroke in rural and remote regions remain a significant challenge despite higher rates of stroke in these regions. Objectives | We present time metrics, logistics, safety and outcome data on stroke patients with large vessel occlusion (LVO) that were aero-medically transferred from rural and remote “spoke” sites to the Gold Coast University Hospital (GCUH) “hub” for endovascular thrombectomy (EVT). Methods | This is a retrospective observational study utilizing prospectively collected stroke database from December 2018 to March 2020. Aeromedical transfers from ten rural and remote Queensland sites and one Northern New South Wales site were included, covering more than 1.8 million km² catchment area. Results | Over the period of 16 months, 20 out of 26 transferred patients underwent EVT. Mean distance was 1350 km, median time of ictus to recanalization was 928 minutes and TICI 2b-3 was achieved in 90% of the patients. One out of 20 patients (5%) had symptomatic intracranial hemorrhage (sICH), and 9 out of 20 (45%) achieved functional independence (mRS 0-2) at 90 days; similar to the recent pivotal trials. Conclusions | Our 12-center network experience confirms real world reproducibility of trial results, including ultra-long transfers, and supports such transfers at other centers worldwide. 1939 Neurostimulation based therapies in New Onset Refractory Status Epilepticus (NORSE): Case report Seppy Pakrah1, Shanthi Sarma 2, Meng Tan 1 1. Department of Neurology, Gold Coast University Hospital, Southport, QLD, Australia 2. Department of Psychiatry, Gold Coast University Hospital, Southport, QLD, Australia Objective | New Onset Refractory Status Epilepticus (NORSE) is a debilitating condition with significant mortality and morbidity despite treatment. There is currently no specific therapy for cryptogenic NORSE. Immunotherapies, hypothermia and ketogenic diet been applied. There are case reports and series utilizing neurostimulation based therapies, individually, to treat super-refractory SE. We report a case of cryptogenic NORSE treated with immunotherapies in conjunction with multiple neurostimulation based therapies. 92 Method | Case report. Result | A 21-year-old female presented with new onset seizures which became refractory within 24 hours. Continuous EEG monitoring in ICU showed generalized burst suppression, interrupted by highly recurrent generalized and multifocal epileptic discharges. Brain MRI showed bilateral temporal T2/ FLAIR hyperintensities. Autoimmune and paraneoplastic antibodies, CSF analysis including viral and fungal PCR and autoimmune encephalitis panel were unremarkable. Ovaries imagings were normal. Along with anticonvulsants, immunomodulatory treatments including methylprednisolone, intravenous immunoglobulin, plasma exchange, rituximab, anakinra and tocilizumab were used. Transcranial magnetic stimulation (TMS) was applied. She received two cycles of electroconvulsive therapy (ECT) commencing day 19 and daily transcutaneous vagus nerve stimulation (tVNS) commencing day 54 of admission. Invasive VNS was also delivered. Unfortunately, sustained seizure control was not achieved despite multiple treatment modalities, and she passed away after 80 days of ICU stay. Conclusion | To our knowledge, this is the first patient with NORSE treated with a series of multiple different neurostimulation therapies in conjunction with immunomodulatory therapies. Further studies are required to assess neurostimulation efficacy and the potential development of a stepwise protocol to integrate novel therapies for this condition. 1940 Subacute fulminant cognitive impairment as a presenting symptom of Multiple Sclerosis in a 51-year-old woman Seppy Pakrah1, Saman Heshmat 1 1. Department of Neurology , Gold Coast University Hospital, Southport, QLD, Australia Objective | Multiple sclerosis rarely presents primarily as debilitating cognitive dysfunction without accompanying disability in motor, sensory, or cerebellar function. We report the case of a 51-year- old woman with subacute fulminant cognitive impairment and amnesia as the first presentation of multiple sclerosis. Method | Case Report. Result | A 51-year-old woman presented with a two week history of severe memory impairment in the context of new behavioral disturbance for eight weeks. Repeated neuropsychological testing confirmed severely disturbed encoding, episodic and semantic memory. Neurological examination revealed right homonymous hemianopia, dyslexia without agraphia and left upgoing plantar reflex without any motor or sensory deficit. Thorough blood and cerebrospinal fluid testing performed to rule out metabolic, infective and autoimmune etiologies with unremarkable findings. Brain MRI showed bilateral hippocampal and temporal pole T2/FLAIR hyperintensity lesions, as well as cortical frontal and occipital lesions. It also showed multiple periventricular, infratentorial and callosal demyelinating lesions, some with contrast enhancement. Oligoclonal bands were present in CSF. Autoimmune encephalitis and preneoplastic antibodies were negative in blood and CSF. Cancer screening was unremarkable. She was treated with steroids and plasma exchange followed by rituximab with impression of Multiple Sclerosis. Unfortunately, the amnestic syndrome has remained unchanged for the last four months. Conclusion | This case represents a patient with new diagnosis of Multiple Sclerosis whose clinical phenotype is characterized by severe cognitive dysfunction and prominent cortical signs presenting as a subacute fulminant amnestic syndrome leading to a chronic clinical course. 1941 Paraneoplastic multiple cranial nerve palsies associated with anti-Hu and anti-Ri antibodies: Case report Seppy Pakrah1, Saman Heshmat 1 1. Department of Neurology, Gold Coast University Hospital, Southport, QLD, Australia 93 Objective | Classical anti-Hu and anti-Ri associated paraneoplastic syndromes include encephalomyelitis, cerebellar degeneration, sensory neuropathy and opsoclonus-myoclonus. Cranial nerve involvement is uncommon in this context. We report a case of paraneoplastic multiple cranial nerve palsies associated with anti-Hu and anti-Ri antibodies. To our best of knowledge, there is only one report in literature of same presentation with anti-Hu antibody. Method | Case Report. Result | A 78-year-old man presented with four-month-history of diplopia, dysphagia, significant functional decline and weight loss. Neurological exam was notable for bilateral partial palsy of cranial nerves III, as well as dysfunction of the left cranial nerves VI, VII, IX and X. Extremities examination was consistent with global 4+/5 power without sensory impairment, present reflexes (1+), and no fatigability. Contrast MRI brain with orbital and multiplanar brainstem images was normal. CT scan of the chest, abdomen and pelvis revealed a lung lesion with fluorodeoxyglucose (FDG) avidity, confirmed as small cell lung carcinoma (T0N2M0 SCLC) on biopsy. Anti-Hu and anti- Ri antibodies were positive on serum neural immunoblot. Additional autoantibody testing including other anti-neuronal, ganglioside GQ1b, acetylcholine receptor and voltage gated calcium channel (VGCC) antibodies were negative. Cerebrospinal fluid analysis and nerve conduction studies were not performed due to accelerated clinical decline. Best supportive care was recommended by the treating oncologist, and the patient passed away shortly after from aspiration pneumonia. Conclusion | This is a case of multiple cranial nerve palsies as a paraneoplastic syndrome associated with anti-Hu and anti-Ri antibodies, without evidence of leptomeningeal metastasis. 1944 Neuropathic pain in multiple sclerosis: impact of spinal cord stimulation, an under-utilised modality? Joel Corbett1, Peter Courtney2, Mike Boggild1 1. Townsville University Hospital, Douglas, QLD, Australia 2. Melbourne Pain Group, Melbourne, VIC, Australia Introduction | Neuropathic extremity pain is reported in 26% (95% CI 7-53%) of patients with multiple sclerosis (MS)(1), causing significant morbidity. Evidence for pharmaceutical management is limited and medications may cause prominent adverse effects. Spinal cord stimulation (SCS) is an effective treatment for central neuropathic pain and has demonstrated efficacy in MS(2-4). Limitations to use include lack of awareness, expense and the consequent inability to perform MRI for MS surveillance(4). Methods | We report four patients from a regional MS clinic with refractory neuropathic pain referred for SCS. Quality of life scores using the multiple sclerosis impact scale (MSIS-29) and medications requirements prior to SCS and at most recent review were assessed. Results | Four female patients, aged 48 to 55, were referred for SPS. All patients had a positive response to trial stimulation and proceeded to SCS implantation. Mean MSIS-29 score was 101/145 (range 91-121) prior to SCS and 54.5/145 (range 40-83) at most recent review. Three patients were able to reduce or cease analgesic medications. There was no associated operative morbidity. Conclusions | Three of the 4 patients in this series with refractory neuropathic pain experienced significant improvement in quality of life, measured by MSIS-29, after SCS implantation and were able to reduce or discontinue analgesic medications. SCS is a potentially effective treatment for refractory neuropathic pain in MS however is under-utilised for this indication. These 4 individuals, identified from a regional MS service, represent the only MS patients referred to this tertiary pain centre in recent year for consideration of SCS. 1. Foley PL, Vesterinen HM, Laird BJ, Sena ES, Colvin LA, Chandran S, et al. Prevalence and natural history of pain in adults with multiple sclerosis: systematic review and meta-analysis. Pain. 2013;154(5):632-42. 2. Kumar K, Nath R, Wyant GM. Treatment of chronic pain by epidural spinal cord stimulation: a 10-year experience. Journal of Neurosurgery. 1991;75(3):402 94 3. Provenzano DA, Williams JR, Jarzabek G, DeRiggi LA, Scott TF. Treatment of Neuropathic Pain and Functional Limitations Associated With Multiple Sclerosis Using an MRI-Compatible Spinal Cord Stimulator: A Case Report With Two Year Follow-Up and Literature Review. Neuromodulation: Technology at the Neural Interface. 2016;19(4):406-13. 4. Abboud H, Hill E, Siddiqui J, Serra A, Walter B. Neuromodulation in multiple sclerosis. Multiple Sclerosis Journal. 2017;23(13):1663-76. 1945 Effectiveness and Tolerability of Perampanel in Epilepsy Patients Treated in Routine Clinical Practice: a Global Pooled Analysis Study Wendyl D’Souza1, Eugen Trinka2, Tony Wu3, Imad Najm4, Manoj Malhotra5, Leock Y Ngo6, Rob McMurray7, Vicente Villanueva8 1. Department of Medicine, St Vincent’s Hospital Melbourne, The University of Melbourne, Melbourne, VIC, Australia 2. Department of Neurology, Christian-Doppler University Hospital, Paracelsus Medical University, Centre for Cognitive Neuroscience, Salzburg, Austria 3. Chang Gung Memorial Hospital Linkou Medical Center and Chang Gung University College of Medicine, Taoyuan, Taiwan 4. Cleveland Clinic Epilepsy Center, Neurological Institute, Cleveland Clinic, Cleveland, Ohio, USA 5. Eisai Inc, Woodcliff Lake, New Jersey, USA 6. Eisai Inc, Woodcliff Lake, New Jersey, USA 7. European Knowledge Centre, Eisai Europe Ltd, Hatfield, Hertfordshire, UK 8. Refractory Epilepsy Unit, Hospital Universitario y Politécnico La Fe, Valencia, Spain Objectives | To assess perampanel (PER) in everyday clinical practice. Methods | This interim pooled analysis of 18 real-world studies included patients with focal and generalized epilepsy treated with PER. Retention was assessed after 3, 6 and 12 months. Effectiveness assessments, evaluated by seizure type at the last visit, included seizure freedom rate (no seizures since at least prior visit) and 50% responder rate (≥50% seizure frequency reduction) for focal and/ or generalized seizures, and responder rate (seizures under control) for status epilepticus. Adverse events (AEs) and AEs leading to discontinuation were evaluated. Results | 3496 patients were included (50.5% female; mean age 41.0 years; mean number of previous antiepileptic drugs, 5.9). Seizure types at baseline were focal only (74.9%), generalized only (12.2%), focal and generalized (11.3%), and status epilepticus (1.6%). Most patients were treated with PER as adjunctive therapy; 3.2% were treated as monotherapy at baseline. Mean PER dosage was 2.4 mg/day at initiation and 6.5 mg/day at last visit. At 3, 6 and 12 months, retention rates were 90.7%, 78.6%, and 61.6%, respectively. At last visit, seizure freedom rates in patients with focal and generalized seizures were 13.9% and 47.6%, respectively; 50% responder rate were 45.6% (focal seizures) and 76.5% (generalized seizures); 36.5% of patients with status epilepticus had responded to treatment. AEs were reported for 55.4% of patients, most frequently dizziness/vertigo (17.1%) and behavioural (16.3%); 18.4% of patients discontinued due to AEs. Conclusion | In everyday clinical practice, PER was effective and generally well tolerated in patients with focal and generalized seizures. 1948 The Role of Magnetic Resonance Imaging in the Evaluation of Transient Ischaemic Attack Julia Lim1, Ian Goh1, Zun Niang Ng1, Nicholas Shearer1, Heather Smith1, Benjamin Clissold1 1. Barwon Health, Geelong, VIC, Australia Objectives | Transient ischemic attack (TIA) is historically defined as focal neurological deficit of a presumed vascular origin lasting <24 hours. This time-based definition was shown to misclassify up to 33% of patients who in fact have sustained acute cerebral infarction.1 With the availability of magnetic resonance imaging (MRI), a tissue-based rather than time-based diagnosis is now preferred.2 At our 95 institution, MRI brain was not a routine investigation in the evaluation of TIA. We aim to determine the incidence of acute cerebral infarction on MRI in patients with suspected TIA. Method | Retrospective analysis was performed via Barwon Health’s integrated network regarding all patients with suspected TIA presenting to the Emergency Department from January 2019 to July 2020. Patients who underwent MRI brain were then followed up to determine if new cerebral infarct was identified. Result | 208 patients were identified; 141 patients had symptoms <24 hours and 67 patients had symptoms >24 hours. MRI was performed in 56.7% (80/141) with symptoms <24 hours versus 73% (49/67) with symptoms >24 hours. Those with symptoms <24 hours, new cerebral infarct was seen on MRI in 5.7% (8/141) versus 7.5% (5/67) with symptoms >24 hours. The median timeframe to MRI was 32 days for patients with symptoms <24 hours, thus potentially underestimating the incidence of acute cerebral infarction. Conclusion | MRI brain is necessary in the workup of suspected TIA as new cerebral infarction could be missed if the diagnosis is solely based on symptom duration. Barwon Health has since incorporated MRI brain in TIA evaluation. 1. Shah SH, Saver JL, Kidwell CS, Albers GW, Rothwell PM, Ay H, et al. A multicenter pooled, patient-level data analysis of diffusion-weighted MRI in TIA patients. Stroke 2007;38:463. 2. Albers GW, Caplan LR, Easton JD, Fayad PB, Mohr JP, Saver JL, et al. Transient ischemic attack: proposal for a new definition. N Engl J Med 2002;347:1713–1716. 1949 Role of Dual Antiplatelet Therapy in Transient Ischemic Attack Julia Lim1, Ian Goh1, Zun Niang Ng1, Nicholas Shearer1, Heather Smith1, Benjamin Clissold1 1. Barwon Health, Geelong, VIC, Australia Objectives | Short-term dual antiplatelet therapy (DAPT) with clopidogrel and aspirin for secondary prevention in transient ischemic attack (TIA) has been shown to reduce 90-day stroke risk, including for symptomatic high-grade carotid stenosis >50%.1-3Beyond 30 days, haemorrhagic complications outweighed benefits.3 At our institution, there remains hesitancy of the use of DAPT. We aim to determine prescription rate of DAPT by Emergency Department (ED) versus Stroke Unit (SU) and evaluate 90-day stroke and bleeding risk. Methods | Retrospective analysis was performed on all TIA patients admitted to Barwon Health between January 2019 to July 2020. Patients commenced on 21-day DAPT were identified. High- risk TIA was defined as symptomatic carotid stenosis >50%. Bleeding risk was defined as major haemorrhage i.e. symptomatic intracranial haemorrhage or gastrointestinal bleeding. Results | 208 TIA patients were identified; 127 patients and 81 patients were admitted to ED and SU respectively. A higher rate of DAPT prescription was seen in the SU at 33.3% (27/81) versus 16.5% (21/127) in ED. 18.5% (5/27) SU patients versus 14.3% (3/21) ED patients were deemed to have had high-risk TIA. No cases of recurrent stroke or major haemorrhage at 90 days were seen in patients receiving DAPT. Conclusion | DAPT prescription is lower in ED when compared to SU. At Barwon Health, consultation with the stroke team is encouraged to facilitate high-risk TIA management. A low 90-day stroke and bleeding risk on short term DAPT for TIA was observed in this study. Given our small sample size, this finding may not be generalisable to different settings. 1. Hackam DG, Spence JD. Antiplatelet Therapy in Ischemic Stroke and Transient Ischemic Attack. Stroke 2019 Mar;50(3):773-778. 2. Wang Y, Wang Y, Zhao X, Liu L, Wang D, Wang C, et al; CHANCE Investigators. Clopidogrel with aspirin in acute minor stroke or transient ischemic attack. N Engl J Med 2013;369:11–19. 3. Johnston SC, Easton JD, Farrant M, Barsan W, Conwit RA, Elm JJ, et al; Clinical Research Collaboration, Neurological Emergencies Treatment Trials Network, and the POINT Investigators. Clopidogrel and aspirin in acute ischemic stroke and high-risk TIA. N Engl J Med 2018;379:215–225. 96 1950 Impact of access to MRI on neurology inpatients in a tertiary referral hospital Alexander E Dunn2, 1, Grace Swart2, 3, James R Burrell2, 1 1. Faculty of Medicine, University of Sydney, Sydney, NSW, Australia 2. Concord Repatriation General Hospital, Concord, NSW, Australia 3. Faculty of Medicine, University of New South Wales, Sydney, NSW, Australia Objectives | Magnetic resonance imaging (MRI) is critical for neurological diagnosis. A major challenge remains access to timely inpatient MRI. This study aims to investigate how delayed access to inpatient MRI may impact time to diagnosis, time to definitive treatment, length of stay, and ultimately hospital budgets in an Australian Tertiary Hospital. Methods | The present study assessed the effect of MRI wait times on time to discharge in neurology inpatients at Concord Repatriation General Hospital (Sydney, Australia), a 750-bed tertiary referral hospital with one MRI scanner, between 1 September and 30 November 2020. Data was analysed using descriptive statistics. Results | The study included 84 patients. Average MRI wait time was 2.1 days. The most common indication for MRI was exclusion of stroke not identified on CT. Overall, 26% of patients experienced a delay to discharge while waiting for MRI. The delay to discharge for these patients was 1 to 4 days, with a median of 1 day. 30% of MRIs allowed same day discharge. The MRI result changed management in 88% of patients. Conclusion | A significant proportion of patients experienced delay to discharge due to delay of inpatient MRI. The result of MRI changed treatment plans for most patients, and allowed same day discharge in almost one third of cases. Timely access to inpatient MRI is a critical and potentially modifiable variable that may reduce length of stay and expedite treatment for neurology inpatients. 1951 A case report of myelin oligodendrocyte glycoprotein antibody positive encephalitis mimicking HaNDL syndrome with subsequent acute optic neuritis Christopher Belder1, Thomas Kimber2 1. Neurology, Flinders Medical Centre, Adelaide, SA, Australia 2. Neurology, Royal Adelaide Hospital, Adelaide, SA, Australia A 22 year-old right-handed female presented with acute onset of speech disturbance. There was no visual/motor deficit or clinical seizure activity. CT brain/angiography/perfusion demonstrated hypoperfusion in the left temporo-parieto-occipital region (not conforming to a single vascular territory). There was subsequent improvement in her speech, and she reported a severe left temporal headache with associated nausea and photophobia. An urgent MRI brain was normal. She was diagnosed with migraine with dysphasic aura and treated symptomatically with ongoing improvement. On review the next day she reported mild ongoing headache, but her neurological examination was normal with no dysphasia. 2 days later, she developed right retro-orbital pain, which was followed a further 3 days later by right eye monocular visual impairment. Clinical examination was consistent with acute right optic neuritis. Repeat MRI brain/orbits demonstrated new changes of right optic neuritis with perineuritis and a left thalamic T2-hyperintense lesion. CSF studies demonstrated a monocytic pleocytosis. The clinical impression was of possible myelin oligodendrocyte glycoprotein (MOG) antibody disease leading to optic neuritis and unilateral cortical encephalitis, manifesting as cortical spreading depression/migraine aura. 97 She was commenced on IV methylprednisolone followed by an oral prednisolone taper with complete symptom resolution. Her serum subsequently tested positive for MOG antibody. MOG-antibody disease can cause a unilateral encephalitis that may cause attacks of cortical spreading depression. This case demonstrates that this may occur in the absence of the previously reported significant unilateral cortical MRI changes – we hypothesise that some patients previously diagnosed with “HANDL syndrome” may have MOG-antibody disease. 1. Ogawa R, Nakashima I, Takahashi T, et al. MOG antibody-positive, benign, unilateral, cerebral cortical encephalitis with epilepsy. Neurol Neuroimmunol Neuroinflamm. 2017;4(2):e322. Published 2017 Jan 16. doi:10.1212/NXI.0000000000000322 2. Matoba, S, Inoue, M, Morihata, H, Takeshima, T. Case report of myelin oligodendrocyte glycoprotein antibody- positive encephalitis mimicking hemiplegic migraine. Neurol Clin Neurosci. 2020; 8: 323– 325. https://doi. org/10.1111/ncn3.12420 1955 Feasibility of Instituting Graduated High Intensity Training for Parkinson Disease (FIGHT-PD); a non contact boxing exercise study David J Blacker1, Travis Cruickshank2, Mitchel Turner2, Claire Tucak1, Rai Fazio1 1. Perron Institute for neurological and translational science, Nedlands, WA, Australia 2. Exercise Physiology, Edith Cowan University, Joondalup, WA, Australia Objectives | Preliminary evidence suggests non-contact boxing exercise is feasible and possibly beneficial for Parkinson Disease (PD). Current studies lack detailed description of component elements and documentation of exercise intensity. We present the protocol of FIGHT-PD; which explores the feasibility, tolerability and safety of a non-contact boxing exercise program for PD, developed by a neurologist (who has PD), a professional boxing trainer, a neurophysiotherapist and exercise physiologists. Methods | Twenty early stage (Hoehn and Yahr 1 and 2) PD subjects will undergo baseline evaluations of PD and cardiac stress testing. Training includes quantifiable balance and movement drills, high intensity aerobic bursts, and sequences of punches using the Fightmaster training machine. Over 15 weeks, three 30-60 minute workouts per week will be conducted in three, 4 week blocks separated by rest weeks. Block one focuses on technique; the second escalates the physical intensity, and the third adds cognitive challenges. Rate of perceived physical exertion (RPE) and mental exertion will be measured by the Borg scale for every component of each workout, and heart rate continuously recorded by Polar monitors. Numerous standardised PD scales and a body chart discomfort scale will be administered at each workout, monitoring the development of pain or injuries. These observations will provide the primary outcomes of tolerability and safety, and secondary outcomes of quantified heart rate measuring exercise intensity, and effect on quality of life. Feasibility details including recruitment, retention and adherence rates will be measured. Conclusions | This trial should provide essential details to plan future exercise based studies. 1957 Neurological manifestations in Rheumatological disease: a case series II Eileen Mc Manus1, Alan Doube1, Jan Schepel1, Matthew Phillips1, Vicky Quinsy1, Kamal Solanki1 1. WDHB, Hamilton, Waikato, New Zealand Rheumatology encompasses a broad range of multisystemic, autoimmune and inflammatory disorders. Neurological manifestations of these diseases are not uncommon. Neurological findings may predate rheumatological findings or may emerge months to years post initial diagnosis. Rheumatological diseases presenting as neurological syndromes can cause diagnostic challenges. We present a 98 range of rheumatological cases with unusual neurological presentations that demonstrate this point. These include systemic lupus erythematosus with cerebritis, orbital pseudo-tumour related to granulomatosis with polyangiitis, intracranial giant cell arteritis (GCA) and ischaemic stroke, mononeuritis multiplex in granulomatosis with polyangiitis, and mononeuritis multiplex in systemic scleroderma (SSc). Familiarity with the neurological manifestations of rheumatologic diseases is important for both rapid diagnosis and appropriate intervention. 1963 Pembrolizumab induced Lambert-Eaton Myasthenic Syndrome Jasmine Ashhurst1, Rami Haddad2, Rob Zielinski3 1. St George Hospital, Sydney, NSW, Australia 2. Neurology, Orange Health Service, Orange, NSW, Australia 3. Medical Oncology, Orange Health Service, Orange, NSW, Australia Case Report | Lambert-Eaton Myasthenic Syndrome (LEMS) is a neuromuscular disorder caused by antibodies directed to the presynaptic voltage-gated calcium channel. It is often paraneoplastic, most commonly associated with Small Cell Lung Cancer (SCLC). This report outlines the case of a patient who developed LEMS secondary to pembrolizumab treatment for metastatic melanoma. An 82 year-old female presented to hospital 1 week after cycle 2 of pembrolizumab treatment for metastatic melanoma. On examination, she was found to have dysphagia, ocular muscle weakness and generalised weakness (most markedly weakness in hip flexors). Her weakness was fatigable and she had a waddling gait. Clinical picture was consistent with a clinical diagnosis of LEMS rather than myositis, which was confirmed by elevated anti-VGCC antibodies and response to Acetylcholinesterase inhibitors. Results | Anti-VGCC antibodies elevated at 119pM(<30). Transiently elevated CK, negative myositis autoantibodies, negative anti-MuSK antibodies, negative AChR antibodies. Although repetitive nerve stimulation did not show increment in the right ulnar CMAP after isometric muscle activation, the clinical picture was consistent with LEMS. Marked improvement to treatment with oral prednisone and pyridostigmine. Due to side effects, pyridostigmine was changed to 3,4-Diaminopyridine therapy with excellent response. Steroids were weaned off and the patient is adequately controlled on 3,4-Diaminopyridine. Conclusion | Our case report shows that LEMS can arise as a result of an immune-related adverse event (irAE) to pembrolizumab; an Anti-PD-1 Monoclonal Antibody. The immune response persists after cessation of this checkpoint inhibitor medication. It is important to recognise and treat this condition early. 1964 “Have I gone MAD?”: A case of Alice in Wonderland Syndrome Jasmine Ashhurst1, Rami Haddad2, Matthew Tait3 1. St George Hospital, Sydney, NSW, Australia 2. Neurology, Orange Health Service, Orange, NSW, Australia 3. Neurosurgery, Nepean Hospital, Sydney, NSW, Australia Case Report | Introduction | Alice in Wonderland Syndrome (AIWS) is a rare neurological disorder that is characterised by unusual distortions to perception. The aetiology of AIWS is unclear, though it has been reported in a number of neurological conditions including infection (esp. EBV), CNS lesions, migraines and as a side effect to medications. 99 This report outlines the case of a 72 year old gentleman who presented to hospital due to visual changes which he described as dysmetropsia (objects appeared distorted – thinner) and episodic chromatopsia. His chromatopsia was described as his entire visual field coloured with an orange hue, though this colour changed throughout the course of his admission. Movements appeared to occur in slow motion and movements of humans had a robotic appearance. Neurological examination was otherwise normal. These symptoms resolved after 2 days, and on subsequent examination his neurological and ophthalmological examinations were unremarkable. Results | MRI brain revealed a right occipito-temporal T2 hyperintense lesion, initially interpreted as a subacute ischaemic infarct. Repeat MRI with contrast revealed a stable appearance of the lesion with areas of subtle contrast enhancement. PET scan showed reduced metabolic activity within the lesion with reduced FDG accumulation. Biopsy of the lesion identified features of a diffuse astrocytoma. Conclusion | AIWS is a poorly recognised syndrome. Symptoms are not typical for an ischaemic event and alternative diagnosis should be investigated as an explanation for the cause of visual distortions. 1965 Post Hoc Analysis of Adjunctive Perampanel and Levetiracetam in Patients with Focal-Onset Seizures Co-administered With or Without Concomitant Enzyme-Inducing Anti-Seizure Medications (EIASMs) Samantha Goldman1, Leock Y Ngo2, Anna Patten1, Manoj Malhotra2 1. Eisai Ltd., Hatfield, Hertfordshire, UK 2. Eisai Inc., Woodcliff Lake, NJ, USA Objectives | We assessed the efficacy and safety of adjunctive perampanel co-administered with levetiracetam across four Phase III Studies (304, 305, 306 and 335) in patients (aged ≥12 years) with focal-onset seizures (FOS), with or without secondarily generalised seizures, stratified by concomitant enzyme-inducing anti-seizure medication (EIASM) use. Methods | Patients receiving perampanel 4–12 mg/day were included. Analyses were split by levetiracetam use: patients who were on concurrent use, had prior use or had never used levetiracetam, and by EIASM use (carbamazepine, eslicarbazepine, oxcarbazepine and phenytoin). Endpoints included 50% responder and seizure-freedom rates (Full Analysis Set [FAS]), and treatment-emergent adverse events (TEAEs; Safety Analysis Set [SAS]). Results | The FAS and SAS included 1386 and 1389 patients, respectively. Fifty-percent responder rates were numerically higher in patients with non-EIASMs compared with those with EIASMs, and were similar in patients with (29.4% vs 44.8% with EIASMs/non-EIASMs, respectively) or without (27.1% vs 43.6% with EIASMs/non-EIASMs, respectively) concurrent levetiracetam use. Seizure-freedom rates were similar across all patient groups with/without EIASM use, and with/without concurrent levetiracetam use (range: 2.0–5.9%). The incidence of TEAEs was slightly lower with EIASMs vs non-EIASMs in patients with/without concurrent levetiracetam use; the most common TEAE was dizziness, regardless of EIASM/levetiracetam use (range: 28.0–40.4%). Conclusions | These data suggest concurrent use of levetiracetam with perampanel does not affect the efficacy and safety of perampanel treatment, regardless of EIASM use. Funding: Eisai Inc. 100 1966 PROVE Study 506: Analysis of a Retrospective, Phase IV Study of Perampanel in Real-World Clinical Care of Patients Based on Seizure Type James Wheless1, Anna Patten2, Leock Y Ngo3, Alejandro Salah3, Manoj Malhotra4 1. University of Tennessee Health Science Center, Le Bonheur Children’s Hospital, Memphis, TN, USA 2. Eisai Ltd., Hatfield, Hertfordshire, UK 3. Formerly: Eisai Inc., Woodcliff Lake, NJ, USA 4. Eisai Inc., Woodcliff Lake, NJ, USA Objectives | PROVE (NCT03208660) was a retrospective, multicenter, non-interventional Phase IV study of perampanel during real-world clinical care of patients with epilepsy. Here, we report real- world efficacy and safety of perampanel from PROVE, based on baseline seizure type. Methods | Data were obtained from records of patients initiating perampanel after 01-Jan-2014. Follow-up completed on 15-Mar-2019. Endpoints included retention rate at 3/6/12/18/24 months (primary), efficacy and safety (secondary). Outcomes were stratified by baseline seizure type: focal- onset seizures (FOS) only; generalised seizures only; generalised tonic-clonic seizures (GTCS) only; myoclonic; absence seizures. Patients with FOS and generalised seizures (n=718) were excluded. Results | In the Safety Analysis Set (n=1703), 24-month retention rates were: 48.1% (FOS only, n=164/341); 47.8% (generalised only, n=120/251); 42.9% (GTCS only, n=27/63); 50.3% (myoclonic, n=100/199); 47.7% (absence, n=84/176). Of 51 patients with seizure data at Months 22–24, median reductions in total seizure frequency/28 days were: 75.0% (FOS only, n=20); 91.6% (generalised only, n=10); 40.0% (GTCS only, n=3); 70.0% (myoclonic, n=7); 100.0% (absence, n=4). Corresponding seizure- freedom rates were: 45.0% (FOS only, n=9/20); 30.0% (generalised only, n=3/10); 0.0% (GTCS only, n=0/3); 14.3% (myoclonic, n=1/7); 75.0% (absence, n=3/4); 50% responder rates were 33.3–100.0% across seizure types. Treatment-emergent adverse events occurred in 231/545 (42.4%; FOS only), 185/440 (42.0%; generalised only), 50/110 (45.5%; GTCS only), 117/328 (35.7%; myoclonic), and 154/301 (51.2%; absence) patients; the most common were dizziness and aggression. Conclusions | Perampanel retention rates and safety were generally similar across seizure types. Seizure frequency reductions were observed across subgroups. Funding: Eisai Inc. 1967 Efficacy and Safety of Perampanel as First Adjunctive Therapy after First or Second Monotherapy in Patients with Focal-Onset Seizures (FOS): Post Hoc Analysis of the FAME Study Ji Woong Lee1, Ji Hyun Kim2, Dong Wook Kim3, Amitabh Dash4, Manoj Malhotra5 1. Eisai Korea Inc., Seoul, Republic of Korea 2. Korea University Guro Hospital, Seoul, Republic of Korea 3. Konkuk University School of Medicine, Seoul, Republic of Korea 4. Eisai Singapore Pte. Ltd., Singapore 5. Eisai Inc., Woodcliff Lake, NJ, USA Objectives | Perampanel is a once-daily oral anti-seizure medication (ASM) for FOS and generalised tonic-clonic seizures. This analysis of the FAME study (NCT02726074) explored whether the efficacy/ safety of first adjunctive perampanel is impacted by the number of previous monotherapies (one/two). Methods | Patients were aged ≥12 years with FOS with/without focal to bilateral tonic-clonic seizures (FBTCS), and had failed ASM monotherapy. Perampanel was up-titrated to ≤12 mg/day (12 weeks), followed by a 24-week Maintenance Period. Endpoints: 50% responder/seizure-freedom rates, median 101 percent change in seizure frequency/28 days and treatment-emergent adverse events (TEAEs). Endpoints were stratified by prior treatment (first/second monotherapy). Results | The Full Analysis Set included 79 patients receiving perampanel 4–10 mg/day with first monotherapies (most commonly: levetiracetam [n=31]; carbamazepine [n=20]; oxcarbazepine [n=14]) and six receiving perampanel 6–12 mg/day with second monotherapies (levetiracetam [n=3]; oxcarbazepine [n=3]). First vs second monotherapy: 50% responder rates were 83.5% (n=66/79) vs 33.3% (n=2/6; P=0.0134), seizure-freedom rates were 50.6% (n=40/79) vs 0.0% (n=0/6; P=0.0274), and median percent reductions in seizure frequency/28 days were 100.0% vs 21.8% (P=0.0039). In patients with FBTCS (all first monotherapy), seizure-freedom rate was 75.0% (n=12/16). TEAE profiles (Safety Analysis Set) were similar with first vs second monotherapy (TEAEs: 75.3% [n=70/93] vs 77.8% [n=7/9]; serious TEAEs: 6.5% [n=6/93] vs 22.2% [n=2/9]; discontinuations due to TEAEs: 14.0% [n=13/93] vs 11.1% [n=1/9]; most common in both groups: dizziness). Conclusions | Adjunctive perampanel was associated with improved seizure control following first/ second monotherapy; supporting early-line perampanel use. However, small patient numbers may limit comparisons. Funding: Eisai Korea Inc. 1968 Efficacy and Safety of Perampanel in Paediatric Patients (Aged 4–<12 Years) with Focal-Onset Seizures (FOS) or Generalised Tonic-Clonic Seizures (GTCS) who Converted to Monotherapy: A Case Series from Study 311 Andras Fogarasi1, Leock Y Ngo2, Anna Patten3, Manoj Malhotra2 1. Epilepsy Center, Bethesda Children’s Hospital, Budapest, Hungary 2. Eisai Inc., Woodcliff Lake, NJ, USA 3. Eisai Ltd., Hatfield, Hertfordshire, UK Objectives | Study 311 (NCT02849626) was a multicentre, open-label study of adjunctive perampanel oral suspension in paediatric patients with FOS (with/without focal to bilateral tonic-clonic seizures [FBTCS]) or GTCS. We report efficacy and safety data from patients who converted to perampanel monotherapy during Study 311. Methods | Baseline demographics were recorded. Assessments included median percent change in seizure frequency/28 days from baseline, seizure-freedom rates and reporting treatment-emergent adverse events (TEAEs). Results | Overall, 4/180 patients converted to perampanel monotherapy. Baseline demographics were (age [years]/gender/seizure type/concomitant anti-seizure medication[s]): Patient 1: 7/female/GTCS/ phenytoin; Patient 2: 4/female/FOS/rufinamide, lacosamide; Patient 3: 10/male/FOS/oxcarbazepine; Patient 4: 9/male/FOS/oxcarbazepine. Median (range) total perampanel treatment duration was 363 (337–367) days; patients were receiving adjunctive perampanel for a median of 203.5 (186–244) days before converting to perampanel monotherapy (median [range] duration, 152 [118–171] days). Perampanel doses received during adjunctive and monotherapy perampanel periods were: Patient 1, 6 and 4 mg/day, respectively; Patient 2, 8 mg/day for both; Patient 3, 14 mg/day for both; Patient 4, 6 and 4 mg/day, respectively. At Weeks 40–52, median percent reduction in seizure frequency/28 days from baseline was 100.0% for Patients 1–3 and 75.0% for Patient 4. Patient 2 achieved seizure freedom at Weeks 40–52. During adjunctive/monotherapy, 29 TEAEs occurred in these 4 patients (prior to monotherapy conversion, n=22; during monotherapy, n=7). Conclusions | In this case series analysis, conversion to perampanel monotherapy provided efficacy and was generally well tolerated. Further investigation is warranted due to the small sample size. Funding: Eisai Inc. 102 1970 Long-Term Efficacy and Safety of Perampanel in Elderly Patients Aged ≥60 from Phase III Open-Label Extension Studies Manoj Malhotra5, Rohit Marawar1, Ilo Leppik2, Robert T Wechsler3, Anna Patten4, Leock Y Ngo5 1. Wayne State University, Detroit, MI, USA 2. University of Minnesota, Minneapolis, MN, USA 3. Idaho Comprehensive Epilepsy Center, Boise, ID, USA 4. Eisai Ltd., Hatfield, Hertfordshire, UK 5. Eisai Inc., Woodcliff Lake, NJ, USA Objectives | The long-term efficacy and safety of adjunctive perampanel was evaluated in patients aged ≥60 years with focal-onset seizures (FOS) with/without focal to bilateral tonic-clonic seizures (FBTCS) who participated in open-label extension Studies 307 (NCT00735397) and 335 (NCT01618695). Methods | Patients received perampanel 2–12 mg/day during Studies 307 (16-week blinded Conversion; 256-week Maintenance) and 335 (4-week Pre-conversion; 6-week Conversion; ≥46-week Maintenance). Assessments included median percent reduction in seizure frequency/28 days vs pre- perampanel baseline, 50% responder and seizure-freedom rates, and treatment-emergent adverse events (TEAEs). Results | The Safety Analysis Set included 71 patients (mean age, 64.0 years). Seizure frequency reductions during Years 1 and 2 were 31.7% (n=71) and 44.5% (n=38) (total FOS), and 95.5% (n=19) and 100.0% (n=9) (FBTCS), respectively. Seizure frequency reductions were observed during Years 3/4; over one-third of patients achieved a ≥50% seizure reduction each year. Seizure-freedom rates for total FOS during Years 1, 2, 3 and 4 were 0.0% (n=0/71), 2.6% (n=1/38), 5.3% (n=1/19) and 0.0% (n=0/14), and for FBTCS were 26.3% (n=5/19), 22.2% (n=2/9), 40.0% (n=2/5) and 0.0% (n=0/4), respectively; however, the small patient numbers at later timepoints may limit interpretation of these data. During Years 1, 2, 3 and 4, TEAE incidence was 87.3% (n=62/71), 60.4% (n=29/48), 47.4% (n=9/19) and 57.1% (n=8/14), respectively; most common were dizziness and fall during Years 1/2 and 3/4, respectively. Conclusions | Perampanel was associated with reductions in seizure frequency over 4 years in elderly patients. The safety profile was consistent with the overall populations. Funding: Eisai Inc. 1971 Shift from chronic to episodic migraine status and high- to low- frequency episodic migraine status among patients with treatment- resistant migraine in a phase 3 galcanezumab study Kathleen A Day1, Mallik Rettiganti1, Margaret B Ferguson1, Chad E Stroud1, Benjamin P Trewin2, Takao Takeshima3, William S Kingston4 1. Eli Lilly and Company, Indianapolis, IN, USA 2. Sharp Neurology, Sydney, NSW, Australia 3. Headache Center, Osaka, Japan 4. Sunnybrook Health Sciences Centre, Toronto, Canada Objectives | To assess shift from chronic migraine (CM) to episodic migraine (EM) status and high- frequency EM (HFEM) to low-frequency EM (LFEM) status (CONQUER study). Methods | Patients were randomized(1:1) to receive galcanezumab 120mg (loading dose:240mg)/ placebo during 3-month, double-blind (DB) period. Patients completing DB period entered an optional 3-month, open-label (OL) period(placebo patients switched to active treatment). Results | At baseline, 193 patients had CM (placebo/galcanezumab=98/95) and 198 had HFEM (placebo/galcanezumab=96/102). Mean (SD) number of migraine headache days/month were CM=18.7(4.7); EM=9.3(2.8); number of headache days/month were CM=20.9(4.4); EM=10.8(2.8). During DB period, significantly greater proportion of galcanezumab patients vs placebo shifted from CM to 103 EM status: Month 1, 49.0 vs 29.1[P=.008]; Month 2, 50.1 vs 25.7[P=.001]; Month 3, 61.9 vs 32.1[P≤.001]. At Month 3, significantly greater proportion of galcanezumab patients vs placebo shifted from CM to EM and sustained that shift in DB period, 35.8% vs 12.4%[P<.001]. By Month 3, patients treated with galcanezumab vs placebo shifted from HFEM to LFEM and VLFEM and sustained that shift in DB period, HFEM to LFEM: 39.9% vs 20.5%; HFEM to VLFEM: 9.8% vs 0%. By Month 6, 51.5% patients in galcanezumab/galcanezumab vs 46.7%[P=.540] in placebo/galcanezumab group had ≥3 consecutive months of shift from CM to EM; 60.2% vs 66.1%[P=.413] shifted from HFEM to LFEM and 18.2% vs 22.5%[P=.437]) shifted from HFEM to VLFEM. Conclusion | The sustained shift to EM and LFEM observed in this study, suggests that galcanezumab may result in reduced rates of disability and consequent lowering of disease burden associated with CM and HFEM. 1972 Perampanel Monotherapy for the Treatment of Epilepsy: Evidence from a Clinical Trial (Study 342) and Real-World Use (Studies 504 and 506) Antonio Gil-Nagel1, James Wheless2, Ji Hyun Kim3, Robert T Wechsler4, Takamichi Yamamoto5 1. Hospital Ruber Internacional, Madrid, Spain 2. University of Tennessee, Health Science Center & Le Bonheur Children’s Hospital, Memphis, TN, USA 3. Korea University Guro Hospital, Seoul, Republic of Korea 4. Idaho Comprehensive Epilepsy Center, Boise, ID, USA 5. Seirei Hamamatsu General Hospital, Hamamatsu, Japan Objectives | Perampanel is a once-daily oral anti-seizure medication for focal-onset seizures (FOS) and generalised tonic-clonic seizures. This analysis assessed efficacy/safety of perampanel monotherapy in patients with epilepsy from clinical and real-world studies. Methods | Patients with epilepsy were included in Studies 504 (prescribed perampanel monotherapy) and 506 (initiating perampanel after 01-Jan-2014); patients received perampanel as primary/secondary monotherapy. Study 342 recruited untreated patients aged 12–74 years with FOS, with/without focal to bilateral tonic-clonic seizures (FBTCS); patients received perampanel 4 mg/day (maximum: 8 mg/ day). Endpoints: retention rate (Studies 504/506, primary); seizure-freedom rate (Study 342, primary; Studies 504/506, secondary); treatment-emergent adverse events (TEAEs). Results | In Study 504, 60 patients received perampanel monotherapy. Retention rates: 55.6% (n=15/27; 12 months) and 60.0% (n=3/5; 24 months). Seizure-freedom rate: 55.0% (n=22/40; ≥3 months). In Study 506, 47 patients received perampanel monotherapy. Retention rates: 48.7% (n=19/39; 12 months) and 45.5% (n=10/22; 24 months). Seizure-freedom rate: 100.0% (n=2/2; Months 22–24). Most patients in Studies 504/506 had refractory epilepsy. In Study 342, 89 patients received ≥1 perampanel dose. Most patients with ≥1 post-dose efficacy measurement in the 4-mg Maintenance Period (n=73) achieved seizure freedom (4 mg/day: 63.0%; 4/8 mg/day: 74.0%). TEAEs occurred in 22/60 (36.7%; Study 504) and 17/47 (36.2%; Study 506) patients, and 57/89 (64.0%; 4 mg/day) and 67/89 (75.3%; 4 and/or 8 mg/day) patients in Study 342; most common was dizziness. Conclusions | These data support perampanel monotherapy as treatment for FOS, with/without FBTCS, and refractory epilepsy. Funding: Eisai Inc., Eisai Co., Ltd. and Eisai Ltd. 104 1974 Long-Term (1 Year) Seizure Freedom with Adjunctive Perampanel in Paediatric Patients (Aged 4–<12 Years) with Focal-Onset Seizures (FOS) or Generalised Tonic-Clonic Seizures (GTCS): Post Hoc Analysis of Study 311 Robert Flamini1, Anna Patten2, Manoj Malhotra3, Leock Y Ngo3 1. Pediatric and Adolescent Neurodevelopmental Associates, Atlanta, GA, USA 2. Eisai Ltd., Hatfield, Hertfordshire, UK 3. Eisai Inc., Woodcliff Lake, NJ, USA Objectives | Perampanel is a once-daily oral anti-seizure medication (ASM) for focal-onset seizures (FOS) and generalised tonic-clonic seizures (GTCS). Long-term seizure-freedom rates with adjunctive perampanel were assessed in paediatric patients aged 4–<12 years with FOS (with/without focal to bilateral tonic-clonic seizures [FBTCS]) or GTCS during Study 311 (NCT02849626). Methods | Seizure-freedom rates (FOS, FBTCS, GTCS) were assessed in patients who achieved seizure freedom during the Core Study Maintenance Period and then remained seizure free for up to 12 months (calculated from the start of their seizure-free period). Data were stratified by concomitant enzyme-inducing ASMs (EIASMs) (with and without) and age (4–<7 and 7–<12 years). Results | For FOS, 6/11 (54.5%) patients remained seizure free for 12 months (with EIASMs, n=2/5 [40.0%]; without EIASMs, n=4/6 [66.7%]; 4–<7 years, n=0/1; 7–<12 years, n=6/10 [60.0%]). For FBTCS, 1/5 (20.0%) patient(s) remained seizure free for 12 months (with EIASMs, n=0/1; without EIASMs, n=1/4 [25.0%]; 4–<7 years, n=0/2; 7–<12 years, n=1/3 [33.3%]). For GTCS, 3/7 (42.9%) patients remained seizure free for 12 months (all 7–<12 years, n=3/6 [50.0%]; no GTCS seizure-free patients received EIASMs). Conclusions | Despite small patient numbers, seizure-freedom rates are maintained during long-term (1-year) perampanel treatment in paediatric patients, consistent with analyses in adolescents/adults. Funding: Eisai Inc. 1984 False positive RT-QuIC test for Creutzfeldt Jakob disease in dementia with status epilepticus Rajiv Wijesinghe1, Craig Anderson2, Miriam Welgampola2, Christine Stehmann3, Steven Collins3, Penelope Spring1 1. Department of Neurology, Concord Repatriation General Hospital, Concord, NSW, Australia 2. Department of Neurology, Royal Prince Alfred Hospital, Camperdown, NSW, Australia 3. Australian National CJD Diagnostic Service, Florey Institute for Neuroscience and Mental Health, Parkville, VIC, Australia We present the case of a 72-year-old woman with likely false positive tests for Creutzfeldt Jakob disease (CJD). She had a background of focal epilepsy and dementia, related to previous alcohol abuse and head trauma. On her initial presentation in October 2019, she was drowsy with continuous left sided focal motor seizures. An EEG demonstrated continuous periodic lateralised epileptiform discharges (PLEDs) arising from the right temporal region, consistent with status epilepticus. She was commenced on levetiracetam and subsequently lacosamide, with seizure resolution over 12 days. Despite control of her seizures, she remained significantly cognitively impaired. A cerebral MRI demonstrated asymmetric cortical and thalamic diffusion restriction and the possibility of sporadic CJD was raised. A lumbar puncture revealed a normal total protein (0.4 g/L), a positive 14-3-3 protein but an undetectable tau protein. She was eventually discharged to a residential aged care facility, however returned to a different hospital in January 2020, obtunded with recurrent generalized seizures. Further results of her previous CSF examination were now available, revealing a positive RT- QuIC assay. However, a repeat MRI brain demonstrated resolution of the previous regions of diffusion restriction. Serial EEGs demonstrated continuous right temporal PLEDs which improved after the 105 addition of sodium valproate. Repeat cognitive screening was markedly improved, however not quite reaching her 2019 baseline. We suggest that a false positive RT-QuIC test probably arose from status epilepticus in the context of significant pre-existing cerebral pathology. Excluding a pre-mortem diagnosis of CJD may be challenging in the setting of recurrent seizures. 1985 Asymptomatic SARS-CoV-2 Infection and Isolated Abducens Palsy Elizabeth Tan1, Aishwarya Anilkumar1, Jonathan Cleaver1, Hamish Morrison1, 2 1. Neurology, Southmead Hospital, Bristol, Avon, United Kingdom 2. University of Bristol, Bristol, Avon, United Kingdom Background | The neuro-ophthalmological complications of SARS-CoV-2 are emerging, but the range and frequency of presentations remains undetermined. Abducens nerve palsy is the most common isolated oculomotor palsy; frequently associated with microvascular disease in older adults1. The aetiology in younger adults is less well known. We present, to our knowledge, the first case of isolated abducens palsy in an individual with no known vascular risk factors and otherwise asymptomatic of SARS-CoV-2 infection. Case | A 44-year-old female presented with 12 hours of diplopia. Her past medical history was of migraine and asthma. Examination revealed an isolated right abducens nerve palsy. She developed a fever and SARS-CoV-2 infection was confirmed by RT-PCR. All other investigation results were normal including blood tests, MRI and lumbar puncture. She remained well; at two week follow up there was complete resolution of the abducens palsy and diplopia. Discussion | A literature search revealed 6 reported cases of abducens palsy in symptomatic SARS- CoV-2 infection. Presenting signs included fever in 3 (50%) and hypoxaemia in 4 (67%) cases. 4 (67%) patients reported at least partial recovery in symptoms days-weeks following the onset2,3,4,5. To our knowledge there are no published cases of ocular nerve palsies in asymptomatic infection. In the absence of any identifiable cause or risk factors, the aetiology was presumed to be microvascular and potentially related to her underlying viral infection. Conclusion | We suggest considering asymptomatic SARS-CoV-2 infection in cases of abducens palsy with no risk factors, and add to the emerging literature on neuro-ophthalmological complications of SARS-CoV-2. 1. Moster ML, Savino PJ, Sergott RC, Bosley TM, Schatz NJ. Isolated Sixth-Nerve Palsies in Younger Adults. Arch Ophthalmol [Internet]. 1984 Sep 1;102(9):1328–30. 2. Falcone MM, Rong AJ, Salazar H, Redick DW, Falcone S, Cavuoto KM. Acute abducens nerve palsy in a patient with the novel coronavirus disease (COVID-19). J Am Assoc Pediatr Ophthalmol Strabismus [Internet]. 2020 Aug;24(4):216–7. 3. Dinkin M, Gao V, Kahan J, Bobker S, Simonetto M, Wechsler P, et al. COVID-19 presenting with ophthalmoparesis from cranial nerve palsy. Neurology. 2020;95(5):221–3. 4. Pascual-Goñi E, Fortea J, Martínez-Domeño A, Rabella N, Tecame M, Gómez-Oliva C, et al. COVID-19-associated ophthalmoparesis and hypothalamic involvement. Neurol – Neuroimmunol Neuroinflammation [Internet]. 2020 Sep 25;7(5):e823 5. Gutiérrez-Ortiz C, Méndez-Guerrero A, Rodrigo-Rey S, San Pedro-Murillo E, Bermejo-Guerrero L, Gordo- Mañas R, et al. Miller Fisher syndrome and polyneuritis cranialis in COVID-19. Neurology [Internet]. 2020 Aug 4;95(5):e601–5. 1990 EBV and NMDA receptor antibody positive Opsoclonus-Myoclonus Syndrome in an immunocompromised patient with renal clear cell carcinoma: a case report Catherine Ashton1, Janavi Dunuwille2 1. Neurology Department, Fiona Stanley Hospital, Murdoch, WA, Australia 2. Department of neurology, Sir Charles Gairdner Hospital, Nedlands, WA, Australia 106 Introduction | Rare cases of double positive Epstein-Barr virus (EBV) and anti-N-methyl-D-aspartate receptor (anti-NMDAR) antibody causing encephalitis have been described associated with solid organ transplant1-4. Opsoclonus-myoclonus syndrome is often a paraneoplastic or parainfectious phenomenon, but has only rarely been described associated with anti-NMDAR and usually with additional neuropsychiatric symptoms or encephalopathy5-6. We describe the rare case of a renal transplant patient with opsoclonus-myoclonus syndrome associated with renal cell carcinoma and anti-NMDAR and EBV DNA detected in cerebrospinal fluid (CSF). Methods | This was a personal case of the authors, with documentation and investigations reviewed from patient medical records at Sir Charles Gairdner Hospital. Results | A 55 years old immunocompromised female patient presented with two weeks of increasing ataxia, oscillopsia and tremor, preceded by 3 weeks of headache, nausea and vomiting. This was on a background of three failed renal transplants for IgA focal segmental glomerulosclerosis and recent resection of stage I renal clear cell carcinoma. Examination was consistent with opsoclonus- myoclonus-ataxia syndrome. There were no features on encephalopathy apart from mild emotional lability. CSF Results: lymphocytosis with an elevated protein, positive EBV using polymerase chain reaction and positive anti-NMDAR. Treatment: Plasma exchange, Rituximab, intravenous acyclovir for two weeks followed by oral acyclovir for 3 months. Improvement was marked, although with residual myoclonus on 2 month follow-up. Conclusions | This rare case of paraneoplastic double-positive EBV and anti-NMDAR opsoclonus- myoclonus syndrome in an immunocompromised patient demonstrates the broadening clinical phenotype of anti-NMDAR and highlights the contentious issue of EBV pathogenicity and treatment in an immunocompromised patient. 1. Garre et al. Letter to the editor: EBV-NMDA double positive encephalitis in an immunocompromised patient. Journal of the Neurological Sciences,2019: (396)76–77 2. Zhao C.Z., Erickson J., and Clinical Reasoning Dalmau J.: Agitation and psychosis in a patient after renal transplantation. Neurology 2012; 79: pp. e41-e44 3. Derksen S., Goraj B., Molenaar J., and van der Hoeven J.J.C.: Severe anti NMDA encephalitis and EBV infection. Netherlands J. Critic. Care 2013; 17: pp. 19-21 4. Cohen D.A., Lopez-Chiriboga A.S., Pittock S.J., Gadoth A., Zekeridou A., Boilson B.A., et al: Posttransplant autoimmune encephalitis. Neurol. Neuroimmunol. Neuroinflam. 2018; 5: pp. e497 5. Armangué, T. et al., 2016. Clinical and Immunological Features of Opsoclonus-Myoclonus Syndrome in the Era of Neuronal Cell Surface Antibodies. JAMA Neurology, 73(4), pp.417–424. 6. Klaas, J.P. et al., 2012. Adult-Onset Opsoclonus-Myoclonus Syndrome. Archives of Neurology, 69(12), pp.1598– 1607. 1991 Contactin-1-mediated chronic inflammatory demyelinating polyneuropathy (CIDP) presenting as an acute case of Guillain Bare Syndrome (GBS) Shane Cameron1, Rami Haddad1 1. Orange Health Service, Orange, NSW, Australia New and emerging evidence describing the role of autoantibodies to nodal and paranodal proteins in the pathogenesis of chronic inflammatory demyelinating polyneuropathy (CIDP) has arisen over the past decade, with significant diagnostic, therapeutic and prognostic implications. Although rare, CIDP with anti-contactin-1 (CNTN1) IgG4 antibodies has a distinct pathogenesis and clinical phenotype that differs from both typical CIDP and its other atypical variants. Here, we report the case of a young normally well 49-year-old man from rural Australia with severe refractory anti-CNTN1-mediated CIPD who presented like a case of Guillain-Barré syndrome (GBS) who improved dramatically after chemoimmunotherapy with Rituximab and who also had an unexpected late response to subsequent treatments with intravenous immunoglobulin (IVIg). In reporting this case, we hope to highlight important considerations in the diagnosis and treatment of patients with severe refractory CIDP, and especially those patients with anti-CNTN1 seropositive disease. 107 1. Vural, A, Doppler, K, & Meini, E 2018, ‘Autoantibodies against the node of ranvier in seropositive chronic inflammatory demyelinating polyneuropathy: diagnostic, pathogenic, and therapeutic relevance’, Frontiers in Immunology, vol. 9, pp. 1-14. 2. Miura, Y, Devaux, JJ, Fukami, Y, Manso, C, Belghazi, M, Wong, AHY, et al. 2015, ‘Contactin 1 IgG4 associates to chronic inflammatory demyelinating polyneuropathy with sensory ataxia’, Brain, vol 138, pp. 1484-1491. 3. Bunschoten, C et al. 2019, ‘Progress in diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy’, The Lancet Neurology, Vol. 18, no. 8, pp. 784-794. 4. Querol, L, Nogales-Gadea, G, Rojas-Garcia, R, et al. 2013, ‘Antibodies to contactin-1 in chronic inflammatory demyelinating polyneuropathy’, Annals of Neurology, vol. 73, pp. 370–380. 5. Querol, L et al. 2015, ‘Rituximab in treatment-resistant CIDP with antibodies against paranodal proteins’, Neurology, Neuroimmunology & Neuroinflammation, vol. 2, no. 5, pp. 1-6. 1992 Neurofilament light chain concentration predicts risk of relapse in participants with relapsing multiple sclerosis in phase 3 ozanimod trials Sarah Harris1, Giancarlo Comi2, Bruce A. C. Cree3, Lawrence Steinman4, James K. Sheffield1, Harry Southworth5, Ludwig Kappos6, Jeffrey A. Cohen7 1. Bristol Myers Squibb, Princeton, New Jersey, United States 2. Department of Neurology, San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy 3. Department of Neurology, Weill Institute for Neurosciences, University of California San Francisco, San Francisco, California, United States 4. Department of Neurology and Neurological Sciences, Beckman Center for Molecular Medicine, Stanford University Medical Center, Stanford, California, United States 5. Data Clarity Consulting Ltd, Stockport, United Kingdom 6. Neurologic Clinic and Policlinic, University Hospital and University of Basel, Basel, Switzerland 7. Department of Neurology, Mellen Center for MS Treatment and Research, Cleveland Clinic, Cleveland, Ohio, United States Objectives | Plasma neurofilament light chain concentration (pNfL-c) is increased in patients with multiple sclerosis (MS) and may serve as a biomarker for neurologic damage and disease activity in relapsing MS. We analyzed changes in pNfL-c and on-treatment risk of relapse with ozanimod vs interferon β-1a (IFN). Methods | In this post hoc analysis of the phase 3 SUNBEAM (NCT02294058; ≥12 months) and RADIANCE (NCT02047734; 24 months) trials, pNfL-c was measured at baseline and after 12 and 24 months of treatment with oral ozanimod 0.46 or 0.92 mg/d or intramuscular IFN 30 µg/wk. Poisson generalized linear models were used to fit the number of relapses as a function of baseline pNfL-c and treatment group with an offset for duration. Predictive modeling of expected annualized relapse rate (ARR) was calculated using median percentage change in pNfL-c from baseline. Results | At end of treatment, median pNfL-c was reduced from baseline by 20%‒23% (P<0.01) and 23%‒27% (P≤0.0001) with ozanimod 0.46 and 0.92 mg, respectively, and by 13%‒15% with IFN. Higher baseline pNfL-c was associated with more relapses (P<0.0001), and greater median reductions in pNfL-c from baseline were associated with lower ARR. Predictive modeling estimated that a 25% reduction in pNfL-c, similar to that observed with ozanimod 0.92 mg, predicts an ARR (standard error [SE]) of 0.18‒0.23 (0.4), whereas a 13% reduction, similar to IFN, predicts an ARR (SE) of 0.29‒0.37 (0.04). Conclusion | Our findings support pNfL-c as a biomarker for relapsing MS disease activity. Ozanimod caused greater dose-dependent reductions in pNfL-c and ARR than IFN. 1993 Bradycardia as a rare neurocardiac prodrome to Leucine-rich glioma inactivated-1 antibody encephalitis Sai Nagaratnam1, Yun T Hwang1, Elizabeth Reyneke1 1. Neurology, Gosford Hospital, Gosford, NSW, Australia 108 Introduction | Leucine-rich glioma inactivated-1 antibody encephalitis has been associated with bradycardia as a neurocardiac prodrome [1]. Concurrent occurrence of cardiac arrhythmia and faciobrachial dystonic seizures have not previously been reported. Case | A 73-year-old male presented with a 6 week history of frequent episodes of an unpleasant sensation associated with sinus bradycardia requiring pacemaker implantation. Episodes continued despite pacemaker. He was diagnosed with a seizure disorder and commenced on levetiracetam without response. Subsequently, on video EEG, subtle facial grimace and upper limb tonicity were captured, in keeping with faciobrachial dystonic seizures without an EEG correlate. MRI Brain showed no radiological evidence of encephalitis. Serum limbic encephalitis panel confirmed LGI1 antibodies. Other autoimmune and paraneoplastic antibodies were negative. He was treated with a course of corticosteroids. Induction dose of intravenous immunoglobulin was prematurely terminated after one dose due to MRSA bacteraemia and tricuspid valve endocarditis, necessitating removal of the pacemaker with no recurrence of seizures or bradycardia at follow up without further treatment. Conclusion | This case illustrates a rare presentation of LGI-1 antibody encephalitis with complete remission following incomplete induction course of intravenous immunoglobulin and corticosteroids. Neurocardiac prodrome as episodic bradycardia or asystole may precede the onset of encephalitis by approximately 2 months [1, 2]. There is a good response to immunotherapy, however relapse is common [3]. This case illustrates that clinically atypical presentations of cardiac arrhythmia may warrant neurological review and raises a possibility that early initiation of immunosuppressive therapy may significantly alter the disease course of LGI-1 antibody encephalitis. 1. Naasan G, Irani SR, Bettcher BM, Geschwind MD, Gelfand JM. Episodic Bradycardia as Neurocardiac Prodrome to Voltage-Gated Potassium Channel Complex/Leucine-Rich, Glioma Inactivated 1 Antibody Encephalitis. JAMA Neurology. 2014;71(10):1300-4. 2. Nilsson AC, Blaabjerg M. More evidence of a neurocardiac prodrome in anti-LGI1 encephalitis. Journal of the Neurological Sciences. 2015;357(1):310-1. 3. van Sonderen A, Thijs RD, Coenders EC, Jiskoot LC, Sanchez E, de Bruijn MAAM, et al. Anti-LGI1 encephalitis. Clinical syndrome and long-term follow-up. 2016;87(14):1449-56. 1995 A putative mechanism for subcortical aphasia Shadi El-Wahsh1, 2, David Greenup3, Gemma White4, Michael J Fulham5, 6, Arun Aggarwal3, 7, G. Michael Halmagyi1 1. Neurology, Royal Prince Alfred Hospital , Sydney, NSW, Australia 2. South Western Sydney Clinical School, University of New South Wales, Sydney, NSW, Australia 3. Department of Rehabilitation Medicine, Balmain Hospital, Sydney, NSW, Australia 4. Speech pathology department, Balmain Hospital, Sydney, NSW, Australia 5. Department of Molecular Imaging (PET and Nuclear Medicine), Royal Prince Alfred Hospital, Sydney, NSW, Australia 6. Sydney Medical School, University of Sydney, Sydney, NSW, Australia 7. Neurology, Concord Repatriation General Hospital, Sydney, NSW, Australia Objectives | The role of subcortical structures in language function are still poorly understood. We aim to provide a putative mechanism for subcortical aphasia through a structural and functional imaging-based case discussion. Methods | We present a case of subcortical aphasia due to basal ganglia hypertensive haemorrhage and discuss serial MRI and PET imaging findings to elucidate the mechanism of profound language impairment in acute subcortical pathology. Results | A 71-year-old right-handed architect presented with acute onset global aphasia and right-sided hemiparesis. CT imaging showed a flame-shaped left-sided basal ganglia haemorrhage. MRI brain showed a left basal ganglia haemorrhage without ischaemic or haemorrhagic damage to the overlying fronto-parietal cortex. FDG-PET imaging showed profound left fronto-parietal cortex hypometabolism, as well as ipsilateral caudate, putamen, thalamic and pontine hypometabolism. MR tractography identified truncation of the arcuate fasciculus around the left angular gyrus as well as disconnection of the left fronto-parietal association fibres. Over 12 weeks of rehabilitation, 109 the patient began to generate verbal output and was discharged home with ongoing word finding difficulties, nominal aphasia, and semantic paraphasias. Progress PET imaging revealed persistent hypometabolism in the aforementioned regions. Conclusion | We believe this is an important educational case for neurologists regarding the presentation of aphasia due to isolated subcortical lesions and raises some interesting hypotheses regarding a putative mechanism for subcortical aphasia due to dominant hemisphere cortical inactivation. 1997 Hemi-cord infarction following vertebral artery dissection in a patient with congenital hypoplastic vertebral artery: A case report Alanna Rottler1, Yew Li (Michelle) Dang1, 2, Wai Foong Hooi1, 3, David A Burrows4, Hong Kuan Kok4, 5, Douglas E Crompton1, 6 1. Department of Neurology, Northern Health, Epping, VIC, Australia 2. Department of Neurology, Eastern Health, Box Hill, VIC, Australia 3. Department of Neurology, Austin Health, Heidelberg, VIC, Australia 4. Department of Radiology, Northern Health, Epping, VIC, Australia 5. School of Medicine, Faculty of Health, Deakin University, Burwood, VIC, Australia 6. Department of Medicine, Melbourne University, Northern Health, Epping, VIC, Australia Background | Whilst often causing posterior circulation strokes, vertebral artery dissections may also, more rarely, cause spinal cord infarction.1 This is the case report of a 39-year-old female with a right- sided high cervical hemi-cord infarction caused by vertebral artery dissection of a hypoplastic right vertebral artery. Presentation | A 39-year-old female with a history of migraines, presented with acute onset right neck pain, headache and right-sided paraesthesia of the arm and leg after rapid rotation of the neck to the left. Due to a headache similar to her usual migraine, the patient took Rizatriptan prior to presentation. Neurological examination revealed findings, including right-sided upper and lower limb paraesthesia, weakness and dysmetria, consistent with right hemi-cord infarction. CT angiogram of the neck and brain revealed a small calibre right vertebral artery of unclear aetiology. Subsequent MRA revealed a hypoplastic right vertebral artery with dissection causing a high cervical right hemi-cord infarction. The patient’s right sided paraesthesia and weakness slowly improved over three months with medical therapy and rehabilitation, however some deficits remain, which affect her quality of life. Conclusion | This unusual case is a poignant reminder to carefully consider alternative diagnoses that may mimic migraines, especially when neurological signs and symptoms are present. Such differential diagnoses, such as vertebral artery dissection and spinal cord infarction, are of particular importance to consider, even in young patients without any risk factors, given that they can cause significant disability which may impact on quality of life.2 1. Hsu J, Cheng M, Liao M, Hsu H, Weng Y, Chang K et al. The etiologies and prognosis associated with spinal cord infarction. Annals of Clinical and Translational Neurology. 2019;6(8):1456-1464. 2. Hsu C, Cheng C, Lee J, Lee M, Huang Y, Wu C et al. Clinical features and outcomes of spinal cord infarction following vertebral artery dissection: a systematic review of the literature. Neurological Research. 2013;35(7):676-683. 2002 An unusual presentation of leptomeningeal carcinomatous. Eileen J Mc Manus2, 1, Weng J Mak2, Margaret Fisher2, Alvin Tan2, Christopher Lynch2 1. Neurology, Waikato hospital, WDHB (waikato district health board), Hamilton, Waikato, New Zealand 2. WDHB, Hamilton, Waikato, New Zealand We present the case of an unusual presentation of leptomeningeal carcinomatous in a 66-year-old Maori gentleman. He initially presented with a 2-week history of progressive binocular horizontal diplopia and was diagnosed with an idiopathic cranial nerve VI palsy. Two months later he re- 110 presented with florid multiple cranial nerve palsies. Initially, he was thought to have tuberculosis, further workup eventually uncovered a stage IV NSCLC. Interestingly, there was no leptomeningeal enhancement or ventricular dilation on MRI imaging. Our case report highlights the heuristic pitfalls, technical challenges and devastating consequences often encountered in diagnosing leptomeningeal carcinomatous. It is important that clinicians are familiar with the appropriate diagnostic approach to leptomeningeal carcinomatous. 2003 Pituitary abscess – a case report Yi Chao Foong1, 2, Felicity Stringer2 1. Neurology, Monash Health, Melbourne, VIC, Australia 2. Neurology, Barwon Health, Geelong, VIC, Australia Pituitary abscess (PA) is a rare but potentially life-threatening condition, representing less than 1% of all pituitary lesions. We present a case of a pituitary abscess complicating a Rathke’s cleft cyst. A 25-year-old female presented to the emergency department with 2 days of severe bifrontal, constant headache associated with nausea and rigors. On examination she was alert and oriented. Vital signs were within normal limits, and she was afebrile. The remainder of the neurological exam was unremarkable with no visual field defect. Her laboratory tests on admission showed a mild neutrophilia. MRI brain revealed a 10x21x13 mm cystic mass occupying the sellar region with peripheral contrast enhancement. The lesion was hypointense on T1 and hyperintense on T2. The MRI was reported as a cystic pituitary macroadenoma or Rathke’s cleft cyst with a haemorrhagic component. Immediately after her MRI scan the patient became febrile, developed nuchal rigidity and mild confusion. She was treated empirically for meningo-encephalitis. A lumbar puncture was performed, which showed polymorph predominant pleocytosis, elevated protein and low glucose. The possibility of a pituitary abscess was raised and the following day the patient underwent trans-sphenoidal drainage. Frank pus was drained, confirming the diagnosis of a pituitary abscess. Histopathology showed features suggestive of a Rathke’s cleft cyst with superimposed infection, but there was no growth on culture. Post-operatively the patient was started on desmopressin, cortisone and thyroxine. She also completed 4 weeks of IV antibiotics. Follow up at 4 weeks post discharge revealed resolution of her symptoms with no recurrence. id 2004 Design of a novel Real World Evidence study of Mayzent (siponimod) onboarding and adherence in Secondary Progressive Multiple Sclerosis patients in Australia utilising the digital support platform MSGo Todd Hardy1, Patrick Aouad2 1. Brain and Mind Centre, University of Sydney, Sydney, NSW, Australia 2. Sharp Neurology, St Leonards, NSW, Australia Objectives | Siponimod (Mayzent) has recently been approved in Australia for the treatment of secondary progressive multiple sclerosis (SPMS) in adults. To support the onboarding process, an integrated digital platform called ‘MSGo’ has been developed by Novartis for Healthcare Professionals and their Multiple Sclerosis (MS) patients. Here we describe the design of a novel Real World Evidence study characterising the onboarding experience and adherence using data derived from MSGo. Methods | Approximately 500 adults with SPMS will be enrolled. Key inclusion criteria include enrolment in the study (via the MSGo platform) and treatment with siponimod. The primary endpoint 111 is the average time for onboarding, defined as the period between patient registration to the first dose of siponimod. Key secondary endpoints will address adherence during titration and the following 3 months and how different variables influence onboarding and adherence. The complete list of variables include patient demographics, pre-screen assessments, adherence rates and discontinuations. Results | The detailed study design will be presented. The design is novel, allowing any HCP across Australia to participate in the study, thereby reflecting the real-world prescribing setting for siponimod. This study has been approved by Ethics Committees in the public and private clinic settings, and will provide the first Real World Evidence for siponimod in Australia, giving local physicians insights into onboarding and adherence. Conclusion | This study will use data derived exclusively from a digital support platform, and represents a novel approach for understanding quality use of medicines for MS. 2006 Double trouble: Papilloedema secondary to idiopathic intracranial hypertension and aplastic anaemia Antonia Clarke1, Charles Shuttleworth2, Rachael Rodgers3, Justine Wang1 1. Neurology, St George Hospital, Kogarah, NSW, Australia 2. Haematology, St George Hospital, Kogarah, NSW, Australia 3. Obstetrics and Gynaecology, Royal Hospital for Women, Randwick, NSW, Australia Objective | We report the case of a 34-year-old female diagnosed concurrently with idiopathic intracranial hypertension (IIH) and aplastic anaemia. Case | A 34-year-old female with recent weight gain presented with headache and fatigue. Clinical examination revealed conjunctival pallor and occasional bruising, with fundoscopy and optic coherence tomography demonstrating bilateral papilloedema (grade III). There were enlarged blind spots bilaterally. The cerebrospinal fluid (CSF) opening pressure was greater than 30 cmH2O. An MRI brain was normal and there was no venous sinus thrombosis. A diagnosis of IIH was made, and she was treated with therapeutic removal of CSF, acetazolamide and weight loss strategies. Concurrently, a bone marrow biopsy to investigate profound panyctopaenia was consistent with aplastic anaemia. The patient had worsening IIH features during fertility preservation treatment in preparation for stem cell transplant. At five months, there was complete resolution of subretinal fluid and clinical papilloedema. Anti-thymocyte globulin and cyclosporine treatment was subsequently commenced. Conclusion | Previous case reports have emphasised the interplay between the pathophysiology of anaemia and IIH,1 with treatment of aplastic anaemia contributing to resolution of IIH.2,3 We believe this is the first reported case of concomitant IIH and aplastic anaemia with resolution of papilloedema prior to treatment of anaemia. We also highlight the challenges of managing IIH during fertility and cyclosporine treatment. 1. Biossue, V. et al. Anaemia and Papilledema. American Journal of Ophthalmology. 2003;135(4):437. 2. Nazir, SA. et. al. Pseudotumor Cerebri in Idiopathic Aplastic Anemia. Journal of AAPOS. 2003;7(1):71. 3. Lilley ER, Bruggers CS, Pollock SC. Papilledema in a patient with aplastic anemia. Arch Ophthalmol 1990;108:1674-5. 2007 Impact of Telehealth on multiple sclerosis (MS) outpatient clinics during the COVID-19 pandemic Vivien Li1, Ai-Lan Nguyen1, Izanne Roos1, Katherine Buzzard1, Chris Dwyer1, Mark Marriott1, Mastura Monif1, Charles Malpas1, Stefanie Roberts1, Lisa Taylor1, Elizabeth Carle1, Nicola Taylor1, Kelsey Tunnell1, Trevor Kilpatrick1, Tomas Kalincik1 1. Neurology, Royal Melbourne Hospital, Parkville, VIC, Australia 112 Objectives | 1. Characterise telehealth use in MS clinics during the COVID-19 pandemic. 2. Assess patient and clinician attitudes towards telehealth. 3. Compare telehealth-based and physical EDSS obtained during period of telehealth implementation. Methods | Clinic records from Mar-Dec 2020 were reviewed. Patients and clinicians completed questionnaires about experiences using Telehealth. The iMed database was searched for EDSS recorded via face-to-face and telehealth appointments during and compared to face-to-face EDSS preceding and following the study period. T-test and Chi-square test were used for between-group comparisons. Results | 2023 appointments (27% face-to-face, 35% video, 37% telephone) were conducted. New referrals were predominantly face-to-face (66%). 89% of patients were satisfied with telehealth. 58% felt they were as good as face-to-face visits, whilst only 11% of clinicians agreed. Many patients favoured a hybrid model. Safety during the COVID-19 pandemic was important to both groups. EDSS increase from the preceding visit was recorded in a significantly higher proportion of face- to-face than telehealth appointments (p=0.027), with the increase driven by patients with baseline EDSS≤4.0. Amongst patients with EDSS increases, similar numbers of suspected relapses were seen via both modalities. Absolute increase in EDSS was also significantly greater amongst patients seen face-to-face (p<0.0001). There was no significant difference in EDSS change at subsequent follow-up in patients with consecutive face-to-face versus intervening telehealth appointments. Conclusion | Patient satisfaction with telehealth was high, whilst clinicians preferred face-to- face consultations. EDSS increase was more frequently recorded via face-to-face than telehealth appointments, which may underestimate lower EDSS. Future clinics could combine both modalities. 2009 Headaches of raised intracranial pressure as a presenting feature of malignant infiltration in the cauda equina Matthew Silsby1, Andrew Martin2, Winny Varikatt3, Steve Vucic1, Victor SC Fung1, Parvathi Menon1 1. Neurology, Westmead Hospital, Westmead, NSW, Australia 2. Neurology, Blacktown Hospital, Blacktown, NSW, Australia 3. Anatomical Pathology, Westmead Hospital, Westmead, NSW, Australia Introduction | Raised intracranial pressure (ICP) headache is rarely caused by spinal cord lesions. We present two patients with rare presentations of uncommon malignancies surrounding the cauda equina resulting in raised ICP headache. Cases | Patient 1, a 54-year-old man, presented with two months of headache, blurred vision and 9kg weight loss. Fundoscopy revealed papilloedema. Shortly after presentation he developed lower limb weakness with absent tendon reflexes. MRI brain was unremarkable. MRI spine showed diffuse nerve root thickening and effacement of the CSF spaces around the cauda equina. Patient 2, a 51-year-old man, presented with four months of headache and blurred vision, progressive lower limb weakness and 20kg weight loss over 12 months. MRI brain showed two small lesions insufficient to cause raised ICP. MRI spine showed extensive lower spinal cord infiltration with diffuse thickening of the cauda equina. Pathological assessments revealed malignant histiocytosis in patient 1 and primary leptomeningeal gliomatosis in patient 2. Discussion | These cases demonstrate the rare presentation of raised ICP headache resulting from spinal lesions. In both cases the original presentation with raised ICP headache and absent lower limb signs led to diagnostic delay. Malignant lesions in the spinal column can increase ICP, though the mechanism is debated. These cases emphasise the importance of a broader search for the aetiology of raised ICP headache in the absence of a causative lesion on primary brain imaging. Further, these cases highlight rare presentations of two uncommon pathological entities. 113 2010 The Effect of Head Cooling and Remote Ischemic Conditioning on Improvement of Patients with Traumatic Brain Injury Amir Moghadam Ahmadi1, Fardin Hodoodi, Mohamad Allahtavakoli 1. Associate Professor of Neurology, Non-Communicable Diseases Research Center, Rafsanjan University of Medical Sciences, Rafsanjan, Iran Objectives | Some studies have shown that IL-10 and TNF-α levels are elevated in severe Traumatic Brain Injury (TBI). The aim of this study was to determine the role of local hypothermia and Remote Ischemic Conditioning (RIC) on oxidative stress and inflammatory response after TBI. Methods | The present study was a clinical trial on 84 TBI patients who were divided into 4 groups. Group A:only hospital routines; Group B:Head Cooling (HC) technique; GroupC:RIC technique; and Group D:both RIC and HC techniques. HC was performed for 1.5 to 6 hours in the first three days. RIC was performed within the golden time in four 5-minute cycles with full cuff and 5 minutes with empty cuff.The data were collected at admission time, 72 hours and 6 days after admission for interleukin, SOD, and TNF levels, as well as the GOSE score, the APACHE-II score, and the Marshall CT scan classification. Results | There was a significant relationship between the interventions and most predictive variables such as serum SOD, serum IL-10, and TNF-α levels 72 hours and 6 days after admission, but not at the time of admission. Conclusion | RIC technique was greater than the HC technique in 72 hours but not in 6 days and 28 days after admission so that the group receiving the HC technique recovered better in the latter two time periods. Generally, the combination of both techniques was more effective in the improvement of patients than each individual technique. Key Words: Traumatic Brain Injury, Head Cooling, Remote Ischemic Conditioning 2012 Retinal artery occlusion may herald the relapse of Diffuse Large B-Cell Non-Hodgkin Lymphoma Natalie C Palavra1, Jonathan Baird-Gunning 1, Emily Sutherland1, Michal Lubomski 1, 2 1. Department of Neurology, Royal North Shore Hospital, Northern Sydney Local Health District, St Leonards, NSW, Australia 2. Department of Neurogenetics, Kolling Institute, Faculty of Medicine and Health, University of Sydney and Northern Sydney Local Health District, St Leonards, NSW, Australia Objectives | Secondary central nervous system lymphoma (SCNSL) infers a devastating prognosis. Recognition and diagnosis of SCNSL is often challenging, due to variable clinical features, dependent on the extent of central nervous system (CNS) involvement. We present an atypical case of relapsed Diffuse Large B-Cell Non-Hodgkin Lymphoma (DLBCL), reflected by progressive blindness, associated with multiple cranial nerve and lumbosacral plexus abnormalities, indicative of leptomeningeal disease. Methods | The case was compiled from records of clinical data, MRI and nuclear medicine imaging as well as multiple histopathological findings. Results | An 81-year-old male with a background of DLBCL without CNS involvement, in remission, and a subacute left retinal artery occlusion, presented with a five-day history of monocular visual loss, later progressing to involve the contralateral eye. Accompanying clinical features included acute ataxia, facial and leg numbness and a productive cough. Persisting hyponatraemia was consistent with a syndrome of inappropriate antidiuretic hormone excretion. MRI imaging revealed widespread cranial nerve and cauda equina hyperintensities and enhancement. Cerebrospinal fluid analysis identified a protein of 4.74g/L, with indeterminant flow cytometry. Chest imaging revealed innumerable diffuse 114 ground-glass and semi-nodular foci of consolidation, suspicious for tuberculosis. Bronchoscopy and later lung biopsy eventually confirmed a relapse of DLBCL. Conclusion | This report illustrates an unusual presentation of SCNSL, initially misdiagnosed as an atheroembolic complication. Clinicians should consider SCNSL in presentations of multiple cranial and peripheral nerve involvement, in patients with a background of haematological malignancy. Retinal artery occlusion from probable lymphatomatous infiltration is rare and may be a heralding feature of SCNSL. 2013 Ceftriaxone therapy for Adult Alexander Disease: report of 2 cases Natalie C Palavra1, Omar Ahmad2, 3 1. Department of Neurology, Royal North Shore Hospital, Northern Sydney Local Health District, St Leonards, NSW, Australia 2. Department of Neurology, Sydney Adventist Hospital, Wahroonga, NSW, Australia 3. Department of Neurology, Hornsby Ku-Ring-Gai Hospital, Northern Sydney Local Health District, Hornsby, NSW, Australia Objectives | Adult onset Alexander Disease (AxD) is a rare leukodystrophy for which there is currently no treatment. Ceftriaxone has been proposed as a potential treatment for AxD. Here we report the clinical outcome of an extended course of intravenous cyclical ceftriaxone therapy in two patients with AxD. Methods | Case 1 is a 64 year old female with a five year history of progressive gait disturbance, bulbar palsy and horizontal diplopia. Case 2 is an 80 year old male with a two-year history of bulbar palsy and gait disturbance. Both cases were confirmed to have AxD and received intravenous ceftriaxone 2g daily for three weeks per month during the initial four months, then for 15 days monthly thereafter. Patients were assessed at baseline and approximately 4-month intervals using the Kurtzke Expanded Disability Status Scale, Modified Ranking Scale, and neurological examination. Results | Both cases displayed functional decline on ceftriaxone therapy, as assessed by outcome measures of disability. Progression on neuroimaging was also observed on MRI brain for both patients. Conclusions | These results suggest that ceftriaxone for AxD may not prevent functional decline. Caution should be applied before suggesting ceftriaxone for the management of AxD. 2015 Improving the quality of life for patients with chronic inflammatory demyelinating polyneuropathy (CIDP) by offering home treatment with subcutaneous immunoglobulin (SCIg) Linley Bielby1, Kaylene Bastin1, Chris Akers1, James Daly2 1. Department of Health Victoria & Australian Red Cross Lifeblood, Blood Matters, West Melbourne, VIC, Australia 2. Australian Red Cross Lifeblood, Brisbane, QLD, Australia Introduction | SCIg allows patients the option of home administration for a range of disorders, negating regular health service admissions for intravenous immunoglobulin (IVIg) administration. SCIg was approved for treatment of patients with immunodeficiencies in 2013 and CIDP since August 2019. To support the development of SCIg access programs the Department of Health Victoria offered seed funding, which was accepted by 11 health services in 2017. Aim | Method | Blood Matters employed a project nurse (November 2017) to work with health services to develop SCIg programs by identifying eligible patients, removing implementation barriers, providing resources and conducing staff training. Results | 17 Victorian public health services have functioning SCIg programs • 356 patients are currently receiving SCIg (16% of those potentially eligible by medical diagnosis) 115 • 482 patients with CIDP are receiving IVIg and eligible for SCIg by medical diagnosis • To date 24 (5%) have transitioned to SCIg. Patients report: • SCIg as a positive experience particularly in well supported programs • Drastic improvement in quality of life • Less anxiety with reduced health services visits and risk of exposure to potential infections (especially COVID 19). Considerable work has been undertaken with private health funds to help facilitate the transition to SCIg for their members. Conclusion | Patients currently receiving IVIg for CIDP have access to SCIg at home. Ensuring these patients are aware of the choice is important, and requires information and education. Resources to support are available through Blood Matters. 2016 Gadolinium encephalopathy presenting as status epilepticus following intrathecal injection Emily Sutherland1, Jonathan Baird-Gunning1, Laura Rudaks1, Natalie Palavra1, Michal Lubomski2, 1, Martin Krause1 1. Department of Neurology, Royal North Shore Hospital, St Leonards, NSW, Australia 2. Neurological Sciences, Prince of Wales Hospital, Randwick, NSW, Australia Objectives | Intra-thecal gadolinium is an alterative contrast agent for computed tomography myelograms. Uncommonly it can cause gadolinium encephalopathy which can present with an array of neurological signs and symptoms. Methods | Review of a case shared by interventional radiology, emergency, neurology and intensive care. This case describes the symptomatology, investigations, and treatment for gadolinium encephalopathy, alongside recommended dosages for intrathecal administration. Results | An 85 year-old female with an allergy to intra-venous iodine presented for a computed tomography myelogram for investigation of bilateral lower limb pain. During the myelogram 8mL of intrathecal gadolinium (Gadovist 1.0) equivalent to 8mmol of gadobuterol was injected in light of the iodine allergy. Ten minutes after completion of the procedure the patient had abrupt onset pelvic pain, nausea, and bilateral lower limb paraesthesiae, which was treated as an allergic reaction with an anti- emetic, analgesia, and steroids. Rapid deterioration followed with extreme agitation and subsequent convulsive status epilepticus. After intubation and treatment with anti-epileptic medication an EEG showed persistent non-convulsive status epilepticus. Intra-venous steroids were introduced, alongside two more anti-epileptics. She was extubated on day 7, and EEG normalised by day 24. The patient was discharged to a rehabilitation hospital with moderate residual cognitive impairments. Conclusion | This case outlines a recognised but infrequently reported response to intrathecal gadolinium. Guidelines for safe intrathecal injection are yet to be identified, as are specific treatment options. 2017 Ganglioside antibodies, meningoencephalitis and longitudinally extensive transverse myelitis: GAME-LETM Xin Zhang1, Liam Beiglari1, Nicolas Urriola1, Kaitlyn Parratt1 1. Royal Prince Alfred Hospital, Camperdown, NSW, Australia Objectives | To describe two uncommon cases of enteritis followed by meningoencephalitis and longitudinal extensive transverse myelitis associated with GM1 antibodies. To define an underrecognized disease entity: GAME-LETM. 116 Methods | Review of two clinical cases and discussion of relevant existing literature. Results | The first case is a 38-year-old man who developed diplopia, ataxia, urinary retention and T4 sensory level with moderate lower limb weakness , 7 days after onset of enteritis. The second case is a 23-year-old man who developed diplopia, ataxia, urinary retention, T4 sensory level and paraplegia 4 to 7 days after onset of enteritis. Both cases had investigations consistent with meningoencephalitis and longitudinally extensive transverse myelitis affecting cervical to lumbar spinal cord; elevated protein and pleocytosis in the CSF; no infective organisms detected in the CSF; and serum GM1 IgM but not IgG antibodies. In one case, Campylobacter jejuni was detected in stool. Both cases made excellent but incomplete recovery with immunotherapy. Conclusion | GM1 antibodies are antibodies against human gangliosides, which are often increased in patients with C. jejuni enteritis preceding Guillain-Barre Syndrome (GBS). These antibodies are suspected to facilitate molecular mimicry between C. jejuni lipo-oligosaccharides and human gangliosides in the peripheral nervous system. Reports of transverse myelitis following enteritis with GM1 antibodies are rare. We describe two cases illustrating similarities in clinical, imaging and serological findings, and along with review of the small number of published cases attempt to define this emerging clinical syndrome as ganglioside antibodies, meningoencephalitis and longitudinally extensive transverse myelitis: GAME-LETM. 1. Baar I, Jacobs BC, Govers N et al. Campylobacter jejuni-induced acute transverse myelitis. Spinal Cord. 2007; 45: 690-694. 2. Llamas Y, Hazel K, Nicholson P and Costelloe L. Longitudinally extensive transverse myelitis after Campylobacter jejuni enteritis. Pract Neurol. 2018; 18:143-145. 3. Rodriguez Y, Rojas M, Pacheco Y et al. Guillain-Barre syndrome, transverse myelitis and infectious diseases. Cellular and Molecular Immunology. 2018; 15: 547-562. 4. Chinn JS, Schuffler MD. Paraneoplastic visceral neuropathy as a cause of severe gastrointestinal motor dysfunction. Gastroenterology. 1988;95(5):1279-1286. 2019 Inflammatory complications of CGRP monoclonal antibodies Jason C Ray2, 1, 3, Penelope Allen4, 5, Ann Bacsi6, Julian Bosco3, 7, Luke Chen2, 3, Michael Eller3, 8, Lyndell Lim4, 5, Hock Kua9, Manjit Matharu10, Mastura Monif2, 3, 11, Richard Stark2, 3, Elspeth Hutton2, 3 1. Department of Neurology, Austin Health, Melbourne, VIC, Australia 2. Alfred Health, Melbourne, VIC, Australia 3. Monash University, Melbourne, VIC, Australia 4. Centre for Eye Research Australia, Royal Victorian Eye and Ear Hospital, East Melbourne, VIC, Australia 5. Department of Surgery, University of Melbourne, Parkville, VIC, Australia 6. Integrated Specialist Medical Care, Macquarie University Hospital and North Shore Private Hospital, Sydney, NSW, Australia 7. Allergy, Asthma and Clinical Immunology, Alfred Health, Melbourne, VIC, Australia 8. Department of Neurology, Monash Medical Center, Melbourne, VIC, Australia 9. Department of Pathology, Monash Medical Center, Melbourne, VIC, Australia 10. University College London (Ucl) Queen Square Institute of Neurology and The National Hospital for Neurology and Neurosurgery, Queen Square, London, Uk 11. Department of Neurology, Royal Melbourne Hospital, Melbourne, VIC, Australia CGRP monoclonal antibodies (mAb) are an effective new preventative treatment for episodic and chronic migraine, with a favourable adverse event profile. In addition to its role in mediating migraine attacks, CGRP is widely expressed throughout the body and in general has an anti-inflammatory/ immunoregulatory role. Thus, inhibitory CGRP may potentiate a pro-inflammatory state. We present a case series of eight patients with new or worsened inflammatory pathology in close temporal association with the start of CGRP mAb therapy. This case series provides novel insights on the potential molecular mechanisms and side-effect profile of CGRP inhibition and warrants vigilance in clinical practice. 117 2020 Acute monoplegia Kristen Lefever2, 1, Jared Eisemann3, Letitia Gore3, Laura Clarke2, Cullen O’Gorman2, 1, Andrew Swayne1, Daniel Schweitzer1 1. Neurosciences, Mater Public Hospital, Brisbane, QLD, Australia 2. Queensland Health, Woollongabba, QLD, Australia 3. Infectious Disease, Mater Hospital, Brisbane, QLD, Australia Objectives | Pyomyositis is a purulent infection of skeletal muscle, most commonly resulting from haematogenous spread of Staphylococcus aureus infection in immunocompromised individuals1. Early recognition and management is important to prevent dissemination of infection and associated complications, including death. Methods | Single case report Results | 63year old female represented to the emergency department with a three day history of a painful left arm, radiating from the neck. More recently, she had developed proximal left arm weakness and paraesthesia. She had suffered from a self-resolving diarrhoeal illness in the 48hours prior. Clinical examination confirmed a proximal left arm weakness of lower motor neuron pattern. Initial investigations demonstrated a normal creatinine kinase (CK) and white cell count, but elevated inflammatory markers. MRI cervical spine imaging excluded degenerative disease but raised suspicion of oedema within the left sided posterior cervical musculature. Subsequent blood cultures isolated methicillin sensitive Staphylococcus aureus. The patient developed lower back pain and fevers prompting further imaging, demonstrating multifocal epidural abscesses. There was no evidence of infective endocarditis. She received six weeks of intravenous flucloxacillin, followed by oral therapy, resulting in normalization of her inflammatory markers. Delayed repeat MRI imaging confirmed resolution of the epidural infection and improvement in left shoulder musculature oedema and hyperenhancement, which correlated with marked clinical improvement. Conclusion | Pyomyositis is increasingly recognised in temperate climates and immunocompetent individuals. Pain is an early feature, with CK often remaining normal throughout the disease1, 2. Pyomyositis should be considered as a differential diagnosis in all patients with new onset painful weakness. 1. Crum (2004). Bacterial pyomyositis in the United States. The American Journal of Medicine, 117: 420-428. 2. Bickels, Ben-Sira, Kessler, Wientroub (2002). Primary pyomyositis. The Journal of Bone and Joint Surgery, 84: 2277-2286. 2021 A case of non-convulsive status epilepticus (NCSE) as first presentation of sporadic Creutzfeldt-Jakob Disease (sCJD) Luke Gagen1, Kristen Lefever2, 3, Daniel Schweitzer3, Laura Clarke2, Cullen O’Gorman3, Andrew Swayne3 1. University of Queensland, Brisbane, QLD, Australia 2. Queensland Health, Woollongabba, QLD, Australia 3. Neurosciences, Mater Public Hospital, Brisbane, QLD, Australia Objectives | sCJD is a neurodegenerative prion disease characterised by rapidly progressive neuropsychiatric symptoms and movement disorder. Seizures are an uncommon first presentation of sCJD. We present a case of a 71 year-old female in whom presented in NCSE. Methods | Single Case Report. Results | A 71-year-old female initially presented with higher cortical impairment characterised by visuospatial deficits and apraxia suggestive of parietal lobe dysfunction. Subsequent MRI showed multifocal patches of gyriform diffusion restriction – cortical ribboning – predominantly in the right parietal cortex. Autoimmune and metabolic cerebrospinal fluid (CSF) testing were unremarkable for markers of autoimmune or infectious encephalitis. EEG demonstrated NCSE arising from this right 118 hemisphere lesion, with severe diffuse encephalopathy. A ward-based anti-epileptic drug regime was instituted without success. ICU management with midazolam infusion and intubation for NCSE was required. Clinically and electrographically the patient improved over a two-week period, with resolution of NCSE on repeat EEG. Unfortunately however a repeat MRI on Day 17 of admission demonstrated progression of cortical ribboning beyond the initial focus across hemispheres. This in combination with a positive 14-3-3 and tau protein on CSF, raised the likelihood of sCJD. Marked clinical deterioration followed extubation, with palliative care involvement and end of life planning. The diagnosis of sCJD was confirmed with brain biopsy at autopsy. Conclusion | The case is notable as it is rare for sCJD to present early with NCSE1 and reinforces the need for atypical presentations to prompt exploration of a broader differential diagnosis in the context of unexpected investigation findings. 1. Espinosa PS, Bensalem-Owen MK, Fee DB. Sporadic Creutzfeldt–Jakob disease presenting as nonconvulsive status epilepticus case report and review of the literature. Clinical neurology and neurosurgery. 2010 Jul 1;112(6):537-40. 2022 A phase 1b open label study of sodium selenate as a disease- modifying treatment for behavioural variant frontotemporal dementia Lucy Vivash1, 4, 2, 3, Charles B Malpas4, 3, Christopher M Hovens4, 3, Dennis Velakoulis4, 3, Terence J O’Brien1, 4, 2, 3 1. Monash University, Melbourne, VIC, Australia 2. Alfred Hospital, Melbourne, VIC, Australia 3. Royal Melbourne Hospital, Melbourne, VIC, Australia 4. University of Melbourne, Melbourne, VIC, Australia Objectives | Hyperphosphorylated tau is a pathological hallmark of ~45% of behavioural variant frontotemporal dementia (bvFTD). Therefore, hyperphosphorylated tau represents a promising treatment target for bvFTD. Sodium selenate stimulates the PP2A enzyme, which directly dephosphorylates hyperphosphorylated tau. This Phase 1b, open-labelled, study investigated sodium selenate as a disease-modifying treatment for patients with bvFTD. Methods | Twelve patients with bvFTD were treated with sodium selenate (15mg tds) for twelve months. Participants underwent a cognitive and behavioural battery, MRI, lumbar puncture and safety assessments at screening, baseline, and regular intervals throughout the study. Results | Twelve patients completed the study. Sodium selenate was safe and well-tolerated, with no study withdrawals. Adverse events included nail changes (n=7), hair loss (n=5), muscles aches, headache, fatigue, and fall (n=3). Dose reduction to 10mg due to adverse events occurred (n=5). No treatment-related serious adverse events occurred. Analyses of efficacy data are ongoing. A small decline in cognition and behaviour was observed, including NUCOG score (b=-0.18, 95%CI=-0.28--0.08) and Cambridge Behavioural Index (b=0.32, 95% CI=0.18-0.46). Brain atrophy ranged from -0.26% to -6.51% (n=7 >-1.8%, n=4 <-1.8%). CSF and plasma tau levels did not change. Exploratory analyses of “responders” (brain atrophy >-1.8%, n=7) found no change in NUCOG (b=-0.03, 95%CI -0.14-0.07) over time. Conclusion | Sodium selenate is safe and well tolerated in patients with bvFTD. Exploratory analyses indicate it may reduce atrophy and halt cognitive decline in a subset of bvFTD patients, and further study into sodium selenate as a potential treatment for bvFTD, and the identification of “responders” is warranted. 2025 Hallucinogenic Persisting Perception Disorder: A case series and review of the literature Hannah HF Ford1, Clare CF Fraser2, Emma ES Solly1, Joanne JF Fielding3, 1, Owen OW White4, 3, Anneke AVW Van der Walt5, 4, 6 1. Department of Neurosciences, The Alfred Hospital, Melbourne, VIC, Australia 2. Neuro-Ophthalmology, Sydney University, Sydney, NSW, Victoria 119 3. Ocular Motor Research Laboratory, Monash University, Clayton, VIC, Australia 4. Neuro-ophthalmology Service, The Alfred Hospital, Melbourne, VIC, Australia 5. Multiple Sclerosis and Neuroimmunology (MSNI), The Alfred Hospital, Melbourne, VIC, Australia 6. Multiple Sclerosis and Neuro-ophthalmology Group, Monash University, Clayton, VIC, Australia Objectives | To report the clinical characteristics and investigation findings of a series of Hallucinogenic Persisting Perception Disorder (HPPD) cases and review previous HPPD case reports from the literature. Methods | Case studies were collected from consultant neuro-ophthalmologists between 2019 and 2020. PubMed and MEDLINE databases were searched for case reports between 2000 and 2020 using the terms “hallucinogenic persisting perception disorder” and “case report” Results | Thirteen case studies were reviewed. Lysergic acid diethylamide (LSD), 3,4-Methyl enedioxy methamphetamine (MDMA) and cannabinoids were the most common drugs used prior to HPPD onset. Twenty-two different visual symptoms were described. The most commonly reported were visual snow, floaters, palinopsia, photophobia and photopsia. Ophthalmic and neurologic investigations were normal. Two patients fully recovered after benzodiazepine treatment or no treatment. Twenty-four literature case reports were identified. LSD, MDMA and cannabinoids were the most frequent drugs used. Seventeen different visual symptoms were described. Ophthalmic and neurologic investigations showed no clinically significant findings in the majority of cases. 25% of cases fully recovered after treatment with benzodiazepines, eye movement desensitisation and reprocessing therapy, anti-epileptic drugs or no treatment. Conclusions | A wide variety of hallucinogenic and non-hallucinogenic recreational substances are implicated in HPPD. Clinical presentation includes a diverse range of positive visual phenomena and overlaps with Visual Snow Syndrome (VSS). Neurologic and ophthalmic investigations are typically normal. Management is complicated due to a lack of high-quality evidence. Controlled trials are needed to better understand the pathophysiology and optimize treatment for HPPD. 2027 The Diagnostic Journey of Mitochondrial Disease Patients Laura I Rudaks1, 2, Eloise Watson1, Michal Lubomski1, Fabienne Edema-Hildebrand1, Kate Ahmad1, Christina Liang1, Ryan Davis1, 3, Carolyn Sue1, 3 1. Royal North Shore Hospital, St Leonards, NSW, Australia 2. Neurology Unit, Concord Repatriation General Hospital, Concord, NSW, Australia 3. Kolling Institute, St Leonards, NSW, Australia Introduction | Mitochondrial disorders can often be challenging to diagnose and patients may undergo protracted investigative odysseys before reaching a diagnosis [1]. This study reviewed the diagnostic journey for genetically confirmed Mitochondrial Disease patients. Methods | Patients with a genetic diagnosis of mitochondrial disease seen at the Department of Neurogenetics, Royal North Shore Hospital, were invited to complete an online survey at their appointment or via telephone. Participant clinical records were reviewed for additional data, including genetic diagnosis. Results | Between October 2018 and April 2020, survey results were obtained from 68 patients. The most common presenting symptoms were fatigue (39%), weakness (31%), and droopy eyelids (31%). The most frequently completed investigations were MRI (55%), neurophysiologic testing (45%) and EEG (44%). 33% of participants had consulted five or more doctors with an overall mean time to diagnosis of 6.2 years. 41% of patients received a diagnosis within two years of symptom onset, 31% between 5 and 15 years, and 11% after 15 years or more. 38% of participants received at least one alternative diagnosis prior to their definitive genetic mitochondrial disease diagnosis. Following diagnosis, 34% of patients joined a support group and 87% felt that this was beneficial. Conclusions | Our results demonstrate that many patients experience long delays, undergo many investigations and see multiple doctors before a diagnosis of mitochondrial disease is reached. It is 120 hoped that advances in diagnostic pathways and access to earlier genetic testing may streamline the process [2]. 1. Grier, J., Hirano, M., Karaa, A. et al. Diagnostic odyssey of patients with mitochondrial disease. Results of a survey. Neurol Genet 2018; 4: e230.doi:10.1212/NXG.0000000000000230 2. Watson E, Davis R and Sue C. New diagnostic pathways for mitochondrial disease. J Transl Genet Genom 2020; 4: 188-202 2028 IgLON5 Autoimmunity in Two Cases with Peripheral Nervous System Features Laura I Rudaks2, 1, Victor SC Fung4, 3, Jean-Pierre Halpern5, Omar Ahmad2, 5 1. Concord Repatriation General Hospital, Concord, NSW, Australia 2. Royal North Shore Hospital, St Leonards, NSW, Australia 3. Neurology Department, Westmead Hospital, Westmead, NSW, Australia 4. Sydney Medical School, University of Sydney, Sydney, NSW, Australia 5. Neurology Department, Sydney Adventist Hospital, Wahroonga, NSW, Australia Introduction | IgLON5 autoimmunity has four main clinical patterns; a sleep disorder, bulbar syndrome, PSP-like pattern and predominant cognitive impairment. Other manifestations include movement disorders, gait instability, dysautonomia and neuropsychiatric features [1-3]. Peripheral nervous system involvement has been occasionally reported [3,4]. We describe two cases of IgLON5 autoimmunity presenting with peripheral neuropathy. Cases | A 72-year-old lady presented with progressive distal lower limb numbness, paraesthesia, incoordination and gait disturbance. Associated features included fluctuating facial numbness, limb and trunk fasciculations, upper limb tremor, anxiety, episodic hyperventilation, nocturnal myoclonus and vocalisations in sleep. Nerve conduction studies (NCS) demonstrated demyelinating features in the lower limbs. Anti-IgLON5 antibodies were detected in cerebrospinal fluid. She was treated with IVIg, oral prednisolone, azathioprine and plasma exchange. A 73-year-old man presented with worsening tremor. Evolving features included facial paraesthesia, imbalance, head ‘fogginess’, visual agnosia, constipation, insomnia, sleep utterances, somnambulism, nocturnal tremor and myoclonus. NCS showed a generalised demyelinating sensorimotor polyneuropathy. Neuropathy screen demonstrated anti-IgLON5 antibodies and IgG kappa paraprotein, leading to a new diagnosis of monoclonal gammopathy of undetermined significance (MGUS). IgLON5 autoimmunity was considered the likely explanation for the peripheral neuropathy, as sural nerve biopsy findings were not typical for MGUS-related neuropathy. He received IVIg, oral prednisolone, plasma exchange and Rituximab. During follow-up, he progressed to multiple myeloma and commenced lenalidomide and dexamethasone. Conclusion | Our two cases and the few published reports suggest an association of peripheral neuropathy and IgLON5 autoimmunity. We recommend cases of IgLON5 autoimmunity undergo routine neurophysiological studies. 1. Sabater L, Gaig C, Gelpi E, et al. A novel NREM and REM parasomnia with sleep breathing disorder associated with antibodies against IgLON5: a case series, pathological features, and characterisation of the antigen. Lancet Neurology 2014;13:575-586 2. Gaig C, Graus F, Compta Y, et al. Clinical manifestations of the anti-IgLON5 disease. Neurology 2017;88:1736-1743 3. Honorat JA, Komorowski L, Josephs KA, et al. IgLON5 antibody: Neurological accompaniments and outcomes in 20 patients. Neurol Neuroimmunol Neuroinflamm 2017;4:e385 doi: 10.1212/NXI.0000000000000385 4. Wenninger S. Expanding the Clinical Spectrum of IgLON5-Syndrome. Journal of Neuromuscular Diseases 2017;4:337-339 121 2030 A persistent false familiarity: recollective confabulation presenting in a patient describing a life of ‘total instant recall’ Xin Zhang1, Nora Breen1, Kaitlyn Parratt1 1. Royal Prince Alfred Hospital, Camperdown, NSW, Australia Objectives | Recollective confabulation is a persistent and convincing perception that the present moment is a repetition of one experienced previously. We present the case of an 80-year-old male patient with recollective confabulation and discuss this clinical phenomenon. Methods | An 80-year-old male patient presented for review describing his life being in a state of total instant recall. This began when he thought his eBook was malfunctioning as he reported reading the same material he had encountered the day before. Symptoms gradually progressed to involve all aspects of episodic memory. In his own words, ‘every day is a repeat of the day before…. I ask my wife, why are they telling us the same news as yesterday?’. The familiarity extended to people, places and inanimate objects encountered daily. Results | Neuropsychological assessment revealed impairment in executive function, moderate reductions in verbal memory, and mild changes in language and visual memory. The patient had been treated for prostate and lung carcinoma hence paraneoplastic aetiology was considered. CSF showed oligoclonal bands but no specific antibodies. MRI brain showed mild generalised atrophy and small vessel disease. PET imaging and ambulatory EEG were unremarkable. The patient was treated with immunotherapy without significant clinical improvement. Conclusion | Recollective confabulation is rarely reported and occurs predominantly in the setting of a primary neurodegenerative condition.(1) It is a clinical encounter unique to all other neuropsychological complaints. We explore the physiological basis of recollective confabulation and outline invaluable insights this may have in our understanding of the physiology of memory. 1. Moulin CJ. Disordered recognition memory: recollective confabulation. Cortex. 2013;49(6):1541-1552. 2031 Fulminant ADEM mimicking a glial tumour Jessica Qiu1, Elizabeth Thompson2, Michael J Fulham3, Mrudula Krishnaswamy4, Michael E Buckland4, Liane Khoo5, Nicolás Urriola6, Kaitlyn Parratt1 1. Department of Neurology, Royal Prince Alfred Hospital, Camperdown, NSW, Australia 2. Department of Radiology, Royal Prince Alfred Hospital, Sydney, NSW, Australia 3. Department of Molecular Imaging, Royal Prince Alfred Hospital, Sydney, NSW, Australia 4. Department of Neuropathology, Royal Prince Alfred Hospital, Sydney, NSW, Australia 5. Department of Haematology, Royal Prince Alfred Hospital, Sydney, NSW, Australia 6. Department of Immunology, Royal Prince Alfred Hospital, Sydney, NSW, Australia Introduction | We describe an atypical case of fulminant acute disseminated encephalomyelitis (ADEM). Case | A 47 year-old Southeast Asian lady presented after developing headache, aphasia and right hemiparesis over four hours, preceded by dry cough for one week and fevers for two days. CT brain noted vasogenic oedema without enhancement in the left frontoparietal lobe, midline shift and incidental upper lobe consolidation and calcified hilar lymph nodes on CT chest. A provisional diagnosis of cerebral tuberculosis was made. MRI brain noted gross mass effect and T2 hyperintensity localised to the white matter, crossing the midline and extending directly to the pons without significant restricted diffusion. Ill-defined enhancement was noted without tuberculomas or leptomeningeal enhancement. MRI spine was unremarkable, as were extensive tests for infectious aetiologies on serum, sputum and CSF. A glial tumour was suspected; FDG-PET-CT did not show regions of increased metabolism. As the patient rapidly deteriorated, empirical corticosteroids, plasmapheresis and IVIg were commenced just prior to decompressive craniectomy and biopsy four days post-presentation. The biopsy demonstrated reactive astrocytosis and perivascular macrophages localised to the white matter, as well as perivascular and 122 perivenular demyelination consistent with ADEM. Absence of a significant lymphocytic infiltrate may have been influenced by the short time to biopsy. The patient made a remarkable recovery following cyclophosphamide, achieving independence in mobility and driving within two months. Conclusion | Atypical features of fulminant ADEM highlight the need for a high index of clinical suspicion and early institution of aggressive immunosuppressive therapy for a favourable outcome. 2032 An atypical case of idiopathic intracranial hypertension Yasser M Mansour1, Nick Saad2, Simon Liew2, Sameen Haque1 1. Nepean Hospital, Kingswood, NSW, Australia 2. Marsden Eye Clinic, Parramatta, NSW, Australia Idiopathic Intracranial Hypertension (IIH) is an increasingly common condition that usually presents with younger obese female patients. Studies report between 74-94% of patients with a BMI>30 (1,2,3,4,5), 95% with ages under 50 (7) and over 87-91% of the patients being overwhelmingly females (8,9). In this case report, we present a 66-year-old man with a BMI of 24.7 kg/m2 who was referred by his ophthalmologist with bilateral papilledema on the ophthalmic examination and OCTs. The 30-2 Humphrey Visual Field testing showed significant loss of his inferior field on the right side. There were also early field losses noted on the left side. Lumbar puncture showed a borderline elevated CSF opening pressure of 25 cmH2O. Initial and subsequent MRI brain and orbits have shown constellation of findings consistent with idiopathic intracranial hypertension. Extensive investigations were carried out to identify any secondary cause. These included CT venogram, CT neck, chest abdomen and pelvis, serum and CSF testing for inflammatory/autoimmune, paraneoplastic, infectious and metabolic causes. His non-compliance with Acetazolamide led to clinical deterioration and optic atrophy on the right side. After 2 years of the onset, the patient is clinically stable on 250 mg TDS of Acetazolamide with normal CSF opening pressure on repeat testing recently. 1. Durcan F, Corbett J, Wall M. The Incidence of Pseudotumor Cerebri. Archives of Neurology. 1988;45(8):875. 2. Galvin J, Van Stavern G. Clinical characterization of idiopathic intracranial hypertension at the Detroit Medical Center. Journal of the Neurological Sciences. 2004;223(2):157-160. 3. Kesler A, Gadoth N. Epidemiology of Idiopathic Intracranial Hypertension in Israel. Journal of Neuro- ophthalmology. 2001;21(1):12-14. 4. Radhakrishnan K. Idiopathic Intracranial Hypertension (Pseudotumor Cerebri). Archives of Neurology. 1993;50(1):78. 5. RADHAKRISHNAN K, AHLSKOG J, GARRITY J, KURLAND L. Idiopathic Intracranial Hypertension. Mayo Clinic Proceedings. 1994;69(2):169-180. 6. Radhakrishnan K, Sridharan R, Ashok P, Mousa M. Pseudotumour cerebri: Incidence and Pattern in North- Eastern Libya. European Neurology. 1986;25(2):117-124. 7. Bruce B, Kedar S, Van Stavern G, Corbett J, Newman N, Biousse V. Atypical idiopathic intracranial hypertension: Normal BMI and older patients. Neurology. 2010;74(22):1827-1832. 8. McCluskey G, Doherty-Allan R, McCarron P, Loftus A, McCarron L, Mulholland D et al. Meta‐analysis and systematic review of population‐based epidemiological studies in idiopathic intracranial hypertension. European Journal of Neurology. 2018;25(10):1218-1227. 9. Fraser J, Bruce B, Rucker J, Fraser L, Atkins E, Newman N et al. Risk factors for idiopathic intracranial hypertension in men: A case–control study. Journal of the Neurological Sciences. 2010;290(1-2):86-89. 2033 Neurological Presentations of Rare Chloromas in the Context of Haematological Malignancy Sophie Chatterton2, 1, Jacob Helou1, James Drummond3, Christopher Ward4, 5, Luke Coyle4, 5, Karl Ng1, 5 1. Department of Neurology, Royal North Shore Hospital, Sydney, NSW, Australia 2. University of New South Wales, Sydney, NSW, Australia 3. Radiology Department, Royal North Shore Hospital, Sydney, NSW, Australia 4. Haematology Department, Royal North Shore Hospital, Sydney, NSW, Australia 5. University of Sydney, Sydney, NSW, Australia 123 Objective | To describe the spectrum of neurological presentations of chloromas. Method | Two case reports. Results | A 72 year-old female presented with a right-sided cavernous sinus syndrome on a background of a myeloproliferative disorder. MRI brain demonstrated a FLAIR hyperintense lesion centred on the right cavernous sinus. Cerebrospinal fluid (CSF) was acellular with negative cytology and flow cytometry. FDG PET/CT demonstrated diffuse marrow hypermetabolism, hepatosplenomegaly and focal FDG uptake in the cavernous sinus and a remote paraspinal region, suspicious for chloromas. The patient developed pancytopaenia with blasts, and tumour lysis syndrome (TLS) consistent with transformation to acute myeloid leukaemia (AML). The patient was given pulse methylprednisolone, hydroxyurea and external beam radiotherapy to the cavernous sinus lesion with symptomatic improvement. The second case is of a 76 year-old male who presented with progressive lower limb weakness with acute faecal incontinence. This was on a background of AML treated with induction STIMULus (cytarabine and thioguanine) followed by remission confirmed on a recent bone marrow biopsy. CSF showed a marked lymphocytosis with many undifferentiated cells, and flow cytometry was consistent with AML. An MRI whole spine demonstrated a solitary avidly enhancing intramedullary T11 cord lesion with associated oedema. In the context of AML and CSF pathology this was thought to reflect an intramedullary chloroma. The patient was treated with external beam radiotherapy, pulse intravenous methylprednisolone and intrathecal chemotherapy with symptomatic improvement. Conclusion | These two cases demonstrate the diversity of neurological clinical presentations of chloromas, an unusual collection of leukaemic cells exerting mass-like effect. 2035 Bilateral abducens nerve palsies following bilateral middle meningeal artery embolisation for chronic subdural haemorrhage Subahari Raviskanthan1, Peter W Mortensen1, Yi J Zhang2, Andrew G Lee1, 3, 4, 5, 6 1. Department of Ophthalmology, Blanton Eye Institute, Houston Methodist Hospital, Houston, Texas, US 2. Department of Neurosurgery, Houston Methodist Hospital and Weill Cornell Medicine, New York, US 3. Department of Ophthalmology, University of Texas Medical Branch, Galveston, Texas, US 4. Department of Ophthalmology, University of Texas MD Anderson Cancer Center, Houston, Texas, US 5. Texas A and M College of Medicine, Bryan, Texas, US 6. Department of Ophthalmology, The University of Iowa Hospitals and Clinics, Iowa City, Iowa, US Middle meningeal artery embolisation (MMAE) has recently been proposed as a treatment for subdural haemorrhage (SDH). MMAE aims to decrease the vascular supply to abnormal membranes produced in response to dural space inflammation in SDH. We report a case of bilateral abducens nerve palsies following bilateral MMAE. A 37-year-old male with Type 1 diabetes, previous traumatic brain injury, and excessive alcohol intake presented with increasing headaches and falls. Brain imaging revealed bilateral SDH with minimal midline shift to the right (2-3mm). He subsequently underwent bilateral MMAE. Immediately post operatively, he noted new binocular horizontal diplopia, with bilateral abducens nerve palsies clinically. Repeat brain imaging revealed stable SDH with new punctate foci of diffusion restriction bilaterally – though none that would explain his abducens nerve palsies. There were no brainstem infarctions and there was no midline shift. 2 month post-operative review showed significant improvement in his bilateral abducens nerve palsies. The vascular supply to the abducens nerve is rich, with multiple external carotid artery and internal carotid artery branches, therefore abducens nerve ischemia is rare. Previous studies with provocative lidocaine testing into the middle meningeal artery for other endovascular procedures showed 3 patients developed transient abducens nerve palsy. There are only two cases of transient diplopia within the MMAE related literature, and it is unclear whether these were CN VI palsies. To our knowledge this is the first reported case of bilateral abducens nerve palsies post MMAE, possibly from indirect ischemia post embolisation of the middle meningeal artery. 124 2036 An Unexpected Cause of Ophthalmoplegia and Areflexia: The Pupils Have It Jessica Qiu1, Albert Vien1, Andrew Hannaford1, Patricia Ferguson2, Mohammad Hamidi3, Amit Vaidya3, Andrew Henderson1 1. Department of Neurology, Westmead Hospital, Sydney, NSW, Australia 2. Department of Infectious Diseases, Westmead Hospital, Sydney, NSW, Australia 3. Department of Intensive Care, Westmead Hospital, Sydney, NSW, Australia Introduction | We describe a case of botulism and its differentiation from acute neurological presentations. Case | A 35 year-old man presented with dysphagia, subjective jaw numbness, dyspnoea and vertigo. He denied alcohol intake but had injected methamphetamines and developed symptoms shortly after. 48 hours post-injection, he was drowsy and developed a complete internal and external ophthalmoplegia, an absent gag reflex with slow tongue movements and facial diplegia with sparing of distal muscle groups. His reflexes were initially present but became absent five hours later. There were no sensory or cerebellar findings. Blood cultures were taken given his history of intravenous drug use. Neuroimaging did not note the presence of a posterior circulation infarct. A lumbar puncture did not demonstrate cytoalbuminological dissociation, and antibody testing for myasthenia gravis and Guillain Barre Syndrome variants were negative, although he was commenced on empirical IVIg prior to these results. He required intubation on day three of admission due to hypoxia secondary to aspiration but needed minimal ventilatory support. Neurophysiological findings were consistent with a presynaptic neuromuscular junction disorder. Blood cultures noted clostridium botulinum with detection of clostridium botulinum toxin B. Antitoxin was administered on day four of presentation with slow but gradual improvement in his neurology and he was extubated on day 16. Conclusion | Acute causes of bilateral ophthalmoplegia include Wernicke’s encephalopathy, Miller- Fisher Syndrome (MFS) variant of Guillain Barre Syndrome, brainstem stroke, myasthenia gravis and botulism. Of these, only MFS and botulism will cause both internal and external ophthalmoplegia. 2037 An integrated neurogenomics clinic – 28-months experience and outcome of a tertiary referral centre Alison McLean1, 2, Michel Tchan3, 4, Sophie Devery1, Renee Smyth1, Kishore Kumar1, 5, 6, Susan Tomlinson1, 4, 7, Stephen Tisch1, 7, 2, Kathy Wu1, 4, 7, 2 1. St Vincents Hospital, Sydney, Darlinghurst, NSW, Australia 2. Faculty of Medicine, University of New South Wales, Sydney, NSW, Australia 3. Westmead Hospital, Sydney, NSW, Australia 4. The University of Sydney, Sydney, NSW, Australia 5. The Garvan Institute of Medical Research, Sydney, NSW, Australia 6. Molecular Medicine in Neurology, Concord Repatriation General Hospital, Sydney, NSW, Australia 7. School of Medicine, University of Notre Dame, Sydney, NSW, Australia Objectives | To retrospectively review attendance and outcomes of a single centre, integrated multi- disciplinary (MDT) neurogenomics clinic at St Vincent’s Hospital, Sydney. Methods | An audit of patients who attended the neurogenomics clinic was conducted over a 28-month period from 2017 to 2020. The clinic comprises of neurologists, clinical geneticists and genetic counsellors assessing each of the patients concurrently during the consultation. Results | In the audit period 99 patients were referred spanning 45 different clinical diagnoses. Following MDT assessment, 23% (23/99) of referring diagnoses were revised. Seventy-nine patients (80%) underwent genetic testing. The type of genetic tests ordered includes 41 exome-based panels, 14 whole genome sequencing, 13 single gene tests, 30 repeat expansion disorders and 2 chromosomal 125 microarrays. Molecular confirmation was achieved in 22 patients following testing a yield of 28% (22/79); of which, 2% had their clinical diagnosis revised following testing. Overall, a diagnosis was achieved in 29/99 patients (29%), of whom 7 patients’ diagnosis was achieved without genetic testing. From referral to the results of genetic testing, 25% (25/99) of patients had their diagnosis revised as a result of MDT input. Conclusions | Provision of an integrated multidisciplinary neurogenomics clinic in a tertiary setting provides an invaluable service with a diagnostic yield of 28%. This model provides a gold standard for diagnostic evaluation of patients with suspected neurogenetic disorders. Psychosocial benefits for patients, such as convenience/satisfaction of an MDT clinic, psychological closure for patients/families, and reproductive options enabled by achieving a genetic diagnosis, will be audited via patient survey. 2038 Atypical Presentations and Course of JC Virus Infection Sophie Chatterton1, 2, Liam Dwyer3, Claire Thomson3, Bruce J Brew4, 1, 5, 6, 7 1. Neurology, St Vincent’s Hospital, Sydney, NSW, Australia 2. University of New South Wales, Sydney, NSW, Australia 3. Lung Transplantation, St Vincent’s Hospital, Sydney, NSW, Australia 4. Royal North Shore Hospital, Brighton-Le-Sands, NSW, Australia 5. University of Notre Dame, Sydney, NSW, Australia 6. Neurosciences Program and Peter Duncan Neurosciences Unit, St Vincent’s Centre for Applied Medical Research, Sydney, NSW, Australia 7. Department of Neuroscience, International Society for NeuroVirology, Temple University School of Medicine, Philadelphia, United States of America Objective | There is increasing evidence that the spectrum of JC virus (JCV) CNS disease includes novel syndromes other than Progressive Multifocal Leukoencephalopathy (PML), the appreciation of which is increasingly important in the context of MS therapies and immunodeficiency states. Our objective is to describe unusual presentations of JCV infection to heighten clinician awareness. Method | Three case reports. Results | A 56 year-old male HIV+ with decades of viral suppression and normal immune function presented with 1 month of non-specific headache that spontaneously resolved despite an MRI showing a new area of PML and CSF being JCV DNA+. He had had two similar episodes in 2003 and 2014 with MRI scans consistent with PML, CSF JCV PCR positivity once and brain biopsy positive twice. Another 61 year-old male presented with subacute binocular vision loss and was found to have newly diagnosed HIV and JCV DNA detected in CSF. MRI brain only demonstrated symmetrical chiasmo-hypothalamic enhancement. There has been some improvement after cART and steroids for IRIS. Thirdly, a 65 year-old presented with subacute progressive confusion and behavioural disturbance, one year post bilateral lung transplantation. MRI brain demonstrated no evidence of PML but CSF on three occasions demonstrated a progressively increasing JCV DNA load. Despite reduction in his immunosuppression the patient developed profound encephalopathy without localising features leading to death two months later. Conclusion | These cases emphasize the atypical presentations of JCV: chronic relapsing, unusual symmetrical visual pathway disease, and non-localizing encephalopathy without MRI evidence of PML. 2039 Clinical variation in the use of disease-modifying therapies for multiple sclerosis between different states and territories in Australia, 2019–20 Kieren Po1, 2, Michael H Barnett2, 3 1. Sydney Medical School, The University of Sydney, Sydney, NSW, Australia 2. Department of Neurology, Royal Prince Alfred Hospital, Camperdown, NSW, Australia 3. Brain and Mind Centre, The University of Sydney, Camperdown, NSW, Australia 126 Objectives | In Australia, patient costs for multiple sclerosis disease-modifying therapies (DMTs) are government-subsidised under the Pharmaceutical Benefits Scheme (PBS). Unlike many countries, any DMT can be selected for a patient who fulfils treatment criteria. We aimed to determine whether clinical variation exists in the use of DMTs in different Australian states and territories. Methods | DMT usage was determined from PBS prescription dispensing statistics for all DMTs available in Australia from July 2019 to June 2020.1 Pa Results | There were 176,163 prescriptions for DMTs dispensed for an estimated 18,596 patients, with a total drug cost of AU$344.1 million and mean drug cost per patient of AU$18,501. Most patients were treated with oral (41.7%) or infusion (33.8%) maintenance DMTs. Fewer received platform injectable DMTs (15.3%) or immune reconstitution DMTs (9.1%) in the study period. The most common DMTs were fingolimod (23.5%), ocrelizumab (21.8%), natalizumab (12.1%), and dimethyl fumarate (10.8%). Platform injectable DMTs were most common in ACT (20.1%) and least common in Victoria (13.0%). Oral DMTs were most common in NSW (44.0%) and least common in Tasmania (29.3%). Infusion DMTs were most common in Tasmania (47.4%) and least common in ACT (27.7%). Conclusion | We have demonstrated marked variation in DMT use in different Australian states and territories during the study period. The use of DMTs in Australia has evolved over time,3 but differences between states/territories remain unexplained. We plan to investigate the observed clinical variation in future studies. 1. Department of Health. Pharmaceutical Benefits Scheme. Canberra: Australian Government; 2021. https://www. pbs.gov.au/ (accessed 19 Feb 2021). 2. WHO Collaborating Centre for Drug Statistics Methodology. ATC-DDD Toolkit: Defined Daily Dose (DDD). Geneva: World Health Organization; 2021. https://www.who.int/tools/atc-ddd-toolkit/about-ddd (accessed 19 Feb 2021). 3. Goudarzi MH, et al. Disease modifying therapies for relapsing-remitting multiple sclerosis: use and costs in Australia (1996-2019). Mult Scler Relat Disord 2021;50:102835. 2041 Painless progressive mononeuritis multiplex secondary to AML associated neuroleukaemiosis Nimalan Harinesan1, Rajiv Wijesinghe1, Nicole Wong-Doo2, 3, Katrina Morris1, 3, James D Triplett1 1. Neurology and Neurophysiology Department, Concord Repatriation General Hospital, Sydney, NSW , Australia 2. Haematology Department, Concord Repatriation General Hospital, Sydney, NSW, Australia 3. Concord Clinical School, The University of Sydney, Sydney, NSW, Australia Objectives | To report the clinical history, imaging and neurophysiology findings of a case of mononeuritis multiplex caused by acute myeloid leukemia (AML) neuroleukaemiosis Case | A 58-year-old male presented with painless and progressive mononeuropathies after completing high dose cytarabine consolidation treatment for AML. Nine days following chemotherapy a right third nerve palsy developed followed by right 5th, right 7th, left 3rd and left radial, ulnar and peroneal neuropathies. Serial MRI and PET imaging was unremarkable and 2 cerebrospinal fluid (CSF) were normal. Nerve conduction studies demonstrated abnormal right blink responses, a peroneal neuropathy and evidence of conduction block at a non-compressible site within the left ulnar nerve, however nerve ultrasound did not demonstrate any causative lesion. Differentials considered included opportunistic fungal infections and a paraneoplastic neuropathy. A third CSF sample performed on day 24 demonstrated myeloblasts, consistent with central nervous system leukaemic infiltration. A diagnosis of neuroleukemiosis was made and intrathecal chemotherapy (initial methotrexate [MTX] and cytarabine, followed by alternating MTX/cytarabine twice a week) plus systemic chemotherapy (fludarabine, cytarabine, idarubicin) and granulocyte- colony stimulating factor was commenced resulting in partial resolution of pre-treatment symptoms. Conclusions | Progressive neuropathies in patients with leukemia are rarely reported and can be diagnostically challenging. Mononeuritis multiplex associated with AML may be painless and focused 127 imaging may fail to demonstrate significant abnormalities. A high index of clinical suspicion is required as the differential diagnoses of neuroleukaemiosis is broad including paraneoplastic syndromes, infection and inflammatory conditions. As in this instance multiple CSF examinations maybe required to confirm its diagnosis. 2046 The impact of device-assisted therapy initiation on the gut microbiome in Parkinson’s disease Michal Lubomski2, 1, Xiangnan Xu3, Andrew J Holmes4, Jean Yang3, Carolyn M Sue2, 1, Ryan L Davis1 1. Department of Neurogenetics, Kolling Institute, University of Sydney and Royal North Shore Hospital, Sydney, NSW, Australia 2. Neurology Department, Royal North Shore Hospital, St Leonards, NSW, Australia 3. School of Mathematics and Statistics. Sydney Precision Bioinformatics, University of Sydney, Camperdown, NSW, Australia 4. School of Life and Environmental Sciences, The Charles Perkins Centre, University of Sydney, Sydney, NSW, Australia Objectives | Several studies have evaluated the impact of oral medication on the gut microbiome (GM) in Parkinson’s disease (PD). However, the impact of PD device-assisted therapies (DAT) on the GM remains to be investigated. We profiled acute temporal GM stability around the initiation of PD DAT. Methods | The GM of 21 PD patients initiating either Deep Brain Stimulation (DBS) or levodopa- carbidopa intestinal gel (LCIG) were compared to 10 spousal healthy control (HC) subjects. 16S amplicon sequencing of the V3-V4 region of stool bacterial DNA was used to compare temporal GM stability between groups and with clinical outcome measures, including disease alternations relative to therapy initiation. GM response to therapy in the PD group was assessed by comparing pre-therapy (-2 and 0 weeks) with post-therapy initiation timepoints (+2 and +4 weeks) and HCs at baseline (0 weeks). Results | Altered GM compositions were noted between the PD and HC groups at various taxonomic levels, including specific differences for DBS and LCIG therapies. Beta diversity changes were also identified across the 4 week post-treatment initiation period, implying a therapy-effect on the GM. Conclusions | We present the first acute longitudinal assessment of GM response to PD DAT. The pre-treatment PD-specific GM (consistent with previous studies) was altered following DAT initiation, indicating DATs have a modulatory impact on the GM in PD. 2047 Cryolipolysis-induced radial mononeuropathy Nimalan Harinesan1, Rajiv Wijesinghe1, Michael Hayes1, James D Triplett1 1. Neurology and Neurophysiology Department, Concord Repatriation General Hospital, Sydney, NSW, Australia Objectives | To report an association between cryolipolysis or “fat-freezing” in the upper arm and development of an acute radial neuropathy likely secondary to a combination of thermal and pressure effects. Results | A 31-year-old female presented with an acute right wrist drop that occurred following a cryolipolysis procedure to the upper right arm. Paraesthesia and numbness occurred towards the end of a 40-minute cryolipolysis procedure, with weakness reported within 24 hours. Examination of the arm 1 week following symptom onset revealed a significant ecchymosis at application site of the fat freezing device. In addition, there was severe weakness of right elbow, wrist and finger extension with an anaesthetic patch over the anatomical snuff box. Ultrasonography showed fascicular oedema of the radial nerve in the upper arm. Nerve conduction studies confirmed an acute axonotmetic radial neuropathy at the spiral groove. The patient was referred for hand therapy and at 4 months regained most of the function in her hand, with some mild persistent sensory impairment. 128 Conclusions | Cryolipolysis is a cosmetic treatment that aims to locally reduce subcutaneous fat. The procedure is performed using a vacuum applicator to cool the selected area to temperatures as low as -9 degrees Celsius. Peripheral neuropathies following the procedure have been rarely described1 but, to our knowledge, this is the first report of an acute neuropathy developing during the procedure. The causative mechanisms of cryolipolysis-induced nerve injury in this case were likely due to nerve compression related to local oedema and thermal effect on the radial nerve. 1. Jong Gyu Baek, Jung A Park, Jung Im Seok. (2017) Radial Neuropathy after Cryolipolysis. Journal of the Korean Neurological Association 35:1, 30-32. 2050 CJD and motor neuron disease: a growing association Adam P Cooper1, Wilson Vallat1 1. Neurology Department, Lyell McEwin Hospital, Adelaide, SA, Australia Introduction | Amyotrophy in Creutzfeldt-Jakob Disease (CJD) is rarely a conspicuous clinical finding. The overlap of CJD (a prionopathy) and motor neuron disease is reported in the literature but it remains to be established whether the neuronopathy is an integral part of CJD presentation, or, whether this represents a distinct variety. Our case describes a male patient with clincopathological diagnosis of sporadic CJD along with evidence of motor neuronopathy on nerve conduction studies. Case summary | At presentation to our neurology service, the patient was a 72-year-old male, living at home with his wife. He was initially referred for progressive short-term memory loss, personality change, and gait disturbance. On review, it was noted that in addition to gait and limb ataxia, and cognitive impairment, he demonstrated prominent generalised fasciculation. Nerve conduction and electromyography studies showed normal nerve conduction but fasciculations in proximal and distal muscle groups of the left upper and lower limbs, in keeping with a motor neuronopathy. CSF 14-3-3 and EEG provided little bearing. MRI demonstrated progressive T2-hyperintense, diffusion-restricted lesions in the bilateral basal ganglia, thalami and medial frontal cortices consistent with CJD. Post- mortem examination demonstrated spongiform encephalopathy and immunohistological staining (12F10) in-keeping with diagnosis of CJD. Conclusion | In our patient, the combination of clinical and neurophysiologic features of motor neuron disease and a confirmed diagnosis of Creutzfeldt-Jakob Disease raises the vexed question of whether this represents a distinct overlap syndrome or an infrequent manifestation of the same pathology. Further research is required to establish this. 2052 Acquired idiopathic generalised anhidrosis, a rare debilitating condition to be aware of Jennifer Nguyen1, Michaela Zallmann1, Robert Puy2, Elspeth Hutton3, Tasos Stavrakoglou1 1. Department of Dermatology, Alfred Health, Melbourne, VIC, Australia 2. Department of Allergy, Asthma and Clinical Immunology, Alfred Health, Melbourne, VIC, Australia 3. Department of Neurology, Alfred Health, Melbourne, VIC, Australia Acquired idiopathic generalised anhidrosis (AIGA) is a rare condition characterised by non-segmental anhidrosis affecting ≥25% of the body surface area (BSA) without underlying secondary causes of anhidrosis1. The pathophysiology of AIGA is unclear however may be related to autoimmune destruction of acetylcholine receptors in sweat glands. To date, more than 100 AIGA cases have been reported from Japan but only a few from the rest of the world2. Recently, diagnostic and management guidelines for AIGA have been published in Japan1. In our case series, six young male patients presented with generalised anhidrosis, debilitating skin pain and cholinergic urticaria. These patients described intense stubbing pain and burning sensation in a large BSA triggered by exposure to increased ambient temperature, exercise or emotional triggers. 129 AIGA diagnosis was confirmed with a thermoregulatory sweat test that showed anhidrosis >25% BSA, reduced sudomotor function in QSART test and no sweat production after 4% pilocarpine intradermal injection. Various treatments including antihistamine, omalizumab and systemic immunosuppression were not effective. Danazol which has been reported to be useful in refractory cholinergic urticaria but not previously reported in AIGA, was successful in significantly improving symptoms in four of our patients, with one more patient now commencing the medication. The incidence of AIGA may be underreported due to the lack of awareness or misdiagnosis. These patients are usually referred to dermatologists or allergists but also neurologists as the predominant symptom is intense stabbing pain and burning sensation. Given the debilitating effects, greater awareness, appropriate diagnosis and management is vital. 1. Munetsugu T, Fujimoto T, Oshima Y, Sano K et al. Revised guideline for the diagnosis and treatment of acquired idiopathic generalized anhidrosis in Japan. Journal of Dermatology. 2017: 44: 394-400. 2. Pargfrieder C, Struhal W, Sega W, Klein G et al. Acquired idiopathic generalized anhidrosis in a young Austrian patient. JAAD Case Reports. 2018: 4:222-5. 3. La Shell MS, England RW. Severe refractory cholinergic urticaria treated with danazol. J Drugs Dermatol. 2006 Jul-Aug;5(7):664-7. PMID: 16865874. 2060 Novel NOTCH1 variant in a patient with spontaneous internal carotid artery dissection. Leon Edwards1, 2, 3, Christopher Blair1, 2, 3, Sulekha Rajagopalan4, Madhura Bakshi4, Nathan Manning5, 6, 7, Alan McDougall1, 2, 3 1. Neurology and Neurophysiology, Liverpool Hospital, Liverpool, NSW, Australia 2. Ingham Institute for Applied Medical Research, Ingham Institute, Liverpool, NSW, Australia 3. South Western Sydney Clinical school, University of New South Wales, Liverpool, NSW, Australia 4. Clinical genetics, Liverpool Hospital, Liverpool, NSW, Australia 5. Institute of neurological sciences, Prince of Wales Hospital, Randwick, NSW, Australia 6. Neurointervention, Liverpool Hospital, Liverpool, NSW, Australia 7. The Florey institute of neurosciences, Melbourne, VIC, Australia Introduction | We report a case of spontaneous left internal carotid artery (ICA) dissection associated with a novel NOTCH1 variant. Case | A 43-year-old lady presented with a 3-day history of severe headache and transient expressive dysphasia. There was no history of preceding trauma. CT brain (CTB) and carotid angiography (CTA) demonstrated small areas of established subcortical infarction with occlusion of the M1-segment of the left middle cerebral artery (LMCA). CT cerebral perfusion (CTP) displayed a region of increased mean transit time and cerebral blood volume consistent with a large ischaemic penumbra. Digital subtraction angiography confirmed an occlusion of the LMCA with luminal irregularity of the supraclinoid ICA suggestive of arterial dissection. An intracranial stent was deployed from the superior M2-division of the LMCA to the cavernous ICA. Progress CTA and CTP demonstrated reperfusion of the LMCA territory. Six-months later, she remains well with no recurrence of symptoms or detectable neurological signs. Targeted gene panel demonstrated a novel heterozygous missense variant (c.56C>T;p.Ala19Val) in exon-1 of the NOTCH1 gene. Segregation testing demonstrated an identical variant in her mother. Conclusion | Spontaneous intracranial ICA dissection is a rare condition described mostly in single case reports. Mortality rates have been reported of up to 75%. The NOTCH-signalling pathway is involved in the embryonic development of arterial endothelium. NOTCH1 variants have been associated with autosomal dominant bicuspid aortic valve aortopathy, and rarely in extracranial arterial dissection. To our knowledge; this is the first reported case of intracranial dissection where a previously undescribed NOTCH1 variant is identified. 130 2061 “No End in Sight”, Management dilemma of refractory MOG antibody positive optic neuritis Samuel SK Kwok1, James JH Hughes1, Dark LD Lisa1 1. Tamworth Hospital, Oxley Vale, NSW, Australia Background | The patient is a 38-year-old lady who presented with impaired visual acuity in her right eye which was accompanied with pain on extra-ocular movements. Her symptoms initially resolved with high dose steroid therapy. • This is on a background of eosinophilic asthma which is refractory to maximal inhaler therapy and IL-5 monoclonal antibody therapy. Investigations and treatment progress | The patient had unremarkable blood results and inflammatory makers and a normal CSF study. She was subsequently found to be myelin oligodendrocyte glycoprotein (MOG) antibody positive but negative for (Aquaporin -4) AQP-4 antibodies. Radiological findings on MRI of the brain and spine was suggestive of multiple sclerosis (MS) • Whilst receiving intravenous immunoglobulin (IVIG) as a bridging therapy to Multiple Sclerosis (MS) modifying therapy, the patient developed severe adverse reaction to IVIG with desquamation of her hands and legs. Furthermore, she did not achieve satisfactory disease control with Ocrelizumab. • Has cushingoid features secondary to long term high dose steroid therapy. Goals and learning points of presentation | To highlight the challenges and difficulty in managing a patient with refractory MOG antibody optic neuritis and its impact on the quality of life of the patient • To demonstrate the gap in available clinical evidence in the management of recurrent MOG antibody positive optic neuritis • To initiate an open discussion regarding the treatment of these challenging group of patients 1. Chen JJ, Tobin WO, Majed M, Jitprapaikulsan J, Fryer JP, Leavitt JA, Flanagan EP, McKeon A, Pittock SJ. Prevalence of Myelin Oligodendrocyte Glycoprotein and Aquaporin-4-IgG in Patients in the Optic Neuritis Treatment Trial. JAMA ophthalmology 2018; 136(4): 419‐422. 2. Chen JJ, Flanagan EP, Bhatti MT, Jitprapaikulsan J, Dubey D, Lopez Chiriboga ASS, Fryer JP, Weinshenker BG, McKeon A, Tillema J-M, Lennon VA, Lucchinetti CF, Kunchok A,. Steroid-sparing maintenance immunotherapy for MOG-IgG associated disorder. Neurology 2020; 95(2): e111‐e120. 2062 Intraventricular migration of intraocular silicone oil Leon Edwards1, 2, 3, Christopher Blair1, 2, 3, Zeljka Calic1, 2, 3 1. Neurology and Neurophysiology, Liverpool Hospital, Liverpool, NSW, Australia 2. Ingham Institute for Applied Medical Research, Ingham Institute, Liverpool, NSW, Australia 3. South Western Sydney Clinical school, University of New South Wales, Liverpool, NSW, Australia Introduction | We report a case of intraventricular migration of intraocular silicone oil. Case | A 53-year-old male presented with a decreased level of consciousness. His past medical history included bilateral retinal detachment, atrial fibrillation and ischaemic stroke. Initial blood tests and chest X-ray were consistent with a community acquire pneumonia. He was started on a 5-day course of IV ceftriaxone and azithromycin with improvement in his symptoms. A CT brain was performed upon presentation given his conscious state. This demonstrated a high density in the right globe with tracking along the optic nerve and bilateral homogenous isointense spherical masses in the frontal horns of the lateral ventricles. Imaging was initially reported as bilateral vitreous haemorrhages, an optic chiasm lesion and intraventricular haemorrhage. Follow-up MRI brain confirmed the biventricular masses with evidence of a chemical shift artefact on T1 and T2 sequences. There was disappearance on the masses on fat-suppressed T1-sequences. Images were 131 reviewed in the combined neurology-neuroradiology meeting with the diagnosis made of cerebral migration of silicone oil. Conclusion | Intraventricular migration is a rarely reported complication following intraocular silicone endotamponade. The precise mechanism of migration is unknown. As silicone appears hyperintense on CT, it can mimic a haemorrhage or mass. Cases of misidentification of intraventricular silicone have resulted in protracted hospital admissions and surgical biopsy. Typical MRI findings and a comprehensive clinical history allow for accurate identification. Early and accurate recognition is critical to avoiding unnecessary investigation and patient morbidity. 2063 Cerebral venous thrombosis following lumbar puncture for optic neuritis and iron deficiency anaemia. Leon Edwards1, 2, 3, Ramesh Cuganesan4, Cecilia Cappelen-Smith1, 2, 3 1. Neurology and Neurophysiology, Liverpool Hospital, Liverpool, NSW, Australia 2. Ingham Institute for Applied Medical Research, Ingham Institute, Liverpool, NSW, Australia 3. South Western Sydney Clinical school, University of New South Wales, Liverpool, NSW, Australia 4. Radiology, Liverpool Hospital, Liverpool, NSW, Australia Introduction | We report a case of cerebral venous thrombosis (CVT) following a lumbar puncture (LP) for optic neuritis (ON) in a patient with concurrent iron deficiency anaemia. Case | A 32-year-old caucasian bank manager presented with a 14-day history of right retrobulbar pain exacerbated with eye movement and progressive complete visual loss. Gadolinium-enhanced MRI-brain confirmed an acute right ON. Initial blood tests demonstrated an iron deficiency anaemia and LP was performed with normal CSF findings. Five-days after dural puncture, she re-presented to hospital with a worsening headache followed by 3 witnessed generalised tonic clonic seizures. Repeat MRI-brain confirmed pachymeningeal enhancement, a right parietal cortical vein thrombosis and small intraparenchymal haematoma. Antiphospholipid antibodies, thrombophilic and vasculitic screens were negative. Treatment with Levetiracetam, IV heparin and warfarin was given. Seizures terminated and the headache resolved. She was discharged 7 days after admission. At follow up 8-weeks later she had returned to work and remained headache and seizure free. Conclusion | Optic neuritis is recognised by the international classification of headache disorders as a painful cranial nerve lesion. LP is often performed in the investigation of ON. CVT is a rare but potentially fatal complication of dural puncture. A few studies have identified an association between iron deficiency anaemia and venous thrombosis. There are no reports linking CVT with LP and iron deficiency anaemia. CVT should be considered in a patient with persistent headache and recent LP. Iron deficiency should also be excluded. Early recognition and treatment is important to prevent morbidity and mortality. 2064 Case Report: A role for Ocrelizumab for Multiple Sclerosis with Ocular Sarcoidosis? Ike Leon Chen1, Glenn Reeves2, Yun Tae Hwang1 1. Neurology Department, Gosford Hospital, Gosford, NSW, Australia 2. Coastal Neurology, Gosford, NSW, Australia Introduction | Multiple sclerosis causes a variety of clinical neurological features which may overlap with sarcoidosis with central nervous system involvement. Rarely, concurrent diagnosis of MS and sarcoidosis in an individual has been reported. Currently, best management strategy for these individuals with this dual pathology remains unknown. 132 Case | A 54-year-old man with a 14-year history of isolated ocular sarcoidosis, as well as type 2 diabetes mellitus, hypertension, and dyslipidemia, presented with ongoing right-hand paresthesia. Gadolinium-enhanced neuroaxis MRI imaging revealed comparatively stable non-enhancing appearances of previously known numerous punctate supratentorial subcortical white matter hyperintensities, and a new non-enhancing right ventrolateral C3/4 cervical spinal cord hyperintense lesion. The radiological pattern in combination with positive oligoclonal bands and elevated protein levels on cerebrospinal fluid testing, with normal serum ACE level, was thought to favour a diagnosis of multiple sclerosis (with McDonald criteria fulfillment), rather than neurosarcoidosis. In addition to his sarcoidosis treatment comprising prednisolone and hydroxychloroquine (later changed to mycophenolate), Ocrelizumab was selected as initial disease modifying therapy for his new relapsing remitting multiple sclerosis, due to postulated benefits for his sarcoidosis as well based on ocrelizumab’s similarity to rituximab, an established treatment option in refractory sarcoidosis. Conclusion | This case highlights several key issues: • Recognising the possibility of co-occurrence of CNS demyelinating disease in sarcoidosis • The diagnostic challenge of ascertaining new neurological changes in sarcoidosis • The challenge of managing concurrent inflammatory conditions of distinctly separate aetiology We propose that Ocrelizumab has potential utility as an option in streamlining therapeutic management of this dual pathology. 2070 Stroke-like migraine attacks after radiation therapy: Be SMART and order an EEG Elaine Pang1, Josephine Chan2, 1, 3, David Blacker1, Thomas Chemmanam1, Nicholas Lawn2, 4, 1, 3 1. Sir Charles Gairdner Hospital, Nedlands, WA, Australia 2. Fiona Stanley Hospital, Murdoch, WA, Australia 3. WA Adult Epilepsy Service, Perth, WA, Australia 4. Royal Perth Hospital, Perth, WA, Australia Background | Seizures may be a feature of SMART (stroke-like migraine attacks after radiation therapy) syndrome but their role in the pathophysiology and clinical presentation is unclear. Method | The clinical and investigation findings in a series of adult patients with SMART syndrome presenting primarily with seizures were reviewed. Results | Four patients with SMART syndrome presenting with seizures were identified (mean age 51 years). Mean time from radiation therapy to SMART syndrome was 22.5 years (range 15-32 years). Indications for radiation were primary brain tumour (three patients) and haematological malignancy (one patient). Two patients had a history of seizures prior to SMART syndrome. Three patients had headaches at presentation. All patients presented with focal impaired awareness seizures with motor features. One patient had refractory non-convulsive status epilepticus requiring intravenous anaesthesia. Three patients had persistent negative motor deficits, associated with ongoing electrographic seizures with no clinical correlate, only identified on repeated EEGs or continuous EEG (cEEG). All patients failed initial anti-seizure medications (ASM), requiring a mean of five ASMs for seizure control. All patients had enhancing cortical MRI changes consistent with SMART syndrome that corresponded to the clinical deficit and ictal changes on EEG. At follow-up all patients improved but had persistent neurological deficits. Conclusion | SMART syndrome presents with seizures and less frequently status epilepticus and may be the basis for the associated clinical features and radiologic abnormalities. Judicious use of EEG and where necessary CEEG to identify non-convulsive seizures should be considered in patients with SMART syndrome presenting with prolonged neurological deficits. 133 2071 Optic Perineuritis in Giant Cell Arteritis Elaine Pang1, 2, Neha Irani1, 2, 3, 4 1. Fiona Stanley Hospital, Murdoch, WA, Australia 2. Sir Charles Gairdner Hospital, Nedlands, WA, Australia 3. Fremantle Hospital, Fremantle, WA, Australia 4. Joondalup Health Campus, Perth, WA, Australia Optic perineuritis (OPN) is a rare orbital inflammatory disease which targets the optic nerve sheath. Although OPN is predominantly idiopathic, it can be secondary to an array of inflammatory, infective or malignant conditions, including giant cell arteritis (GCA). We describe a case of a 75-year-old man with a four-week history of headaches with associated periorbital swelling, and monocular decrease visual acuity without significant constitutional or systemic symptoms. This was in the context of initially normal ESR and CRP. MRI head demonstrated bilateral OPN and GCA was subsequently confirmed based on temporal artery biopsy. He was managed with high dose prednisolone and upadacitinib. This case highlights the perineuritis as a rare manifestation of GCA. 2072 Developing a Quality Assurance Framework for Neuro-ophthalmology Referrals using NODE – the Neuro-ophthalmology Database Anoushka Lal1, Olga Roche 1, Owen White 1, Wendy Wang1, Shivanand Sheth2, Rahul Chakrabarti2, Lana del Porto 2, Anthony Fok 2, Neil Shuey2, Clare Fraser 3, Lionel Kowal 2, Anneke Van der Walt1 1. Department of Neuroscience, Central Clinical School, Monash University, Melbourne, VIC, Australia 2. Royal Victorian Eye and Ear Hospital, Melbourne, VIC, Australia 3. Sydney Eye Hospital, Sydney, NSW , Australia Objective | Quality assurance (QA) in neuro-ophthalmology (NOPH) is often lacking. The QA registry, NODE (Neuro-ophthalmology Database), was established and implemented in tertiary NOPH clinics in Australia. We developed a consensus on triage categories according to Australian standardised triage categories;P1 (consult<=30 days), P2 (consult<=30-60 days) and P3 (consult>60 days). Methods | Data on 410 patients at Alfred Hospital, Melbourne was collected with NODE. We developed a consensus on assignation of NOPH conditions to triage categories using recommendations from a panel of neuro-ophthalmologists with the modified Delphi approach. The average days from referral to triage and triage to the initial consultation were compared to the developed triage category standard. Results | Most patients presenting to the service were female (n=262, 64%), aged 21 to 30 years. Common diagnoses were Idiopathic Intracranial Hypertension, IIH (24%), Optic Neuropathy, ON (17%), Headaches, (11%) Cranial Nerve Defects, CND (9%) and Eye Movement Disorders, EOMD (9%). The mean time from referral to triage was <2 days for all the common NOPH conditions. The mean time (days, +-standard deviation) from P1 category triage to initial consult for IIH was 26 (±7), ON 27 (±11), and CND was 17 (±5). The mean time (days) from P2 triage to initial consultant for Headaches was 27 (±12), and EOMD was (±17). The mean time (days) from P3 triage to initial consultant for Myasthenia Gravis was 30 (±10). Conclusion | We have established a consensus agreement on triage categories for neuro- ophthalmological conditions. We established a QA framework for other NOPH clinics in Australia. 2073 A diagnostically challenging case of sciatic mononeuritis Laura I Rudaks2, 1, Jonathan Baird-Gunning2, Emily Sutherland2, Mark Thieben2, 3 1. Concord Repatriation General Hospital, Concord, NSW, Australia 2. Royal North Shore Hospital, St Leonards, NSW, Australia 3. Neurology Department, Northern Beaches Hospital, Frenchs Forest, NSW, Australia 134 Introduction | We describe a patient presenting with sciatic neuritis. Diagnostic possibilities included mononeuritis secondary to rheumatoid vasculitis, IgA-related vasculitis or TNF-α inhibitor therapy. Case | A 41 year old lady presented with acute neuropathic pain in her right leg and a foot drop. She had a two-year history of seronegative rheumatoid arthritis controlled with etanercept, a 15-year history of episodic haematuria, presumed due to IgA nephropathy, and a 15-year history of recurrent episodic lower limb rash. One month prior to her presentation, she had a gastrointestinal illness. On examination she had 0/5 power on right ankle dorsiflexion, eversion, and mild weakness of knee flexion and hip extension. Reflexes were normal. Sensation was impaired in a common peroneal distribution. Delayed nerve conduction studies showed absent right peroneal motor and sensory, and sural responses. MRI demonstrated right sciatic neuritis. Nerve biopsy was not undertaken. She received intravenous methylprednisolone, followed by tapering prednisolone and etanercept was ceased. Weakness did not improve and she commenced intravenous immunoglobulin. This was discontinued due to haematuria with infusions. Etanercept was reintroduced. She clinically improved over months, with mild residual weakness of great toe dorsiflexion only and reduced sensation over the dorsal foot. Conclusions | Mononeuritis may occur with rheumatoid vasculitis, TNF-α inhibitor use or IgA- mediated vasculitis [1,2,3]. Differentiating the cause is important in cases, such as ours, with underlying autoimmune disorders refractory to other immunomodulators. In our case, although not confirmed, the suspected aetiology was rheumatoid vasculitis given improvement occurred after resuming etanercept. 1. Makol, A., Crowson, C., Wetter, D., et al. Vasculitis associated with rheumatoid arthritis: a case-control study. Rheumatology 2014; 53: 890 2. Sokumbi, O., Wetter, D., Makol, A, et al. Vasculitis Associated With Tumor Necrosis Factor-Inhibitors. Mayo Clin Proc 2012; 87: 739-745 3. Park, M., Lee, Y and Choi, YC. Henoch-Schönlein Purpura Presenting as Mononeuritis Multiplex. J Clin Neurol 2018; 14: 112-114 2076 Embouchure dystonia complicating a case of focal cerebral vasculitis Ariadna Fontes-Villalba1, Suran Fernando2, John Parratt 1 1. Neurology , Royal North Shore Hospital, Sydney, NSW, Australia 2. Department of Immunology, Royal North Shore Hospital, Sydney, NSW, Australia Background | Primary central nervous system vasculitis (PCNSV) is a rare disorder that normally develops with bilateral brain lesions. We report a case of pathologically confirmed hemispheric PCNSV with associated brain atrophy and symptoms of focal dystonia. Method | case report. Result | A 45-year-old female presented with fluctuating apraxia, dystonia, cognitive dysfunction and numbness of the face. She had embouchure dystonia. She was taking lamotrigine for a longstanding history of well controlled partial seizures. A cerebral MRI revealed multiple left hemispheric cortical and subcortical white matter lesions. Many of the lesions enhanced. The lesions came and went over a period of three years but cortical atrophy of the fronto-parietal gyri developed. The CSF demonstrated matched oligoclonal bands. The autoimmune serology was negative. A cerebral PET demonstrated hypo-metabolism in the affected areas.A cortical lesion that was enhancing was biopsied and the histology showed several small foci of punctuate necrosis (infarcts) with lymphohistiocytic infiltration of blood vessels consistent with small vessel vasculitis. The patient was treated with prednisone and mycophenolate and improved clinically, albeit her clarinet playing did not return to pre-morbid levels. The MRI lesions resolved and did not recur. Conclusion | Focal vasculitis is rare but may result in neuronal loss and specific cortical damage and atrophy, in this case leading to embouchure dystonia. 135 1. Adult primary central nervous system vasculitis. Salvarani C, Brown RD Jr, Hunder GG. Lancet. 2012 Aug 25;380(9843):767-77. 2. Unilateral hemispheric primary angiitis of the central nervous system. Ho MG, Chai W, Vinters HV, Hathout G, Mishra S, Yim C, Valdes-Sueiras M, Nishimura R. J Neurol. 2011 Sep;258(9):1714-6. 3. Unihemispheric central nervous syste 2077 Lambert Eaton Myaesthenic Syndrome in the Absence of Malignancy Joseph B Stockwell1, Leon S Edwards1, Daniel Wardman1, Neil Griffith1 1. Neurology, Liverpool Hospital, Sydney, NSW, Australia Introduction | We report a case of Lambert Eaton Myaesthenic Syndrome (LEMS) in an 85-year-old gentleman with no active malignancy. Case | An 85-year-old gentleman presented with a 3-month history of proximal weakness, confusion, nausea and vomiting. His medical history included gastric adenocarcinoma with curative resection 21-years ago and a 2-year history of a stable sensorimotor peripheral neuropathy. During his admission he experienced an episode of new onset fluctuating diplopia. Neurological examination demonstrated mild upper and lower limb non-fatiguable weakness. There was no detectable cranial nerve palsy. A myasthenia antibody panel was ordered. Voltage-gated-calcium-channel-antibodies were positive (47pM) (normal range < 30pM). Repetitive nerve stimulation demonstrated an increment in compound muscle action potential of the right nasalis and right abductor digiti minimi following exercise and high-rate stimulation consistent with the clinical diagnosis of LEMS. Investigation for malignancy including tumour markers, CT chest, abdomen and pelvis, MRI-pancreas and whole body PET scan were unremarkable. The patient underwent a 1-month period of inpatient rehabilitation and was discharged home. At 6 months, he remains well with no further episodes of diplopia or weakness. To date, no malignancy has been identified. Conclusion | LEMS in absence of an identified malignancy is an uncommon diagnosis. Those cases that have been documented are also more likely to occur in younger patients. The case we present here highlights a constellation of vague seemingly discordant symptoms with a unifying diagnosis and offers the patient a chance to be actively monitored for the development of malignancy in the future. 2078 An undifferentiated autoimmune neuroinflammatory illness associated with low CSF hypocretin & central hypothalamic dysregulation Hoang-Mai Dinh1, Nicholas Gazy1, Logan Gardner1, 2, Stephen Tisch1, Andrew Carr1 1. St Vincents Hospital Sydney, Sydney, NSW, Australia 2. Westmead Hospital, Sydney, NSW, Australia Introduction | This case report explores a possible undifferentiated autoimmune neuroinflammatory illness presenting with recurrent fevers, abdominal pain, hypersomnolence and sleep attacks with low cerebrospinal fluid (CSF) hypocretin, and a partial response to anakinra, a human interleukin 1 receptor antagonist. Case presentation | A 19 year old female presented with 5 years of abdominal pain and fatigue with no clear aetiology identified following extensive investigation. She subsequently was found to have recurrent fevers to 38°C, an intermittent fine, macular rash and sudden sleeping at inappropriate times. Her brain MRI was normal and (CSF) showed normal protein and no white cells, but a low hypocretin level (<200 units). Further investigations including whole exome sequencing, gastrointestinal, autoimmune and metabolic assessments, yielded limited findings. Previous therapy with colchicine had been ineffective. 136 Management and outcome | For a presumptive diagnosis of an undifferentiated autoinflammatory disorder, she was received prednisolone 10 mg daily for 4 weeks with no benefit. She then initiated anakinra, which improved in rash and sleep attacks. Despite initially controlling her recurrent fevers for a period of four weeks, this symptom ultimately recurred, with ongoing abdominal pain. Discussion | Low levels of hypocretin in the CSF has been associated with narcolepsy type 1 and has thought to been associated with an undefined autoimmune mechanism. It is hypothesised that her hypothalamic orexin has been altered due to these inflammatory changes leading to body temperature dysregulation and sleep disorder. Interestingly the hypersomnolence appear to have improved with anakinra, a therapy not typically used in narcolepsy. 2079 Overlapping autoimmunity: a case of concomitant Aquaporin-4 and Myelin Oligodendrocyte Glycoprotein (MOG) antibody positivity in Neuromyelitis Optica Spectrum Disorder Miriam Wronski1, 2, Justine J Wang2 1. Royal North Shore Hospital, Sydney, NSW, Australia 2. St George Hospital, Sydney, NSW, Australia Objectives | To describe a rare case of double antibody positive Neuromyelitis Optica Spectrum Disorder (NMOSD) with both Aquaporin-4 and MOG antibodies, occurring following a Pertussis infection in a patient with a history of auto-immunity. Methods | Retrospective review of clinical records. Results | A 41-year-old Chinese woman with a history of Systemic Lupus Erythematosus presented with a sub-acute onset of progressive gait ataxia and urinary retention occurring seven days after a confirmed Bordetella pertussis infection. Magnetic resonance imaging revealed extensive subcortical and thalamic T2/FLAIR hyperintensities with subtle enhancement, and a longitudinally extensive non- enhancing spinal cord lesion (T1-T7), without optic nerve involvement. Cerebrospinal fluid protein was raised (0.55 g/L) with 7 mononuclear cells and matched oligoclonal bands. Viral PCRs were negative including JC virus and Pertussis. Established live cell-based immunoassays revealed positivity for both Aquaporin-4 antibodies (in CSF and serum) and MOG antibodies in high titres. Our patient meets the 2015 Consensus Diagnostic Criteria for NMOSD.1 Treatment with high dose corticosteroids and rituximab lead to clinical and radiological improvement, but she had a clinical relapse 10 months later with new LETM (T3-T7), necessitating increased immunosuppression with more rigorous rituximab dosing of 1000mg every 6 months. Conclusions | Double positivity for both Aquaporin-4 and MOG antibodies in NMOSD is rare. We describe a case of double-positive NMOSD occurring following an infective illness. This case demonstrates that NMOSD may occasionally masquerade as post-infectious Acute Disseminated Encephalomyelitis and highlights the importance of checking antibodies in these patients, given the treatment strategies and risk of relapse differs considerably. 2080 Anti-Leucine-rich glioma inactivated 1 (LGI1) encephalitis associated with high grade papillary urothelial carcinoma Jessica Stabler1, David Williams1 1. John Hunter Hospital, Newcastle West, NSW, Australia Introduction | LGI1 encephalitis is a rare form of limbic encephalitis, that was first recognised as a primary autoimmune phenomenon, and subsequently described in association with a limited number of malignancies1. We report a novel case of LGI-1 encephalitis occurring concurrent to a high-grade papillary urothelial carcinoma. 137 Case Presentation | A previously well 72-year-old male presented to a rural hospital with a first episode generalised tonic-clonic seizure, confusion and progressive behavioural change. He was diagnosed with LGI-1 encephalitis, with positive CSF antibodies, and mesial temporal T2 hyperintensity on MRI brain. There was no response to first line treatment with steroids, intravenous immunoglobulin, and mycophenolate. Malignancy screening revealed a lesion within the upper pole of the left kidney, favoured to represent a transitional cell carcinoma. Biopsy demonstrated a low grade papillary urothelial carcinoma. The patient’s encephalopathy continued to worsen over a period of months, despite ongoing immunosuppression. He underwent a left nephrouretectomy, and histology demonstrated a high-grade papillary urothelial carcinoma. Subsequent to this, there was improvement in cognition and behaviour. Psychotropic and immunosuppressive medications were slowly weaned. At 9-month follow-up, the patient has returned close to baseline function, and has been clinically stable off all immunosuppressive treatment. Conclusions | LGI-1 encephalitis has previously been described in association with thymoma, lymphoma, teratoma, and more recently with lung and prostate cancer2, 3. We believe our case is the first report of association between LGI-1 encephalitis and high grade papillary urothelial carcinoma. 1. Van Sonderen A et al. Anti- LGI1 encephalitis. Neurology Oct 2016, 87 (14): 1449-1456. 2. Navalli D, Mutalik NR, Jayalakshmi G. Leucine-Rich Glioma-Inactivated Protein 1 Antibody-Positive Limbic Encephalitis in a Patient with Adenocarcinoma of Prostate: A Case Report. Ann Indian Acad Neurol. Jan-Mar 2019: 22(1): 121-122. 3. Virupakshaiah A, Dalakas M, Desai N, Mintzer S, Ratliff J. A Report on LGI1 Encephalitis in association with Squamous Cell Carcinoma of the Lung. Neurology Apr 2019, 92 (15 Supp): 2.2-028. 2081 Contrast-induced encephalopathy after carbon dioxide angiography in the upper extremity and iodinated contrast – a case report Hue Mun Au Yong1, 2, Matthew K Ligtermoet3, Douglas Crompton3 1. Department of Neurology, Alfred Health, Melbourne, VIC, Australia 2. Department of Neurology, Royal Melbourne Hospital, Parkville, VIC, Australia 3. Department of Neurology, Northern Health, Epping, VIC, Australia Objective | Carbon dioxide(CO2) is used as an alternative contrast agent in angiography for patients with iodinated contrast allergy or impaired renal function. CO2 angiography is contraindicated in cerebral circulation based on demonstrated neurotoxicity in animals.1,2 We present a case of reversible neurological complications post CO2 angiography and iodinated contrast. Methods & Results | A 65-year-old man presented with an ischaemic finger from steal syndrome post-arteriovenous fistula ligation, on a background of end-stage renal disease and type 2 diabetes. He underwent CO2 angiography for evaluation of right upper limb circulation. Immediately after the first CO2 injection into the right brachial artery, he became unresponsive and spontaneously recovered after 1-minute. Due to poor distal opacification with CO2, 15ml of iodinated contrast was administered. At 3-hour post-procedure, he developed left facial droop, left hemiparesis and left visual neglect. CT brain, angiogram and perfusion study at 5-hour post-procedure showed no acute changes. Overnight, he worsened to dense left hemiplegia. Non-contrast CT brain at 11-hour post-procedure showed oedema and hyperdensity in the right hemisphere. He had a seizure on day 1 post-procedure. MRI brain performed 24-hour post-procedure showed dramatic resolution of right hemispheric cerebral oedema with no diffusion restriction. All neurological deficits completely resolved 7-day post-procedure. The CO2 which refluxed into the cerebral circulation from the brachial artery, caused the breakdown of blood-brain barrier, allowing penetration of iodinated contrast and subsequent right hemispheric cerebral oedema. Conclusions | This case highlights the risk of air embolism and neurotoxicity of CO2 angiography and the rare occurrence of contrast-induced encephalopathy. 1. Coffey R, Quisling RG, Mickle JP, et al. The cerebrovascular effects of intraarterial CO2 in quantities required for diagnostic imaging. Radiology 1984;151(2):405-10. doi:10.1148/radiology.151.2.6424174 138 2. Kozlov DB, Lang EV, Barnhart W, et al. Adverse cerebrovascular effects of intraarterial CO2 injections: development of an in vitro/in vivo model for assessment of gas-based toxicity. Journal of vascular and interventional radiology : JVIR 2005;16(5):713-26. doi: 10.1097/01.Rvi.0000153114.05700.61 [published Online First: 2005/05/06] 2084 A tail of two NMDA receptor antibody encephalitidies: aggressive treatment, divergent outcomes Christopher Blair1, Nicolas Urriola2, 3, Leon Edwards1, Patrick Aouad1 1. Department of Neurology & Neurophysiology, Liverpool Hospital, Liverpool, NSW, Australia 2. Department of Immunology, Liverpool Hospital, Liverpool, NSW, Australia 3. Department of Immunology, Royal Prince Alfred Hospital, Camperdown, NSW, Australia Background | Autoimmune encephalitis is an increasingly recognised disease that presents with seizures, neuropsychiatric symptoms, dystonic movements, and autonomic dysfunction1. As the mainstay of treatment immunosuppressive therapies are most effective when subject to early initiation and timely escalation, both of which are recognised to affect outcomes2. Nevertheless approximately half of patients with NMDA-R antibody encephalitis do not respond adequately to first- line therapy, and a significant proportion (12-30%) relapse2. Cases | A 19 year old lady presented with new-onset seizures and psychosis. EEG showed focal spike- and-wave discharges and MRI brain a focal area of restricted diffusion consistent with recent seizure activity. NMDA-R antibodies were present in both CSF and serum. Following early treatment with corticosteroid, plasma exchange, IVIG and rituximab the patient recovered, returning to college after 6 months. A 50 year old gentleman presented with a two day history of myalgias and confusion. EEG showed spike-and-wave discharges and MRI brain increased T2 signal in the mesial temporal lobes. NMDA-R antibodies were present in both CSF and serum. He was treated with corticosteroid, plasma exchange, IVIG and rituximab, and continued on oral prednisone and mycophenolate. Response to treatment was poor with persistent ongoing physical and cognitive impairment at 6 months. Serial MRI showed substantial (~30%) loss of parenchymal brain volume. Discussion | These cases illustrate that timely and aggressive management of NMDA-R antibody encephalitis with favourable prognostic markers is no guarantee of recovery. Several novel clinical and immunological predictors of response to therapy have been postulated, and currently await broader validation3. 1. Dalmau J, Graus F. Antibody-Mediated Encephalitis. N Engl J Med. 2018 Mar 1;378(9):840-851. 2. Titulaer MJ, McCracken L, Gabilondo I, Armangué T, Glaser C, Iizuka T et al. Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurol. 2013; 12(2):157-65. 3. Rüegg S, Yeh EA, Honnorat J. Forecasting outcomes in anti-NMDAR encephalitis: Clearer prognostic markers needed. Neurology. 2019 Jan 15;92(3):119-120. 2086 The diagnosis and management of five cases of spinal neurosarcoidosis Ariadna Fontes-Villalba1, Dayna Griffiths2, Natasha Gerbis3, Jonathan Baird-Gunning1, Patrick Aouad4, Suran Fernando5, John Parratt1 1. Neurology, Royal North Shore Hospital, Sydney, NSW, Australia 2. Neurology, Neurology Dept. Gosford Hospital, Gosford, NSW, Australia 3. Neurology, Northern Beaches Hospital, Frenchs Forest, NSW, Australia 4. Department of Neurology, Liverpool Hospital, Sydney, NSW, Australia 5. Department of Immunology, Royal North Shore Hospital, Sydney, NSW, Australia 139 Objectives | Neurosarcoidosis may present with longitudinally extensive transverse myelitis (LETM) posing a diagnostic challenge. We describe the clinical features, radiology and management of five patients with spinal neurosarcoidosis (SNS). Methods | We retrospectively identified five patients with a diagnosis of SNS and the clinical, radiological and pathological data were reviewed. Results | There were three females and two males who were, on average, 49 years at onset. A histopathological diagnosis of sarcoid was confirmed in two cases (lymph node and cerebral lesion) and the diagnosis was radiological in the others (LETM with persistent enhancement in 3 patients and the trident sign in 2 patients). The average span of the spinal cord lesions was 4 vertebral bodies. Three patients had FDG-avid mediastinal lymph nodes. Cerebral disease was identified in two cases, and cardiac involvement in one. Two patients required spinal decompression surgery. All patients received intravenous and oral steroids and some had rituximab (n=2), tocilizumab (n=1), cyclophosphamide(n=1), adalimumab (n=2), and infliximab (n=4). Disease control was achieved with TNF-alpha (tumour necrosis factor-alpha) blocking in 4 cases and another responded to cyclophosphamide. The mean follow-up was 55 months. Conclusions | SNS is a cause of LETM and can be suspected by the trident sign on MRI. Persistent enhancement may be another differentiating feature. Spinal cord oedema requiring surgery may occur and patients typically respond to treatment with TNF-alpha inhibitors. 1. Flanagan EP, Kaufmann TJ, Krecke KN, Aksamit AJ, Pittock SJ, Keegan BM, Giannini C, Weinshenker BM. Discriminating long myelitis of neuromyelitis optica from sarcoidosis. Ann Neurol 2016 Mar;79(3):437-47. 2. Zalewski NL, MD, Krecke KN, Weinshenker BG, MD, Aksamit AJ, Conway BL, McKeon A, Flanagan EP. Central canal enhancement and the trident sign in spinal cord sarcoidosis. Neurology. 2016 Aug 16;87(7):743-4. 2087 Pair-wise differences of penumbra and core volume estimates from three computed tomography perfusion software packages are influenced by site of large vessel occlusion Peter SW Park1, 2, Robbie Chan1, Channa Senanayake1, Stanley MK Tsui1, Alun Pope2, Helen M Dewey1, 2, Philip MC Choi1, 2 1. Department of Neurosciences, Box Hill Hospital, Eastern Health, Box Hill, VIC, Australia 2. Eastern Health Clinical School, Faculty of Medicine, Nursing and Health Sciences, Box Hill, VIC, Australia Objectives | Computed tomography perfusion (CTP) data are important for hyperacute stroke decision making. Comparisons between outputs of different CTP software packages are limited. We aimed to assess the pair-wise differences in infarct and penumbra estimates produced by three CTP software packages – MIStar, RAPID, and Vitrea. Methods | Consecutive patients with suspected acute ischaemic stroke who underwent CTP between July 2020 and June 2021 at our hospital were independently reviewed by two expert readers. Pair-wise differences between software estimates of penumbra and core volumes were calculated for each patient, with analysis stratified by large vessel occlusion (LVO) status (no-LVO, proximal M2, M1 and internal carotid artery-T [ICA-T]). Results | 580 CTP studies were performed; 262 were normal, 146 technically poor, with 172 included in the final analysis. 79/172 (45.9%) had LVO; proximal M2 (n=21), M1 (n=38) and ICA-T (n=20). Overall, statistically significant pair-wise differences were seen for both penumbra and core estimates (P <0.001). The largest difference in mean core estimates were seen between Vitrea and MIStar ([mean, 95% confidence interval] no-LVO [5.8ml, 3.2–8.4]; proximal M2 [10.4ml, 3.9–17.0]; M1 [17.7ml, 8.9–26.6]; ICA-T [38.9ml, 20.2–57.7]). More comparable penumbra estimates were observed between RAPID and MIStar (no-LVO [1.79ml, -3.9–7.51]; proximal M2 [13.1ml, -0.24–26.5]; M1 [10.7ml, -5.9–27.3]; ICA-T [28.4ml, 0.78–56.0]). 140 Conclusion | Core and penumbra volume estimates vary significantly between CTP software packages. There are minimal differences in patients with non-LVO stroke, with the greatest differences seen in patients with ICA-T occlusions. 2088 Botulinum toxin for a refractory head tremor arising from cerebellar metastases Christopher Blair1, Leon Edwards1, Dennis Cordato1 1. Department of Neurology & Neurophysiology, Liverpool Hospital, Liverpool, NSW, Australia Background | Tremor is an involuntary, rhythmic, oscillatory movement of a body part that can be a clinical manifestation of a range of underlying pathologies1. Of those tremor subtypes for which adequate management is often elusive, head tremor is among the most debilitating. Taking ‘yes- yes’, ‘no-no’, and mixed forms, available treatments for head tremor include medication, surgery, and botulinium toxin injections2. We report a case of severe head tremor arising from focal cerebellar metastases that showed a durable response to botulinium toxin treatments in a palliative setting, despite underlying disease progression. Case | A 60 year old lady was referred to our clinic with a 3 month history of ‘no-no’ head tremor. Originally diagnosed with ER- breast cancer in 2008, she underwent surgical resection but suffered a disease recurrence in 2017 when she presented with a solitary posterior fossa metastasis. This was resected and adjuvant radiotherapy was given, however in mid-2019 she developed first a left arm and then a coarse, persistent head tremor that severely limited her daily life. MRI brain revealed several new vermal and left cerebellar metastatic deposits. A combination of botulinium toxin injections to splenius capitis/sternomastoid and regular oral gabapentin effectively ameliorated her symptoms over three sessions. Discussion | Two open label studies and one RCT have shown that individualised botulinium toxin injections can be used to effectively treat essential head tremor2, and we demonstrate here that such an approach may also be a useful in the management of head tremor due to rarer and more aggressive aetiologies. 1. Louis ED. Diagnosis and Management of Tremor. Continuum (Minneap Minn). 2016 Aug;22(4 Movement Disorders):1143-58. 2. Mittal SO, Lenka A, Jankovic J. Botulinum toxin for the treatment of tremor. Parkinsonism Relat Disord. 2019 Jun;63:31-41. 2089 Delayed neurological worsening in an immunocompetent adult with Cryptococcus gattii meningoencephalitis Kristen Lefever1, Joel Corbett1, Nabeel Sheikh1, Helen Brown1 1. Queensland Health, Woollongabba, QLD, Australia Objective | While typically considered a condition of immunocompromised patients, Cryptococcus gattiimeningoencephalitis is increasingly observed in immunocompetent individuals, where the clinical outcomes are generally worse1,2. Methods | Case report Results | 24year old male represented with a three week history of progressively worsening headache, lethargy, generalised weakness, binocular diplopia, hearing loss and unintentional weight loss. Two weeks prior, he had presented with coryzal symptoms and received outpatient treatment for community acquired pneumonia. A lumbar puncture was performed with an opening pressure greater than 34cmH2O, pleocytosis and positive India ink stain. Cryptococcus gattii was cultured at a titre of 1:2048. MRI brain demonstrated bilateral basal ganglia change and leptomeningeal 141 enhancement consistent with Cryptococcal meningitis. Serum HIV was negative. Induction treatment with ambisome-flucytosine was initiated. Lumbar drain and subsequent VP shunt were required for management of persistent symptomatic increased intracranial pressure. After 6weeks of therapy he was transitioned to consolidation fluconazole. Repeat CSF demonstrated improved Cryptococcal Ag titre of 1:512. Two months into rehabilitation he suffered a seizure and rapid progressive neurological decline. EEG demonstrated a moderately severe diffuse encephalopathy. Repeat CSF cryptococcal Ag was stable. CSF limbic encephalitis and NMDA antibodies were negative. Repeat MRI brain demonstrated worsening supratentorial leptomeningeal enhancement and parenchymal vasogenic oedema, consistent with paradoxical upgrading reaction (PUR). Prednisolone 1mg/kg was initiated and the patient improved in days. Conclusions | PUR is an immune-reconstitution like event that can occur in immunocompetent patients. It represents an important cause of neurological deterioration in Cryptococcus gattii meningoencephalitis, requiring differentiation from relapse on consolidation therapy. 1. Franco-Paredes et al. (2014). Management of Cryptococcus gattii meningoencephalitis. Lancet Infect Dis. 15 (3): 348-355. 2. Chen et al. (2014). Cryptococcus gattii infections. Clin Microbiol Rev. 27 (4): 980-1024. 2090 Favourable outcome following early treatment with rituximab in a patient with probable Susac’s syndrome Yangyang (Erin) Xiao1, Elham Khalilidehkordi1, Po Sheng Yang1, Andrew Wong1, Claire Muller1 1. Neurology, Royal Brisbane and Women’s Hospital, Brisbane, QLD, Australia Objective | We report a favourable outcome following early treatment with rituximab in a patient with probable Susac’s syndrome (SuS) Background | Delayed treatment of SuS leads to significant morbidity, however there is no consensus in its management. Results | A 34-year-old man presented with severe headache, subacute confusion and blurred vision developing over 4 months. The MRI brain revealed multiple supratentorial and infratentorial FLAIR/T2 hyperintense lesions in white and gray matter, including characteristic corpus collosum “snow ball” lesions. The fundus fluorescein angiography (FFA) showed typical branch retinal artery occlusion, consistent with his bilateral decrease in visual fields. CSF showed high protein (3000mg/L) and pleocytosis (18X106/L). Following diagnosis of probable SuS, he was treated with high-dose corticosteroids on day 3 of presentation, followed by IVIG, mycophenolate and rituximab. He had significant improvement within 1 week. By 3 months, he returned to his neuropsychological baseline in majority of cognitive domains from moderate-severe dysfunction, with concurrent MRI demonstrating resolving white matter lesions and FFA showing less evident vasculitis. The treatment response was maintained with tapering of steroids (25mg at 12 months). He was able to return to his previous occupation as a paramedic by 1 year. Conclusion | SuS is a rare, immune-mediated microangiopathy in which early recognition with aggressive immunosuppression is required to achieve optimal outcome. No randomized controlled trial (RCT) exists for the management of this condition. In this report, early recognition through multidisciplinary input and aggressive immunotherapy with rituximab resulted in a favourable outcome. However, RCT evidence is needed to guide management. 142 2091 Clinical and neurophysiological improvement in Hereditary sensory and autonomic neuropathy type I (HSAN-1) following high dose serine therapy James D Triplett1, Garth Nicholson1, 2, 3, Con Yiannikas1, 2, 4 1. Department of Neurology and Neurophysiology, Concord Repatriation General Hospital, Sydney, Australia, Concord, NSW, Australia 2. Sydney Medical School, University of Sydney, Sydney, NSW, Australia 3. Northcott Neuroscience Laboratory, ANZAC Research Institute, Sydney, NSW, Australia 4. Department of Neurology, Royal North Shore Hospital, Sydney, NSW, Australia Objective | To report clinical stabilisation and improved summated compound motor action potentials (CMAP) in a patient with Hereditary sensory and autonomic neuropathy type I (HSAN-1) following high dose serine therapy. Case | A 52-year-old male presented in 2006 with a typical HSAN-1c phenotype and over the ensuing years had progressive distal to proximal sensory disturbance, associated lancinating pains, and mild progressive distal predominant limb weakness. A Ser384Phe mutation in the SPTLC2 gene located on chromosome 14q24 was identified in this patient in 2017. In late 2018 high dose serine therapy (11 grams TDS) was commenced, resulting in stabilisation of clinical weakness. Lower limb motor and sensory responses were absent at presentation in 2006 with initial summated bilateral upper-limb median and ulnar nerve CMAP being 31.2mV and steadily declining to 4.2mV in March 2019. Following Serine therapy, the summated CMAP increased to 5.4mV in August 2020 and 9.5mV in February 2021, furthermore previously absent upper-limb sensory nerve responses are now recordable. Conclusions | High dose Serine replacement therapy may lead to clinical stabilisation and improved neurophysiological parameters in HSAN-1. HSAN-1, an autosomal dominant sensory neuropathy occurs secondary to mutations in the enzyme Serine-Palmitoyltransferase (SPT), an essential enzyme in the de-novo synthesis of sphingolipids. The administration of high dose Serine may overcome altered SPT substrate specificity in HSAN-1, which preferentially uses L-alanine and L-glycine instead of L-serine and allow the formation of typical 1-deoxysphingolipids as opposed to atypical 1-deoxysphingolipids generally seen in this condition, with early treatment possibly preventing clinical progression. 2092 Man-in-the-barrel syndrome as a complication of cervical decompressive surgery Rajiv Wijesinghe1, Con Yiannikas1 1. Department of Neurology, Concord Repatriation General Hospital, Concord, NSW, Australia Objective | To present a case of painful brachial diplegia following cervical decompressive surgery. Case | A 73 year-old male presented with a 6 month history tripping over his left leg, resulting in near falls. An MRI demonstrated severe spondylotic cervical canal stenosis at C4/5 with myelomalacia and he subsequently underwent a cervical decompression and fusion at this level. On post-operative day five he developed severe pain in his neck and shoulders and mild weakness of his left arm. One week later he underwent a second operation with decompression and rhizolysis at C5 to C8. On post-operative day 3 he awoke with severe pain similar to previously, followed a day later by profound weakness of all movements around the shoulders bilaterally, but movements around the elbows, wrists and fingers were normal. While walking his arms hung limply beside him, giving him the appearance of a man in a barrel. Routine nerve conduction studies and median somatosensory evoked potentials were normal. Eelectromyography confirmed denervation within the C5 myotome bilaterally, however with selective sparing of the rhomboids bilaterally. This suggested a lesion distal to the branch to rhomboids, and a diagnosis of bilateral post-operative brachial neuritis was made. 143 Conclusion | Acute proximal arm weakness is an uncommon complication of cervical surgeries, referred to commonly as a post-operative C5 palsy. Investigations performed in this case however suggest that a brachial plexus lesion may be the cause of this peculiar syndrome. This syndrome may lie on the spectrum of post-surgical inflammatory neuropathies. 2094 Erroneously normal active B12 level in a case of subacute combined degeneration of the cord Nicholas Halliwell1, Cecily Forsyth2, Nicholas Taylor3, Sarah Mangalasseril2, Jonathan Sturm1 1. Neurology, Gosford Hospital, Gosford, NSW , Australia 2. Haematology, Gosford Hospital, Gosford, NSW, Australia 3. Clinical Biochemistry, Douglass Hanly Moir Pathology, Wentworth , NSW, Australia Objectives | Vitamin B12 is crucial for neurologic function, red blood cell production, and DNA synthesis. Deficiency can lead to a wide spectrum of haematologic and neuropsychiatric disorders including subacute combined degeneration of the cord. This report presents a case of a 47 y.o female who presented with subacute combined degeneration of the spinal cord with a normal active B12 level. Results | MRI demonstrated high T2 signal throughout the dorsal columns of the cervical and upper thoracic cord without enhancement consistent with subacute combined degeneration of the cord. Her blood count revealed a mild macrocytic anaemia. Total vitamin B12 was <80 pmol/L and active B12 was >128 pmol/L, confirmed on repeat testing. Functional vitamin B12 deficiency was confirmed by an elevated homocysteine level of 38.6 umol/L (reference range 4.4 to 13.6 umol/L) and elevated serum methylmalonic acid of 20.75 umol/L (reference range <0.32 umol/L). Intrinsic factor and gastric parietal cell antibodies were detected. Conclusion | Our patient had a clinical presentation consistent with B12 deficiency with an erroneously high active B12 level. Functional assays confirmed B12 deficiency, and a serological diagnosis of pernicious anaemia was made. This case illustrates the importance of not relying on any single test to exclude B12 deficiency. 2096 Paraneoplastic progressive-supranuclear palsy like brainstem syndrome associated with lung adenocarcinoma Natasha Gerbis1, Ariadna Fontes-Villalba2, Patrick Aouad3, 4, Suran Fernando2, 3, John DE Parratt2, 3 1. Northern Beaches Hospital, Frenchs Forest, NSW, Australia 2. Royal North Shore Hospital, St Leonards, NSW, Australia 3. University of Sydney, Sydney, NSW, Australia 4. Liverpool Hospital, Sydney, NSW, Australia Objectives | Progressive supranuclear palsy (PSP) is a neurodegenerative condition characterised by Parkinsonian features, cervical dystonia and ophthalmoparesis. Paraneoplastic PSP has previously been reported in the literature in association with several different cancer types but is very rare (Refs 1, 2, 3). Methods | Case review Results | A 74 year old Chinese man was diagnosed with Stage 1b EGFR positive lung adenocarcinoma and underwent a left upper lobectomy. Twelve months later he presented with rapidly progressive neck stiffness, reduction in motor function and gait (over 8 weeks) and recalcitrant disequilibrium. He had hypomimia, frontalis over-activation, blepharospasm, blepharotremor and a supranuclear gaze palsy. There was marked axial rigidity, bradykinesia and cervical dystonia. An MRI brain and spine were unremarkable. Vestibular function tests were normal. Serum antineuronal antibodies were negative. The CSF analysis was unremarkable. 144 The patient responded to plasma exchange on a two to three-weekly basis with significant improvement in saccadic eye movements and Parkinsonism. However, disequilibrium remained a persistent problem despite the discovery and excision of a second EGFR wild type non-small cell lung cancer, and Rituximab was recently started. Cervical dystonia was treated partially with Botulinum toxin injections, but the patient responded poorly to L-dopa. Conclusions | This suspected paraneoplastic disease exhibits several features of PSP. In particular, the supranuclear palsy, Parkinsonism and dystonia are similar to the typical syndrome. However, the rapidly progressive presentation and disequilibrium are unusual and a response to plasma exchange, suggests humorally mediated neuronal pathology. In rapidly evolving PSP-like cases with cancer, investigation for immunopathology is warranted. 1. Dash D, Choudhary R, Ramanujam B, Vasantha PM, Tripathi M. Paraneoplastic syndrome mimicking progressive supranuclear palsy. J Clin Neurosci. 2016 Oct;32:162-3. doi: 10.1016/j.jocn.2016.02.032. Epub 2016 Jun 16. PMID: 27318371. 2. Takkar A, Mehta S, Gupta N, Bansal S, Lal V. Anti- RI antibody associated progressive supranuclear palsy like presentation in a patient with breast carcinoma. J Neuroimmunol. 2020 Oct 15;347:577345. doi: 10.1016/j. jneuroim.2020.577345. Epub 2020 Jul 26. PMID: 32763584. 3. Tan JH, Goh BC, Tambyah PA, Wilder-Smith E. Paraneoplastic progressive supranuclear palsy syndrome in a patient with B-cell lymphoma. Parkinsonism Relat Disord. 2005 May;11(3):187-91. doi: 10.1016/j. parkreldis.2004.09.003. PMID: 15823484. 2097 Multiple sclerosis presenting as a rapidly progressive dementia Natasha Gerbis1, John DE Parratt2, 3 1. Northern Beaches Hospital, Frenchs Forest, NSW, Australia 2. Royal North Shore Hospital, St Leonards, NSW, Australia 3. University of Sydney, Sydney, NSW, Australia Objectives | Rapidly progressive dementias manifest with cognitive decline that progresses over weeks to months. It is rare for dementia to present early in the course of multiple sclerosis (MS). However, several case series on rapidly progressive dementia include a small number of patients with MS, and one small case series specifically examined dementia presenting within five years of MS diagnosis. Methods | Case study Results | A 66 year old woman presented to hospital with progressive left upper limb weakness and subacute cognitive decline over six to nine months. MRI brain revealed a right frontoparietal tumefactive lesion. Oligoclonal bands were detected in the CSF and a brain biopsy confirmed demyelination. The patient’s serum was tested in our research lab and revealed a novel antibody. The patient experienced a fluctuating clinical course but responded well to treatment with intravenous (IV) methylprednisolone, IV immunoglobulin (IVIG) and Cyclophosphamide. Unfortunately, further deterioration occurred five months after treatment with Cyclophosphamide and Rituximab was initiated. Clinical improvement in cognition was again observed, albeit briefly. Six months later, the patient died due to bronchopneumonia. The period from initial presentation to death spanned 30 months. A non-coronial autopsy was performed and revealed widespread incomplete cortical myelin loss and atrophy. Conclusions | Although a few cases have previously been reported in the published medical literature, dementia in the early stages of MS is extremely rare and may in fact be due to a distinct immunopathologic process. 1. Zhang, W et al, Analysis of clinical features for 8 patients with autoimmune dementia, Zhonghua Yi Xue Za Zhi. 2014 Feb 11; 94(5):359-63. 2. Mendes, MF et al. Early and severe cognitive impairment in multiple sclerosis, Dement Neuropsychol, 2012 Jan-Mar; 6(1); 48-52. 145 Poster Listing Posters are listed in alphabetical order by presenting author name. Number Title Authors 2010 The Effect of Head Cooling and Remote Ischemic Amir Moghadam Ahmadi, Conditioning on Improvement of Patients with Traumatic Fardin Hodoodi, Mohamad Brain Injury Allahtavakoli 1889 Prevalence of hyperammonemia in patients on Valproic Iqra Ali Acid therapy in tertiary care hospital of Karachi 1963 Pembrolizumab induced Lambert-Eaton Myasthenic Jasmine Ashhurst, Rami Syndrome Haddad, Rob Zielinski 1964 “Have I gone MAD?”: A case of Alice in Wonderland Jasmine Ashhurst, Rami Syndrome Haddad, Matthew Tait 1990 EBV and NMDA receptor antibody positive Opsoclonus- Catherine Ashton, Janavi Myoclonus Syndrome in an immunocompromised patient Dunuwille with renal clear cell carcinoma: a case report 1899 MR-based intramuscular fat fraction assessment in Stephanie L Barnes, Michael V hereditary sensory neuropathy type 1 Chan, Stephen Morris, Garth A Nicholson 1951 A case report of myelin oligodendrocyte glycoprotein Christopher Belder, Thomas antibody positive encephalitis mimicking HaNDL syndrome Kimber with subsequent acute optic neuritis 2015 Improving the quality of life for patients with chronic Linley Bielby, Kaylene Bastin, inflammatory demyelinating polyneuropathy (CIDP) Chris Akers, James Daly by offering home treatment with subcutaneous immunoglobulin (SCIg) 1955 Feasibility of Instituting Graduated High Intensity Training David J Blacker, Travis for Parkinson Disease (FIGHT-PD); a non-contact boxing Cruickshank, Mitchel Turner, exercise study Claire Tucak, Rai Fazio 2084 A tail of two NMDA receptor antibody encephalitidies: Christopher Blair, Nicolas aggressive treatment, divergent outcomes Urriola, Leon Edwards, Patrick Aouad 2088 Botulinum toxin for a refractory head tremor arising from Christopher Blair, Leon cerebellar metastases Edwards, Dennis Cordato Contactin-1-mediated chronic inflammatory demyelinating Shane Cameron, Rami Haddad polyneuropathy (CIDP) presenting as an acute case of Guillain Bare Syndrome (GBS) 2033 Neurological Presentations of Rare Chloromas in the Sophie Chatterton, Jacob Context of Haematological Malignancy Helou, James Drummond, Christopher Ward, Luke Coyle, Karl Ng 2038 Atypical Presentations and Course of JC Virus Infection Sophie Chatterton, Liam Dwyer, Claire Thomson, Bruce J Brew 146 Number Title Authors 2064 Case Report: A role for Ocrelizumab for Multiple Sclerosis Ike Leon Chen, Glenn Reeves, with Ocular Sarcoidosis? Yun Tae Hwang 1898 Multiple Cranial Neuropathies In A Patient With Syphilitic Melissa Chu, Shejil Kumar, Meningitis Jonathan Sturm 2006 Double trouble: Papilloedema secondary to idiopathic Antonia Clarke, Charles intracranial hypertension and aplastic anaemia Shuttleworth, Rachael Rodgers, Justine Wang 2050 CJD and motor neuron disease: a growing association Adam P Cooper, Wilson Vallat 1944 Neuropathic pain in multiple sclerosis: impact of spinal Joel Corbett, Peter Courtney, cord stimulation, an under-utilised modality? Mike Boggild 1945 Effectiveness and Tolerability of Perampanel in Epilepsy Wendyl D’Souza, Eugen Trinka, Patients Treated in Routine Clinical Practice: a Global Tony Wu, Imad Najm, Manoj Pooled Analysis Study Malhotra, Leock Y Ngo, Rob McMurray, Vicente Villanueva 2078 An undifferentiated autoimmune neuroinflammatory Hoang-Mai Dinh, Nicholas Gazy, illness associated with low CSF hypocretin & central Logan Gardner, Stephen Tisch, hypothalamic dysregulation Andrew Carr 1950 Impact of access to MRI on neurology inpatients in a Alexander E Dunn, Grace Swart, tertiary referral hospital James R Burrell 2060 Novel NOTCH1 variant in a patient with spontaneous Leon Edwards, Christopher internal carotid artery dissection. Blair, Sulekha Rajagopalan, Madhura Bakshi, Nathan Manning, Alan McDougall 2062 Intraventricular migration of intraocular silicone oil Leon Edwards, Christopher Blair, Zeljka Calic 2063 Cerebral venous thrombosis following lumbar puncture for Leon Edwards, Ramesh optic neuritis and iron deficiency anaemia. Cuganesan, Cecilia Cappelen- Smith 1995 A putative mechanism for subcortical aphasia Shadi El-Wahsh, David Greenup, Gemma White, Michael J Fulham, Arun Aggarwal, G. Michael Halmagyi 2076 Embouchure dystonia complicating a case of focal Ariadna Fontes-Villalba, Suran cerebral vasculitis Fernando, John Parratt 2086 The diagnosis and management of five cases of spinal Ariadna Fontes-Villalba, Dayna neurosarcoidosis Griffiths, Natasha Gerbis, Jonathan Baird-Gunning, Patrick Aouad, Suran Fernando, John Parratt 2003 Pituitary abscess - a case report Yi Chao Foong, Felicity Stringer 2025 Hallucinogenic Persisting Perception Disorder: A case Hannah HF Ford, Clare CF series and review of the literature Fraser, Emma ES Solly, Joanne JF Fielding, Owen OW White, Anneke Van der Walt 147 Number Title Authors 2096 Paraneoplastic progressive-supranuclear palsy like Natasha Gerbis, Ariadna brainstem syndrome associated with lung adenocarcinoma Fontes-Villalba, Patrick Aouad, Suran Fernando, John DE Parratt 2097 Multiple sclerosis presenting as a rapidly progressive Natasha Gerbis, John DE dementia Parratt 1972 Perampanel Monotherapy for the Treatment of Epilepsy: Antonio Gil-Nagel, James Evidence from a Clinical Trial (Study 342) and Real-World Wheless, Ji Hyun Kim, Robert T Use (Studies 504 and 506) Wechsler, Takamichi Yamamoto 1965 Post Hoc Analysis of Adjunctive Perampanel and Samantha Goldman, Leock Levetiracetam in Patients with Focal-Onset Seizures Y Ngo, Anna Patten, Manoj Co-administered With or Without Concomitant Enzyme- Malhotra Inducing Anti-Seizure Medications (EIASMs) 1905 The indications and utility of electroencephalogram, an Nicholas F Halliwell, Lucy audit of hospital practices Zhang, Stephan Golja, Ellie Skacel, Renee Pope, Callan A Coventry, Chinthuran Thilagarajan, Georgie CJ Dixson, Anna Schutz 2094 Erroneously normal active B12 level in a case of subacute Nicholas Halliwell, Cecily combined degeneration of the cord Forsyth, Nicholas Taylor, Sarah Mangalasseril, Jonathan Sturm 2004 Design of a novel Real World Evidence study of Mayzent Todd Hardy, Patrick Aouad (siponimod) onboarding and adherence in Secondary Progressive Multiple Sclerosis patients in Australia utilising the digital support platform MSGo 2041 Painless progressive mononeuritis multiplex secondary to Nimalan Harinesan, Rajiv AML associated neuroleukaemiosis Wijesinghe, Nicole Wong- Doo, Katrina Morris, James D Triplett 2047 Cryolipolysis-induced radial mononeuropathy Nimalan Harinesan, Rajiv Wijesinghe, Michael Hayes, James D Triplett 1992 Neurofilament light chain concentration predicts risk of Sarah Harris, Giancarlo Comi, relapse in participants with relapsing multiple sclerosis in Bruce A. C. Cree, Lawrence phase 3 ozanimod trials Steinman, James K. Sheffield, Harry Southworth, Ludwig Kappos, Jeffrey A. Cohen 1925 Ofatumumab Reduces Disability Progression Independent Suzanne Hodgkinson, Ludwig of Relapse Activity in Newly Diagnosed, Treatment- Naïve Kappos, Xavier Montalban, Patients with Relapsing Multiple Sclerosis Patricia Coyle, Jacqueline Nicholas, Sven Meuth, Bingbing Li, Krishnan Ramanathan, Wendy Su, Roman Willi, Dieter A Haring, Stephen Hauser 1932 An adult case of acute cerebellitis as a manifestation of Amanda Ji, Michael Roizman, Mycoplasma Pneumoniae infection Andrew Swayne 148 Number Title Authors 2061 “No End in Sight”, Management dilemma of refractory MOG antibody positive optic neuritis. 2072 Developing a Quality Assurance Framework for Neuro- Samuel Kwok, James JH ophthalmology Referrals using NODE - the Neuro- Hughes, Dark LD Lisa ophthalmology Database 1967 Efficacy and Safety of Perampanel as First Adjunctive Ji Woong Lee, Ji Hyun Kim, Therapy after First or Second Monotherapy in Patients with Dong Wook Kim, Amitabh Dash, Focal-Onset Seizures (FOS): Post Hoc Analysis of the FAME Manoj Malhotra Study 2020 Acute monoplegia Kristen Lefever, Jared Eisemann, Letitia Gore, Laura Clarke, Cullen O’Gorman, Andrew Swayne, Daniel Schweitzer 2021 A case of non-convulsive status epilepticus (NCSE) as first Luke Gagen, Kristen Lefever, presentation of sporadic Creutzfeldt-Jakob Disease (sCJD) Daniel Schweitzer, Laura Clarke, Cullen O’Gorman, Andrew Swayne 2089 Delayed neurological worsening in an immunocompetent Kristen Lefever, Joel Corbett, adult with ‘Cryptococcus gattii’ meningoencephalitis Nabeel Sheikh, Helen Brown 2007 Impact of Telehealth on multiple sclerosis (MS) outpatient Vivien Li, Ai-Lan Nguyen, clinics during the COVID-19 pandemic Izanne Roos, Katherine Buzzard, Chris Dwyer, Mark Marriott, Mastura Monif, Charles Malpas, Stefanie Roberts, Lisa Taylor, Elizabeth Carle, Nicola Taylor, Kelsey Tunnell, Trevor Kilpatrick, Tomas Kalincik 1923 Eosinophilic Granulomatosis with Polyangiitis presenting Julia Lim, Yi Chao Foong, with simultaneous central and peripheral nervous system Abhishek Malhotra involvement 1924 A Case of Chronic Inflammatory Demyelinating Julia Lim, Abhishek Malhotra Polyradiculoneuropathy Presenting as Unilateral Lumbosacral Plexopathy 1948 The Role of Magnetic Resonance Imaging in the Evaluation Julia Lim, Ian Goh, Zun Niang of Transient Ischaemic Attack Ng, Nicholas Shearer, Heather Smith, Benjamin Clissold 1949 Role of Dual Antiplatelet Therapy in Transient Ischemic Julia Lim, Ian Goh, Zun Niang Attack Ng, Nicholas Shearer, Heather Smith, Benjamin Clissold 2046 The impact of device-assisted therapy initiation on the gut Michal Lubomski, Xiangnan Xu, microbiome in Parkinson’s disease Andrew J Holmes, Jean Yang, Carolyn M Sue, Ryan L Davis 1970 Long-Term Efficacy and Safety of Perampanel in Elderly Manoj Malhotra, Rohit Marawar, Patients Aged ≥60 from Phase III Open-Label Extension Ilo Leppik, Robert T Wechsler, Studies Anna Patten, Leock Y Ngo 149 Number Title Authors 1902 Rare late onset neutropenia in a patient with multiple Babar Malik, Shoaib Dal, Robert sclerosis treated with ocrelizumab and review of literature McGrath 2032 An atypical case of idiopathic intracranial hypertension Yasser M Mansour, Nick Saad, Simon Liew, Sameen Haque 1888 A unique New Zealand case of Generalised Cutaneous Eileen Mc Manus, Peter PW dysesthesia and review of the literature Wright, Amanda Oakley 1893 Neurological manifestations in Rheumatological disease: a Eileen J Mc Manus, Douglas case series I White, Alan Doube, Jan Schepel, Matthew Phillips, Kamal Solanki 1894 Extended Interval Dosing Natalizumab infusion and impact Eileen J Mc Manus, Karen M on neuropsychological deficits in Relapsing-Remitting Clark, Jamie A.B Macniven, Jan Multiple Sclerosis Schepel 1895 Metabolic syndrome in a New Zealand Glioblastoma cohort Eileen J Mc Manus, Christopher 2005-2020: A retrospective analysis and review of the Frampton, Alvin Tan, Matthew literature Phillips 1957 Neurological manifestations in Rheumatological disease: a Eileen Mc Manus, Alan Doube, case series II Jan Schepel, Matthew Phillips, Vicky Quinsy, Kamal Solanki 2002 An unusual presentation of leptomeningeal carcinomatous. Eileen J Mc Manus, Weng J Mak, Margaret Fisher, Alvin Tan, Christopher Lynch 2037 An integrated neurogenomics clinic - 28-months Alison McLean, Michel experience and outcome of a tertiary referral centre Tchan, Sophie Devery, Renee Smyth, Kishore Kumar, Susan Tomlinson, Stephen Tisch, Kathy Wu 1993 Bradycardia as a rare neurocardiac prodrome to Leucine- Sai Nagaratnam, Yun T Hwang, rich glioma inactivated-1 antibody encephalitis Elizabeth Reyneke 1968 Efficacy and Safety of Perampanel in Paediatric Patients Andras Fogarasi, Leock Y Ngo, (Aged 4–<12 Years) with Focal-Onset Seizures (FOS) or Anna Patten, Manoj Malhotra Generalised Tonic-Clonic Seizures (GTCS) who Converted to Monotherapy: A Case Series from Study 311 1974 Long-Term (1 Year) Seizure Freedom with Adjunctive Robert Flamini, Anna Patten, Perampanel in Paediatric Patients (Aged 4–<12 Years) with Manoj Malhotra, Leock Y Ngo Focal-Onset Seizures (FOS) or Generalised Tonic-Clonic Seizures (GTCS): Post Hoc Analysis of Study 311 1918 Predictive Value of Signs and Symptoms in Code Strokes Mei Yan Ngun, Mark Tacey, for Diagnosis of Ischaemic Stroke or TIA Douglas Crompton 2052 Acquired idiopathic generalised anhidrosis, a rare Jennifer Nguyen, Michaela debilitating condition to be aware of Zallmann, Robert Puy, Elspeth Hutton, Tasos Stavrakoglou 150 Number Title Authors 1938 Outcome of endovascular thrombectomy for ultra-long Seppy Pakrah, Karen Huang, aeromedical transfers: The experience of one “hub” and Haylee Berrill, Meng Tan, Peter eleven “spoke” sites covering more than 1.8 million km Bailey, Arman Sabet, Laetitia Devilliers, Hal Rice, Darshan Shah 1939 Neurostimulation based therapies in New Onset Refractory Seppy Pakrah, Shanthi Sarma , Status Epilepticus (NORSE): Case report Meng Tan 1940 Subacute fulminant cognitive impairment as a presenting Seppy Pakrah, Saman Heshmat symptom of Multiple Sclerosis in a 51-year-old woman 1941 Paraneoplastic multiple cranial nerve palsies associated Seppy Pakrah, Saman Heshmat with anti-Hu and anti-Ri antibodies: Case report 2012 Retinal artery occlusion may herald the relapse of Diffuse Natalie C Palavra, Jonathan Large B-Cell Non-Hodgkin Lymphoma Baird-Gunning, Emily Sutherland, Michal Lubomski 2013 Ceftriaxone therapy for Adult Alexander Disease: report of Natalie C Palavra, Omar Ahmad 2 cases 2070 Stroke-like migraine attacks after radiation therapy: Be Elaine Pang, Josephine SMART and order an EEG Chan, David Blacker, Thomas Chemmanam, Nicholas Lawn 2071 Optic Perineuritis in Giant Cell Arteritis Elaine Pang, Neha Irani 2087 Pair-wise differences of penumbra and core volume Peter SW Park, Robbie Chan, estimates from three computed tomography perfusion Channa Senanayake, Stanley software packages are influenced by site of large vessel MK Tsui, Alun Pope, Helen M occlusion Dewey, Philip MC Choi 1921 Long-Term Reduction of Relapse Rate and 48-Week Gavin Giovannoni, John Parratt, Confirmed Disability Progression After 6.5 Years of Ludwig Kappos, Jerome De Ocrelizumab Treatment in Patients With RMS Seze, Stephen Hauser, James Overell, Harold Koendgen, Hans-Martin Schneble, Kalpesh Prajapati, Qing Wang, Jerry S Wolinsky 1933 Prognostic Value of Serum NfL for Subclinical Disease John Parratt, Tjalf Ziemssen, Activity and Worsening in Patients with RMS: Results from Douglas L Arnold, Enrique the Phase 3 ASCLEPIOS I and II Trials Alvarez, Anne H Cross, Roman Willi, Bingbing Li, Petra Kukkaro, Harald Kropshofer, Krishnan Ramanathan, Martin Merschhemke, Wendy Su, Dieter A Haering, Stephen L Hauser, Ludwig Kappos, Jens Kuhle 2039 Clinical variation in the use of disease-modifying therapies Kieren Po, Michael H Barnett for multiple sclerosis between different states and territories in Australia, 2019–20 151 Number Title Authors 2031 Fulminant ADEM mimicking a glial tumour Jessica Qiu, Elizabeth Thompson, Michael J Fulham, Mrudula Krishnaswamy, Michael E Buckland, Liane Khoo, Nicolás Urriola, Kaitlyn Parratt 2036 An Unexpected Cause of Ophthalmoplegia and Areflexia: Jessica Qiu, Albert Vien, The Pupils Have It Andrew Hannaford, Patricia Ferguson, Mohammad Hamidi, Amit Vaidya, Andrew Henderson 2035 Bilateral abducens nerve palsies following bilateral middle Subahari Raviskanthan, Peter meningeal artery embolisation for chronic subdural W Mortensen, Yi J Zhang, haemorrhage Andrew G Lee 2019 Inflammatory complications of CGRP monoclonal Jason C Ray, Penelope Allen, antibodies Ann Bacsi, Julian Bosco, Luke Chen, Michael Eller, Lyndell Lim, Hock Kua, Manjit Matharu, Mastura Monif, Richard Stark, Elspeth Hutton 1997 Hemi-cord infarction following vertebral artery dissection Alanna Rottler, Yew Li in a patient with congenital hypoplastic vertebral artery: A (Michelle) Dang, Wai Foong case report Hooi, David A Burrows, Hong Kuan Kok, Douglas E Crompton 2027 The Diagnostic Journey of Mitochondrial Disease Patients Laura I Rudaks, Eloise Watson, Michal Lubomski, Fabienne Edema-Hildebrand, Kate Ahmad, Christina Liang, Ryan Davis, Carolyn Sue 2028 IgLON5 Autoimmunity in Two Cases with Peripheral Laura I Rudaks, Victor SC Fung, Nervous System Features Jean-Pierre Halpern, Omar Ahmad 2073 A diagnostically challenging case of sciatic mononeuritis Laura I Rudaks, Jonathan Baird-Gunning, Emily Sutherland, Mark Thieben 1922 Serum Ig Levels and Risk of Serious Infections by Baseline Amit Bar-Or, Robert Bemel, Ig Quartile in the Pivotol Phase III Trials and Open-Label Martin S Weber, Richard W Extensions of Ocrelizumab in Multiple Sclerosis Hughes, Chien-Ju Lin, Jianmei Wang, Annette Sauter, Harold Koendgen, Licinio Craveiro, Stephen Hauser, Tobias Derfuss, Neil Shuey 2009 Headaches of raised intracranial pressure as a presenting Matthew Silsby, Andrew Martin, feature of malignant infiltration in the cauda equina Winny Varikatt, Steve Vucic, Victor SC Fung, Parvathi Menon 2080 Anti-Leucine-rich glioma inactivated 1 (LGI1) encephalitis Jessica Stabler, David Williams associated with high grade papillary urothelial carcinoma 152 Number Title Authors 2077 Lambert Eaton Myaesthenic Syndrome in the Absence of Joseph B Stockwell, Leon S Malignancy Edwards, Daniel Wardman, Neil Griffith 2016 Gadolinium encephalopathy presenting as status Emily Sutherland, Jonathan epilepticus following intrathecal injection Baird-Gunning, Laura Rudaks, Natalie Palavra, Michal Lubomski, Martin Krause 1908 Spinal cord presentation of biopsy-proven PET-positive Grace Swart, Sapna Balgobind, giant cell arteritis. Charles Chan, Michael Fulham, Sean Riminton, Emma Mitchell, Stephen Reddel 1892 A case of isolated musculocutaneous nerve injury Oshi Swarup, Belinda Cruse, following skydiving simulation Daniela Leupold, John King 1985 Asymptomatic SARS-CoV-2 Infection and Isolated Elizabeth Tan, Aishwarya Abducens Palsy Anilkumar, Jonathan Cleaver, Hamish Morrison 1906 Under PRESsure - An unusual trigger of posterior James O Thomas, David B reversible encephalopathy Williams, Christopher Grainge 1917 Ischaemic stroke as the only manifestation of anti- John Tran, Joshua Abasszade, neutrophilic cytoplasmic autoantibody associated Yew Li (Michelle) Dang, Doug vasculitis Crompton 1971 Shift from chronic to episodic migraine status and Kathleen A Day, Mallik high- to low-frequency episodic migraine status among Rettiganti, Margaret B Ferguson, patients with treatment-resistant migraine in a phase 3 Chad E Stroud, Benjamin galcanezumab study P Trewin, Takao Takeshima, William S Kingston 2091 Clinical and neurophysiological improvement in Hereditary James D Triplett, Garth sensory and autonomic neuropathy type I (HSAN-1) Nicholson, Con Yiannikas following high dose serine therapy 2022 A phase 1b open label study of sodium selenate as a Lucy Vivash, Charles B Malpas, disease-modifying treatment for behavioural variant Christopher M Hovens, Dennis frontotemporal dementia Velakoulis, Terence J O’Brien 1934 Characteristics and Outcome of COVID-19 in Patients with Kate Martel, Robert A Relapsing Multiple Sclerosis Receiving Ofatumumab Walker, Anne H Cross, Silvia Delgado, Mario Habek, Maria Davydovskaya, Natalia Totolyan, Ratnakar Pingili, Linda Mancione, Roseanne Sullivan, Martin Zalesak, Wendy Su, Krishnan Ramanathan, Xavier Montalban, Kevin Winthrop 1966 PROVE Study 506: Analysis of a Retrospective, Phase James Wheless, Anna Patten, IV Study of Perampanel in Real-World Clinical Care of Leock Y Ngo, Alejandro Salah, Patients Based on Seizure Type Manoj Malhotra 1984 False positive RT-QuIC test for Creutzfeldt Jakob disease Rajiv Wijesinghe, Craig in dementia with status epilepticus Anderson, Miriam Welgampola, Christine Stehmann, Steven Collins, Penelope Spring 153 Number Title Authors 2092 Man-in-the-barrel syndrome as a complication of cervical Rajiv Wijesinghe, Con Yiannikas decompressive surgery 1935 An Atypical Presentation of Transient Epileptic Amnesia Benjamin D Wood, Amanda Lasting Six Hours Ji, Michael Roizman, Andrew Swayne 2079 Overlapping autoimmunity: a case of concomitant Miriam Wronski, Justine J Aquaporin-4 and Myelin Oligodendrocyte Glycoprotein Wang (MOG) antibody positivity in Neuromyelitis Optica Spectrum Disorder 2090 Favourable outcome following early treatment with Yangyang (Erin) Xiao, Elham rituximab in a patient with probable Susac’s syndrome Khalilidehkordi, Po Sheng Yang, Andrew Wong, Claire Muller 1916 Presence of VGCC antibodies with possible late onset Po Sheng Yang, Viral Upadhyay, multiple acyl-CoA dehydrogenase deficiency Thomas Robertson, Robert Boots, Robert Henderson 2081 Contrast-induced encephalopathy after carbon dioxide Hue Mun Au Yong, Matthew K angiography in the upper extremity and iodinated contrast Ligtermoet, Douglas Crompton – a case report 2017 Ganglioside antibodies, meningoencephalitis and Xin Zhang, Liam Beiglari, longitudinally extensive transverse myelitis: GAME-LETM Nicolas Urriola, Kaitlyn Parratt 2030 A persistent false familiarity: recollective confabulation Xin Zhang, Nora Breen, Kaitlyn presenting in a patient describing a life of ‘total instant Parratt recall’ 154