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ISSN 1473-9348 VOLUME 13 ISSUE 7 JANUARY/FEBRUARY 2014 ACNRwww.acnr.co.uk ADVANCES IN CLINICAL NEUROSCIENCE & REHABILITATION In this issue Camilla Clark and Jason Warren – The Neurology of Humour Michael Parkinson, Fion Bremner, Paola Giunti – ARSACS Philip Holland and Shazia Afridi – Migraine Pathophysiology Alex Leff – The Future of Stroke Rehabilitation: recovery of language and vision Andrew Larner – Parkinson's Disease Before James Parkinson AWARDS AND APPOINTMENTS > BOOK REVIEWS > CONFERENCE REPORTS > EVENTS DIARY Textbook of Clinical Neuropsychiatry and Behavioral Neuroscience 3rd Edition NEW TO THIS EDITION: A completely new section covering the fundamentals of neuroscience Additions to the clinical coverage including chronic fatigue conditions, personality disorders, dissociative disorders, and impulse control disorders Thoroughly redesigned with extensive use of high-quality color clinical photographs to help illustrate the most difficult concepts Access to a free eBook containing all the text and a wealth of supplementary material to be accessed online or from the reader’s desktop June 2012 | 864pp | ISBN 9781444121346 David P. Moore, Basant K. Puri More Pack - Book and Online £170.00 £136.00 20% discount when you order online and enter discount code LBM18 at checkout Expires 31/12/2013 FREE standard shipping when you order online. www.crcpress.com CONTENTS CONTENTS JANUARY/FEBRUARY 2014 ISSN 1473-9348 VOLUME 13 ISSUE 7 JANUARY/FEBRUARY 2014 04 From the Editor... ACNRwww.acnr.co.uk ADVANCES IN CLINICAL NEUROSCIENCE & REHABILITATION 06 Awards and Appointments Review Article 09 The Neurology of Humour Camilla Clark and Jason Warren Review Article 12 Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay In this issue Camilla Clark and Jason Warren – The Neurology of Humour Michael Parkinson, Fion Bremner, Paola Giunti – ARSACS (ARSACS) Philip Holland and Shazia Afridi – Migraine Pathophysiology Alex Leff – The Future of Stroke Rehabilitation: recovery of language and vision Michael Parkinson, Fion Bremner, Paola Giunti Andrew Larner – Parkinson's Disease Before James Parkinson AWARDS AND APPOINTMENTS > BOOK REVIEWS > CONFERENCE REPORTS > EVENTS DIARY Review Article Cover image: www.istockphoto.com/agsandrew 17 Advances in our Understanding of the Brain Mechanisms of Freezing of Gait in Parkinson’s Disease James Shine and Simon Lewis Headache Series 19 Migraine Pathophysiology ACNR Philip Holland and Shazia Afridi Published by Whitehouse Publishing, 1 The Lynch, Mere, Wiltshire, BA12 6DQ. Publisher. Rachael Hansford Special Report E. [email protected] 22 Collaborative Research Networks Angelos Kolias , Ian Coulter, Chris Cowie, Andrew Tarnaris, Peter Hutchinson and Paul Brennan ADVERTISING Rachael Hansford T. 01747 860168 M. 07989 470278 History of Neurology E. [email protected] 24 Parkinson’s Disease Before James Parkinson COURSE ADVERTISING Cathy Phillips E. [email protected] Andrew Larner EDITORIAL Stroke Article John Gustar E. [email protected] 26 The Future of Stroke Rehabilitation:recovery of language PRINTED BY Buxton Press T. 01298 21 2000 and vision Alex Leff Copyright: All rights reserved; no part of this publication may be reproduced, stored in a retrieval system or transmitted in any form or by any means, electronic, mechanical, photo- Regulars copying, recording or otherwise without either the prior written permission of the publisher or a license permitting restricted photocopying issued in the UK by the Copyright 07 Journal Reviews Licensing Authority. Disclaimer: The publisher, the authors and editors accept no 28 Diary responsibility for loss incurred by any person acting or refraining from action as a result of material in or omitted 29 Conference Reports from this magazine. Any new methods and techniques described involving drug usage should be followed only in 35 Book Reviews conjunction with drug manufacturers' own published litera- ture. This is an independent publication - none of those contributing are in any way supported or remunerated by any of the companies advertising in it, unless otherwise clearly stated. Comments expressed in editorial are those of the author(s) and are not necessarily endorsed by the editor, Exclusive content on www.acnr.co.uk editorial board or publisher. The editor's decision is final and no correspondence will be entered into. Special Feature Godfrey Hounsfield and the Early Days of CT Richard Waltham Case Report Meningomyelitis, Cranial Neuropathy and Cerebral Vasculitis Bookmark Secondary to Epstein Barr Virus and Varicella Zoster Virus www.acnr.co.uk Co-infection of the Central Nervous System for exclusive Emma Blackwood and David Sharpe online content Conference Report Improving Outcome in CNS Tumours ACNR > VOLUME 13 NUMBER 7 > JANUARY/FEBRUARY 2014 > 3 FROMTHEEDITOR ... Episenta® (sodium valproate) Prescribers should consult the Summary of Product Characteristics before prescribing Episenta® Sodium valproate available as Episenta® 150 or 300mg Prolonged-release Capsules, Episenta® Sachets containing 500mg or 1000mg Prolonged-release Granules and Episenta® 100mg/ml Solution for Injection. Indication: Epilepsy. Solution for injection: For use in patients normally maintained on oral sodium valproate but temporarily not possible. Oral: For treatment of manic episode in bipolar disorder when lithium is contraindicated or not tolerated. The continuation of treatment after manic episode could be considered in patients who have responded to valproate for acute mania. Dose and Administration: Epilepsy: Oral: Monotherapy: Adults: 600mg daily increasing by 150-300mg at 3-day intervals until controlled; usual dose range 1000mg to 2000mg/day. Max dose 2500mg/day. Children >20kg: 300mg/day increasing until controlled; usual dose range 20-30mg/kg/day. Max dose 35mg/kg/day. Children <20kg: 20mg/kg per day; in severe cases up to 40mg/kg/day. Daily dosage should be given in 1-2 single doses. Contents of the capsule/sachet may be sprinkled or stirred into soft food or drinks (cold or room temperature) and swallowed immediately without chewing or crushing the granules. Changing from sodium valproate enteric coated tablets to Episenta®, keep the same daily dose. Elderly: Care when adjusting dosage. Dosage should be determined by seizure control. Renal insufficiency: May be necessary to decrease dosage. Hepatic insufficiency: see Contraindications, Warnings and Precautions and Undesirable effects. Salicylates should not be used concomitantly. Combined Therapy: Start Episenta® in patients already on anticonvulsants gradually. Target dose reached after about 2 weeks. In combination with barbiturates, barbiturate dose should be reduced, particularly if sedation observed. Solution for injection: Adults: 400-800mg daily increasing by 150-300mg at 3-day intervals until controlled; usual dose range 1000mg to 2000mg/day. Max dose 2500mg/day. Children: 300mg/day increasing until controlled; usual dose range 20-30mg/kg/day. Max dose 40mg/kg/day, only in Mike Zandi, Editor. patients in whom plasma levels can be monitored. Above 40mg/kg/day clinical chemistry and haematology should be monitored. Patients already satisfactorily treated with oral continue at current dosage. The total daily dose divided into 3-4 single slow intravenous injections or given by continuous or repeated infusion. Should not be administered via same line with other drugs. Should be replaced with oral therapy as soon as practicable. Close monitoring of plasma levels n this first issue of 2014, Camilla Clark and Jason required during therapy and when changing to/back from parenteral therapy. Manic episodes: Adults: initial daily dose 750mg or 20mg/kg body weight, rapid dose increase if possible, mean Warren from the Dementia Research Centre, UCL daily dose btw. 1000 and 2000mg. Patients receiving daily doses higher than 45mg/kg/day body review the neurology of humour in the healthy weight should be carefully monitored. Contraindications: Active liver disease; History of severe I hepatic dysfunction, especially drug related; Porphyria; Hypersensitivity to valproate or and diseased brain. They explain the functional excipients. Warnings and Precautions: Monitor for signs of suicidal ideation/behaviour. Discontinue gradually. Patients should be closely monitored for early signs of liver damage. imaging and electrophysiological evidence for Use with carbapenem not recommended. Risk of severe liver damage, including fatal hepatic humour’s subdivision into separable neural failure; children <3 years most at risk especially with multiple anticonvulsants, severe seizure disorders, organic brain disease, diseases associated with mental retardation. Avoid correlates, and show us how the study of the concomitant salicylates in children <3 years. Salicylates should not be used in children <16 years. Measure liver function before treatment and during the first 6 months. Risk of severe or appreciation of humour can provide an opportunity fatal pancreatitis, particularly young children; risk decreases with increasing age. Blood cell to examine a range of diseases of the brain. Michael count, platelet count, bleeding time and coagulation tests recommended prior starting therapy before surgery, or if spontaneous bruising/bleeding. Caution in patients with systemic lupus Parkinson, Fion Bremner and Paola Giunti, from UCL, erythematosus. Risk of hyperammonaemia in patients with urea cycle enzymatic deficiency. write a comprehensive review on Autosomal Risk of weight gain. Caution in women of childbearing potential. Interactions: