Evidence-basedNeurology:Management ofNeurological DisordersEDITED BY

Livia CandeliseCochrane Neurological NetworkOspedale Maggiore PoliclinicoUniversity of MilanMilan, Italy

WITH

Richard HughesKing’s College London School of Medicine at Guy’sKing’s College and St Thomas’ HospitalsLondon, UK

Alessandro LiberatiUniversità di Modena e Reggio EmiliaModena, Italy

Bernard M.J. UitdehaagVU University Medical CenterAmsterdam, The Netherlands

Charles WarlowUniversity of EdinburghWestern General HospitalEdinburgh, UK

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Evidence-basedNeurology:Management ofNeurological Disorders

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Evidence-basedNeurology:Management ofNeurological DisordersEDITED BY

Livia CandeliseCochrane Neurological NetworkOspedale Maggiore PoliclinicoUniversity of MilanMilan, Italy

WITH

Richard HughesKing’s College London School of Medicine at Guy’sKing’s College and St Thomas’ HospitalsLondon, UK

Alessandro LiberatiUniversità di Modena e Reggio EmiliaModena, Italy

Bernard M.J. UitdehaagVU University Medical CenterAmsterdam, The Netherlands

Charles WarlowUniversity of EdinburghWestern General HospitalEdinburgh, UK

© 2007 by Blackwell PublishingBMJ Books is an imprint of the BMJ Publishing Group Limited, used under licenceBlackwell Publishing, Inc., 350 Main Street, Malden, MA 02148-5020, USABlackwell Publishing Ltd, 9600 Garsington Road, Oxford OX4 2DQ, UKBlackwell Publishing Asia Pty Ltd, 550 Swanston Street, Carlton, Victoria 3053, Australia

The right of the Author to be identified as the Author of this Work has been asserted inaccordance with the Copyright, Designs and Patents Act 1988.

All rights reserved. No part of this publication may be reproduced, stored in a retrievalsystem, or transmitted, in any form or by any means, electronic, mechanical, photocopying,recording or otherwise, except as permitted by the UK Copyright, Designs and Patents Act1988, without the prior permission of the publisher.

First published 20071 2007

Library of Congress Cataloging-in-Publication Data

Evidence-based neurology : management of neurological disorders / edited byLivia Candelise ... [et al.].

p. ; cm.“BMJ Books.”Includes bibliographical references and index.ISBN 978-0-7279-1811-6 (hardback : alk. paper)

1. Nervous system—Diseases. 2. Neurology. 3. Evidence-based medicine.I. Candelise, Livia. [DNLM: 1. Nervous System Diseases—therapy. 2. Evidence-Based

Medicine. WL 140 E93 2007]

RC346.E972 2007616.8—dc22

2006038415

ISBN: 978-0-7279-18116

A catalogue record for this title is available from the British Library

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Contents

Contributors, vii

Preface, x

Part 1 Introduction

1 Evidence-based medicine: its contributions in the way we search, appraise and apply scientific information to patient care, 3Alessandro Liberati, Lorenzo P. Moja, Ivan Moschetti

2 What to do when there is no evidence, 11Charles Warlow

3 Outcome and adverse effect measures in neurology, 15Jorge G. Burneo, Samuel Wiebe

4 Systematic reviews of diagnostic research, 23Bernard M.J. Uitdehaag, Peter M. Rothwell

Part 2 Neurological symptoms/problems

5 Acute migraine attacks, 29Anne MacGregor

6 Back and neck pain, 40Harry McNaughton

7 Neuropathic pain, 47Giorgio Cruccu, Nadine Attal, Rod Taylor

8 Vertigo and dizziness: treatment of benign paroxysmal positional vertigo,vestibular neuritis and Menière’s disease, 59Michael Strupp, Thomas Brandt, Christian Daniel Cnyrim

9 Sleep disorders, 70Michel Billiard

10 Cognitive rehabilitation of non-progressive neuropsychological disorders, 79Stefano F. Cappa, Thomas Benke, Stephanie Clarke, Bruno Rossi, Brigitte Stemmer, Caroline M. van Heugten

11 Management and pharmaceutical treatment of central and spinal spasticity, 89David T. Shakespeare

12 Background to neurorehabilitation, 100Kathryn McPherson, Paula Kersten, Lynne Turner-Stokes

v

Part 3 Neurological diseases

13 Acute stroke management and prevention of recurrences, 113Gord Gubitz

14 Aneurysmal subarachnoid haemorrhage, 127Jan van Gijn, Gabriel J.E. Rinkel

15 Acute traumatic brain injury, 142Miguel F. Arango, Walter Videtta, Corina Puppo

16 Corticosteroids in central nervous system infections, 151Anu Jacob, Tom Solomon, Paul Garner

17 Brain tumours, 161Michael G. Hart, Robin Grant

18 Epilepsy, 173Sridharan Ramaratnam, Anthony Marson

19 Dementia treatment: let the evidence lead us, 184Bart M. Demaerschalk, Bryan K. Woodruff, Richard J. Caselli

20 Parkinson’s disease, 199Miguel Coelho, Joaquim Ferreira, Cristina Sampaio

21 Multiple sclerosis: critical review of the evidence for each question, 221Graziella Filippini, George Ebers

22 Motor neurone disorders, 234Douglas Mitchell

23 Peripheral nerve disorders, 244Richard Hughes, Eva L. Feldman, Jeremy D.P. Bland

24 Muscle disorders, 258Fiona Norwood, Michael R. Rose

Index, 267

vi Contents

Contributors

Miguel F. Arango, MDAssistant ProfessorDepartment of Anesthesia and Perioperative MedicineLondon Health Sciences Centre (University Campus)London, Canada

Nadine Attal, MD, PhDNeurologist and Pain SpecialistINSERM U 792 & Centre d’Evaluation et de Traitement de la DouleurHopital Ambroise ParéBoulogne-Billancourt, France

Thomas Benke, MDKlinik für NeurologieMedizinische Universität InnsbruckInnsbruck, Austria

Michel Billiard, MDProfessor of NeurologySchool of MedicineGui de Chauliac HospitalMontpellier, France

Jeremy D.P. BlandConsultant in Clinical NeurophysiologyDepartment of Clinical NeurophysiologyKent and Canterbury HospitalCanterbury, UK

Thomas Brandt, MD, FRCPProfessor and ChairmanDepartment of NeurologyLudwig-Maximilians-UniversityMunich, Germany

Jorge G. Burneo, MD, MSPHAssistant Professor of NeurologyCo-director, UWO Evidence-Based Neurology GroupUniversity of Western OntarioLondon, Canada

Livia CandeliseProfessor of NeurologyCochrane Neurological NetworkDepartment of Neurological SciencesOspedale Maggiore PoliclinicoUniversity of MilanMilan, Italy

Stefano F. Cappa, MDProfessor of NeuropsychologyDepartment of PsychologyUniversità Vita-Salute San RaffaeleMilan, Italy

Richard J. Caselli, MDProfessor of NeurologyChairman, Department of NeurologyMayo Clinic College of MedicineMayo Clinic ArizonaScottsdale, USA

Stephanie ClarkeProfessor of NeuropsychologyCentre Hospitalier Universitaire VaudoisHead of the Division of NeuropsychologyFaculty of Biology and MedicineUniversity of LausanneLausanne, Switzerland

Christian Daniel CnyrimResident, Department of NeurologyUniversity Hospital ‘Grosshadern’University of MunichMunich, Germany

Miguel Coelho, MDNeurologist, Neurological Clinical Research UnitInstitute of Molecular MedicineCentro de Estudos Egas MonizFaculdade de Medicina de LisboaLisbon, Portugal

Giorgio CruccuProfessor of NeurologyDepartment of Neurological SciencesLa Sapienza UniversityRome, Italy

Bart M. Demaerschalk, MD, MSc, FRCPCAssistant Professor of NeurologyDivision of Cerebrovascular Diseases and Critical Care NeurologyMayo Clinic ArizonaScottsdale, USA

vii

George Ebers, MD, MA, FRCPC, FMedSci, FRCPAction Research Professor of NeurologyDepartment of Clinical Neurology University of OxfordRadcliffe InfirmaryOxford, UK

Eva L. Feldman, MD, PhDRussell N. DeJong Professor of NeurologyUniversity of MichiganAnn Arbor, USA

Joaquim Ferreira, MDNeurologist, Neurological Clinical Research UnitInstitute of Molecular MedicineCentro de Estudos Egas MonizFaculdade de Medicina de LisboaLisbon, Portugal

Graziella Filippini, MDDirector, Unit of NeuroepidemiologyNational Neurological Institute ‘Carlo Besta’Milan, Italy

Paul Garner, MD, FFPHMInternational Health GroupLiverpool School of Tropical MedicineLiverpool, UK

Jan van Gijn, MD, FRCP, FRCPEdinProfessor and ChairmanDepartment of NeurologyUniversity Medical CentreUtrecht, The Netherlands

Gord Gubitz, MD, FRCPC, FRCPEdinAssistant Professor of NeurologyDivision of NeurologyHalifax InfirmaryHalifax, Canada

Robin GrantConsultant Neurologist, Honorary Senior LecturerDepartment of Clinical NeurosciencesWestern General HospitalEdinburgh, UK

Michael G. HartDepartment of Clinical NeurosciencesWestern General HospitalEdinburgh, UK

Caroline M. van Heugten, PhDNeuropsychologistSenior Researcher in Cognitive RehabilitationInstitute for Rehabilitation Research HoensbroekInstitute of Brain and BehaviourUniversity of MaastrichtHoensbroek, The Netherlands

Richard Hughes, MD, FRCP, FMedSciProfessor of NeurologyDepartment of Clinical NeuroscienceKing’s College London School of Medicine at Guy’sKing’s College and St Thomas’ HospitalsLondon, UK

Anu Jacob, MD, MRCP, DMConsultant NeurologistThe Walton Centre for Neurology and NeurosurgeryLiverpool, UK

Paula Kersten, BSc, MSc, PhDSenior Lecturer, School of Health Professions and Rehabilitation

SciencesUniversity of SouthamptonSouthampton, UK

Alessandro LiberatiProfessor of Clinical Epidemiology and BiostaticsDepartment of Hematology and OncologyUniversità di Modena e Reggio EmiliaModena, Italy

Anne MacGregor, MB, BS, MFFP, DIPMDirector of Clinical ResearchThe City of London Migraine ClinicLondon, UK

Anthony MarsonSenior Lecturer and Honorary ConsultantThe Division of Neurological ScienceUniversity of LiverpoolLiverpool, UK

Harry McNaughton, PhDProgramme Director, Rehabilitation ResearchMedical Research Institute of New ZealandWellington, New Zealand

Kathryn McPherson, RN, RM, BA (Hons), PhDProfessor of Rehabilitation (Laura Fergusson Chair)Division of Rehabilitation and Occupation StudiesAuckland University of TechnologyAuckland, New Zealand

Douglas Mitchell, MD, FRCPDirector, Preston MND Care and Research CentreRoyal Preston HospitalPreston, UK

Lorenzo P. MojaResearcherCentro Cochrane ItalianoIstituto Mario NegriMilan, Italy

viii Contributors

Ivan Moschetti, MDResearcherCentro Cochrane ItalianoIstituto Mario NegriMilan, Italy

Fiona Norwood, PhD, MRCPConsultant NeurologistKing’s Neuroscience CentreKing’s College HospitalLondon, UK

Corina Puppo, MDAssistant ProfessorIntensive Care UnitHospital de ClínicasUniversidad de la RepúblicaMontevideo, Uruguay

Sridharan RamaratnamSenior Consultant NeurologistApollo HospitalsChennai, India

Gabriel J.E. Rinkel, MDProfessor of NeurologyDepartment of NeurologyUniversity Medical CentreUtrecht, The Netherlands

Bruno RossiProfessor of Physical Medicine and RehabilitationDirector, Academic Unit of NeurorehabilitationDepartment of NeuroscienceUniversity of PisaPisa, Italy

Michael R. Rose, MD, FRCPConsultant and Honorary Senior Lecturer in NeurologyDepartment of NeurologyKing’s College HospitalLondon, UK

Peter M. Rothwell, MD, PhD, FRCPProfessor of Clinical NeurologyUniversity Department of Clinical NeurologyRadcliffe InfirmaryOxford, UK

Cristina Sampaio, MD, PhDProfessor, Laboratório de Farmacologia Clinica e TerapeuticaFaculdade de Medicina de LisboaLisbon, Portugal

David T. Shakespeare, MSc, MRCPConsultant in Rehabilitation MedicinePreston Neuro-Rehabilitation UnitPreston, UK

Tom Solomon, BA, BM, BCh, MRCP, DCH, DTMH,PhDMRC Senior Clinical Fellow, Senior Lecturer in NeurologyMedical Microbiology and Tropical MedicineUniversity of LiverpoolLiverpool, UK

Brigitte Stemmer, MD, PhDProfessor, Canada Research Chair in Neuroscience and

NeuropragmaticsCentre de recherche, IUGMUniversity of MontrealMontreal, Canada

Michael Strupp, MDProfessor of Neurology and Clinical NeurophysiologyUniversity of MunichUniversity Hospital ‘Grosshadern’Munich, Germany

Rod Taylor, MSc, PhDReader in Health Services ResearchPeninsula Medical SchoolExeter, UK

Lynne Turner-Stokes, DM, FRCPDirector, Regional Rehabilitation UnitNorthwick Park and Herbert Dunhill Chair of RehabilitationKing’s College LondonLondon, UK

Bernard M.J. Uitdehaag, MD, PhDAssociate Professor of Neurology and EpidemiologyDepartment of Neurology and Department of Clinical Epidemiology and BiostatisticsVU University Medical CentreAmsterdam, The Netherlands

Walter Videtta, MDNeurocritical Care, ICUHospital Nacional ‘Prof. A. Posadas’De Buenos Aires, Argentina

Charles WarlowProfessor of Medical NeurologyDepartment of Clinical NeurosciencesUniversity of EdinburghWestern General HospitalEdinburgh, UK

Samuel Wiebe, MD, MSc, FRCPCProfessor and Head, Division of NeurologyFoothills Medical CentreCalgary, Canada

Bryan K. Woodruff, MDInstructor in NeurologyMayo Clinic College of MedicineMayo Clinic ArizonaScottsdale, USA

Contributors ix

Preface

A carefully selected group of collaborators contributed to thisbook on evidence-based neurology, which aims to answermore than 100 clinical questions about treatment and man-agement of neurological disorders. Participating authors, all of whom have clinical experience in specific neurological dis-orders, used a standard approach to search and summarize theresults of existing scientific evidence in an attempt to providereaders with a straightforward text.

Thus we hope that this volume will be a useful tool for allgeneral neurologists, from the oldest to the youngest, intheir everyday clinical practice.

We aim to offer a product that:• summarizes the most recent and important findings ontreatments for neurological patients;• provides answers to at least one treatment uncertainty;• measures the benefit and, when applicable, the risk ofharm inherent in specific neurological interventions.

We do not pretend to have provided complete coverage ofthe neurological field, because some important topics werenot included. We also do not pretend to be completely evi-dence based, because some important clinical questions donot have an evidence-based answer. We selected a set of top-ics on the basis of practical judgement, and we systematicallysearched the literature that contained answers. We hopethat the reader will not be disappointed that the whole ofneurological treatment is not covered, or that the informationprovided for each topic is not 100% comprehensive.

Nowadays regulatory agencies such as the FDA and EMEAplay a role in guiding clinical practice. Guidelines and prac-tice parameters, more and more frequently, produce inter-nationally and locally applicable clinical rules. Of courseclinicians must be able to obtain these guidelines and knowhow to use them in their practice. However, sometimes theyalso need to refer back to primary studies and to weigh theirpersonal experience against specific clinical research, takinginto account both the design and results of that research.They need to know the characteristics of the patientsincluded in the studies, which modalities were used to givethe intervention being evaluated, which drugs and dosageswere used, and they need a complete list of the outcomemeasures evaluated and the side effects reported by the orig-inal papers. Furthermore, they need to understand to what

degree these studies are in line with their knowledge of thephysio-pathology and of the natural history of the disease.This volume provides some of this information. It isintended to fill the gap between guidelines and primarystudies as well as between primary and secondary scientificmedical literature.

Book structure

This book has three sections and 24 chapters. Part 1 includesfour introductory chapters on methodological issues relatedto evidence-based neurology (EBN). Part 2 (8 chapters) covers some routine interventions for symptoms common toseveral neurological diseases. Part 3 (12 chapters) covers arange of common or important illnesses and conditions.

We followed the Cochrane Collaboration guidelines forsearch strategies (Cochrane Reviewers’ Handbook 4.2.2;Chapter 5: Locating and selecting studies for reviews) unlessotherwise specified in each chapter. We used different typesof studies to present the best possible summary of the evi-dence, joining a number of different sources using the usualhierarchical order proposed by the evidence-based move-ment. We preferred systematic reviews (Cochrane and non-Cochrane) as first choices. Randomized clinical trials (RCTs)were considered the gold standard. Other types of studieswere only used if clinical questions could be better answeredwith non-randomized studies. We tried to select articles andstudies of good methodological quality and to provide a crit-ical appraisal of those of lower levels of quality.

We graded evidence in few cases because we didn’t wantto provide guidelines or recommendations. When we used agrading system we followed the European Federation ofNeurological Societies (EFNS) grading system (EuropeanJournal of Neurology 2004; 11: 577–81).

The selection of chapter titles and topics was done in col-laboration with the editors and the authors of each chapter.We tried to identify important and common clinical condi-tions seen in neurological wards and outpatient clinics.

Each chapter starts with a brief definition of the clinicalcondition/s, together with its/their incidence and natural his-tory. A clear formulation of a real-world scenario or question

x

Preface xi

usually follows. The questions are structured using the stan-dard three elements of patient, intervention, and outcome.The section covering critical review of the evidence for eachquestion is mostly devoted to the presentation of the findings.The results are as quantitative as possible. Quantifying theexpected benefit/harm for any intervention, rather than com-ing up with a single yes-or-no answer provides useful elem-ents for deciding whether the expected benefit outweighs thepotential harm. It also gives insight into the probability for apatient to gain or lose if treated with the intervention. Tablessummarizing the quantitative, and, when this was not possible,the qualitative, results are included in each chapter. For eachcomparison, the tables list the number of patients, the risk in the control group, the relative risk (RR), and the absoluterisk reduction (ARR). The weighted mean difference (WMD) isused for continuous outcome variables. A qualitative descrip-tion of the results is provided when quantitative data are notavailable from the primary studies. Some chapters include theimplications that the findings could have on clinical practiceand on organization of health care, as well as an insight intothe questions that have no answers and for which further evi-dence is required for better informed decisions.

Within this general framework among chapters, differencesremain due to an intrinsic non-homogeneity of the primaryreference studies, and also to the different cultural back-grounds of the internationally represented authors. This couldbe a limitation of the volume, or indeed a strength, making itmore lively and nearer to real clinical needs. We hope thatwith future updating, which will of course be necessary (mostof our references do not supersede 2006), we will be able toimprove the format and the homogeneity of the chapters.

Finally, a few people deserve special acknowledgement fortheir contribution to this book. Debbie Jordan was responsiblefor all communications. She served as a valuable liaisonbetween the publisher, editors and authors. Rodrigo Salinascontributed to the initial concept, helping to outline the chap-ter titles and topics. Mary Banks at Blackwell Publishing pro-vided capable and consistent support throughout the project.I would like to express my sincere appreciation for this work.

Livia Candelise

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PART 1

Introduction