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Neuropsychiatry Update Neuropsychiatry Update Newsletter of the Faculty of Neuropsychiatry: Royal College of Psychiatrists DECEMBER 2017 #13 02 Contents Editorial Conference Reports 03 What do I do? 22 Faculty of Neuropsychiatry, Sept 2017 Norman Poole Thomas Anderson 25 The 3rd International Conference on Functional Special Article (Psychogenic) Neurological Disorders (FND), Edinburgh, Scotland, September 6th - 8th 2017. 05 An Update On Diseases Caused By Neuronal Joana Macedo da Cunha Membrane Antibodies Rebecca Pollard and Belinda Lennox AOB 10 Diagnostic Implications of the C9orf72 Mutation in Clinical Psychiatry 27 Request For Help Simon Ducharme Phillip Slack 13 Group Hysteria: Evidence-base and experience of using group treatments for Functional Neurological Disorder Sarah Cope Editors Norman Poole Neuropsychiatry News is produced twice yearly. Articles, case- Editorial Board Eileen Joyce reports and service descriptions should be submitted in a MS Word format by email and should not exceed 2000 words unless agreed Address for correspondence with the Editor. Letters should not exceed 200 words. The Editor Norman Poole reserves the right to edit contributions as deemed necessary. Neuropsychiatry Service, Opinions expressed in the newsletter are of the authors and not of 2nd floor Grosvenor Wing, the College. Copyright of submissions are retained by its author, but St George’s Hospital, SW17 0QT the College reserves the right to reproduce the article on the Faculty website pages. Email: [email protected] Graphic Design Jamie Paton - [email protected] 03 Editorial What do I do? attractions of the specialism, but can Norman Poole also make it daunting. At times, I must have exactly the same expression of Consultant bewilderment as my interlocutor from the Neuropsychiatrist at dinner party. Each of the articles in this St George’s Hospital edition was commissioned in response to cases seen on the wards and clinic where I work, and will hopefully assist others who face the same question, “What do I do?” The discovery of autoimmune encephalitis associated with antibodies to neuronal membrane receptors has gripped many psychiatrists given the symptom overlap with major mental disorders. Since then the number of cases reported and variety of autoantibodies implicated has swelled. More recently, autoantibodies have been found in a significant minority of patients in the first episode of psychosis. Psychiatrists “What do you do?” is a question that in Early Intervention Services must now generally crops up within moments of be anxiously wondering how to identify meeting someone new, as the questioner those at highest risk and what to do with sizes up the other. The response, “I’m a a positive test result. but to date, research neuropsychiatrist” is satisfyingly abstruse findings and their implication for clinicians as most medics, let alone non-medics, have often been contradictory and so have no idea what to make of it. I have I am grateful to Rebecca Pollard and Dr developed a few stock phrases that Belinda Lennox for eloquently summarising transforms the bewildered expression to the latest developments in the field and one of comprehension and, sometimes, answering my question about what to do. fascination. “That sounds really interesting,” I often hear, and of course it is. The An elderly patient with a very bizarre range and rarity of conditions seen in and fluctuant presentation triggered my neuropsychiatry is one of the many requesting Dr Simon Ducharme write 04 a paper on the C9orf72 mutation. We the two. However, there are now so many were caught in the classic struggle of people diagnosed with FND that we need colleagues in neurology telling us that she effective treatments that are deliverable had a depressive stupor triggered by her to a large, and growing, population of I’m now dog being put to sleep, while I insisted the sufferers. Dr Cope describes what we busy using presentation was neurological but was have done locally and the outcomes from my retro- unable to give a diagnosis. It was actually multidisciplinary group interventions for spectoscope a neurology trainee who suggested the FND. It’s not rare but still fascinating. possibility of a C9orf72 mutation, which to diagnose I have to be honest caused a wave of “What do I do?” is not only a practical other bewilderment to pass over me that I question but an existential one too. There perplexing may not have fully concealed by nodding is insufficient space in this newsletter for cases I’ve along sagely. I’m now busy using my my own such musings but it has been seen over retrospectoscope to diagnose other heartening to observe two trainees the years perplexing cases I’ve seen over the years, wrestle with this question and find in and I strongly recommend anyone who neuropsychiatry a possible resolution. A hasn’t yet heard of the condition to read very welcome European guest, Dr Joana the review article. Neurosyphilis was Macedo da Cunha attended the recent once known as the “great mimic” and three day FND conference in Edinburgh to both C9orf72 mutation and autoimmune find plenty of other visitors from around encephalitis while not so numerous can also the world and different specialities. It’s an lay claim to being fine imitators of primary exciting time for research in FND and the mental disorders. conference helped Dr Macedo da Cunha refine her PhD proposal on intentional Talking about great mimics, Dr Sarah binding. Dr Thomas Anderson, an FY2 Cope, who I have the pleasure of working in neuropsychiatry, arrived with the alongside, has taken a different approach same look as the dinner party guest. But to the question of what to do. Functional bewilderment passed to comprehension Neurological Disorder (FND) mimics any and, as you’ll discover from his Faculty of neurological condition, but we are now Neuropsychiatry Conference report, to pretty good at discriminating between fascination. I trust yours will too. 05 Special Articles An Update On Diseases Caused By Neuronal Membrane Antibodies Discovery of autoimmune causes of encephalitis The recognition that antibodies against neuronal cell surface targets can directly cause central nervous system disease is a relatively recent occurrence. The first descriptions were of antibodies to the Voltage- gated Potassium Channel Complex (VGKCC) in two cases of limbic encephalitis (Buckley et al., 2001). Further cases were then described, effectively treated using immunotherapy, confirming that antibodies to Rebecca Pollard and Dr Belinda Lennox the VGKCC could be pathogenic. Since then, several other neuronal membrane antigenic targets have been identified and implicated in patients with encephalitis. Rebecca Pollard - Research Assistant The most common of these are against the N-Methyl- SINAPPS research group (SINAPPS2/PPiP2), Department of D-Aspartate (NMDA) Receptor, a glutamatergic Psychiatry, University of Oxford neuronal membrane receptor which is particularly important for memory consolidation. These NMDA Belinda Lennox DM FRCPsych, receptor antibodies have been demonstrated to bind to Department of Psychiatry, University of Oxford the NR1 (GluN1) subunit of NMDA receptor, and cause cross-linking and internalisation of NMDARs. Introduction Antibodies to the NMDA receptor were first described Autoimmune encephalitis associated with antibodies to clinically by Dalmau et al. in female patients who had a neuronal membrane receptors is now a well- rapidly progressing, sometimes fatal, encephalitis and established disorder in neurology. Whilst a ovarian teratomas. Resection of the tumour alongside neuroimmunological disorder, the clinical phenotype immunotherapy such as plasma exchange, resulted in overlaps with psychiatric disorders, and patients often good clinical outcomes (Dalmau, 2008). present initially to psychiatric services. It is therefore important for all psychiatrists to be aware of this Subsequently, cases of encephalitis associated disorder and know who to screen, and what to do if a with NMDAR antibodies have been described patient has a positive result. in a range of patients; males and females, with 06 or without identified tumours (Irani et al., 2010; patients who present with psychosis are unlikely to be Dalmau et al., 2011). NMDAR encephalitis is now tested for antibodies, without the presence of other considered a mainstream diagnosis within neurology, neurological symptoms.(Prüss & Lennox 2016). diagnosed in several hundred patients worldwide. We have recently completed an observational cohort Clinical experience shows that when treatment is study The Prevalence of Pathogenic antibodies in delivered early, outcomes are significantly better. Psychosis (PPiP1). The serum of 228 Patients with first episode psychosis were tested for neuronal membrane In all of the case series to date patients with NMDAR antibodies across 37 different NHS mental health antibody encephalitis initially present with cognitive trusts across England over two years. The patient dysfunction, behavioural change, psychosis or population in this study were aged 14-35, in their first seizures (Irani et al 2010, Dalmau et al 2008) In the episode of psychosis and had been taking antipsychotic initial case series 77 of 91 patients with NMDAR medication for 6 weeks or less. Importantly, none encephalitis initially presented to psychiatric of these patients had other neurological disease, or services with their symptoms. (Dalmau et al., 2008). encephalitis and all were being treated in psychiatric Screening patients when they present to services
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