Application of the International Classification of Diseases to Neurology \ Second Edition World Health Organization Geneva 1997 First edition 1987 Second edition 1997 Application of the international classification of diseases to neurology: ICD-NA- 2nd ed. 1. Neurology - classification 2. Nervous system diseases - classification I. Title: ICD-NA ISBN 92 4 154502 X (NLM Classification: WL 15) The World Health Organization welcomes requests for permission to reproduce or translate its publications, in part or in full. Applications and enquiries should be addressed to the Office of Publications, World Health Organization, Geneva, Switzerland, which will be glad to provide the latest information on any changes made to the text, plans for new editions, and reprints and translations already available. ©World Health Organization 1997 Publications of the World Health Organization enjoy copyright protection in accordance with the provisions of Protocol 2 of the Universal Copyright Convention. 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Typeset in Hong Kong Printed in Canada 95/10663-Best-set/Tri-Graphics-8000 Contents Preface v Acknowledgements vii Section I Introduction 1 Section II Instructions and recommendations for the use of ICD-NA 13 Section III List of block titles 19 Section IV Tabular list of neurological and related disorders 31 Section V Morphology of neoplasms 459 Index 477 Table of drugs and chemicals 565 iii Preface The Application of the International Classification of Diseases to Neurology (ICD-NA) is one of several adaptations of the Tenth Revision of the Interna­ tional Statistical Classification of Diseases and Related Health Problems (ICD-10) being produced by the World Health Organization to respond to the needs of specialist disciplines such as neurology. A previous edition of ICD-NA was developed in 1984-85 with the help of a group of experts convened by Dr L. Bolis (International Foundation Fatebenefratelli, Milan, Italy), then in charge of activities dealing with the prevention and control of neurological disorders in WHO's Division of Mental Health (MNH). Taking into account the recommendations of this expert group and advice received from the Neuroepidemiology Group of the World Federation of Neurology, chaired by the late Professor B.S. Schoenberg (National Institutes of Health, Bethesda, MD) and from other nongovernmental organizations, Professor J.-M. Orgogozo and Professor J.F. Dartigues (University of Bordeaux, France) drafted the text of the first edition in English and French. That edition provided an extensive selection, but a limited expansion, of the neurologically relevant codes of ICD-9. The present edition has been developed with the broader aim of providing an individual code for almost every neurological condition, so that a uniform classification is available for epidemiological and clinical research as well as for routine statistical reporting. The synopsis of the second edition of ICD-NA was produced by Professor W.G. Bradley (University of Miami, Miami, FL), Professor J.-M. Orgogozo, Dr N. Sartorius (then Director of WHO's Division of Mental Health) and Dr J. van Drimmelen (WHO, Geneva, Switzerland). It was discussed with repre­ sentatives of nongovernmental organizations active in the field of neurology and with experts in WHO and from various Member countries and then used as a framework for the development of the ICD-NA. The initial draft of ICD-NA was produced by Professors Bradley and Orgogozo with the help of Dr van Drimmelen on the basis of the first edition and of detailed suggestions from the Ad Hoc Committee on Disease Clas­ sification of the American Neurological Association, chaired by Professor Bradley; in the Acknowledgements section (pages ix-xii), the names of the members of the committee are marked with an asterisk. The draft was exam­ ined by nongovernmental organizations active in the field of neurology (listed on page xi) and by numerous advisers. Their valuable comments contributed v ICD-NA to the production of the penultimate version of ICD-NA, which was again reviewed by the participating nongovernmental organizations. The final version reflects the best resolution of many, often competing, needs. It should be kept in mind that ICD-NA had to be based upon the structure of ICD-10. This has prevented the introduction of some changes that were recommended, but has ensured that all of the 5-, 6- and ?-character codes of the ICD-NA can be contracted back into the original 3- or 4-character codes of ICD-10; compatibility with the official classification can therefore be main­ tained, whatever the purpose or level of utilization. The ICD-NA index was produced by Mr M. Catan and Dr van Drimmelen of WHO, using a preliminary partial draft produced by Drs H.J. Freyberger and C. Kessler (Liibeck, Germany), with extensive help from Mr A. L'Hours of WHO's Division of Health Situation and Trend Assessment. Guidance from Mr l'Hours also helped to ensure the congruence of ICD-NA with the parent ICD-10. An outline of the history of the International Classification of Diseases, infor­ mation on the structure of ICD-NA and instructions on its use are given in Sections I and II of this publication. Dr N.P. Napalkov Assistant Director-General World Health Organization vi Acknowledgements The following organizations provided invaluable support to the development of this publication: American Academy of Neurology American Neurological Association American Sleep Disorders Association European Federation of Neurological Societies European Stroke Council International Brain Research Organization International Bureau for Epilepsy International Cerebral Palsy Society International Child Neurology Association International Federation of Multiple Sclerosis Societies International Headache Society International League Against Epilepsy International Movement Disorder Society International Society of Neuropathology International Stroke Society World Federation for Mental Health World Federation of Neurology World Federation of Neurosurgical Societies Special thanks are due to Lord Walton of Detchant, whose continuing and strong support greatly facilitated the work on this publication, and to Professor Armand Lowenthal, of the World Federation of Neurology (WFN), who was particularly helpful in obtaining comments from Committees and Research Groups of the World Federation of Neurology. Administrative support for this work was provided by Ms J. Gilmore, Mrs J. Joseph and Mrs T. Drouillet. The work done by the following individuals, whose comments were particularly helpful and thoughtful, is gratefully acknowledged. Members of the Ad Hoc Committee on Neurological Classification of the American Neurological Association are indicated with an asterisk (*). *Dr R. Ackerman, Boston, MA, USA *Dr H. Adams, Glasgow, Scotland vii ICD-NA Dr Y. Agid, Paris, France Dr A. Ahmed, Karachi, Pakistan Dr L. Amaducci, Florence, Italy DrS. Araki, Kumamoto, Japan *Dr B. Amason, Chicago, IL, USA *Dr J. Bale, Iowa City, lA, USA *Dr B. Banker, Hanover, Germany Sir Roger Bannister, Oxford, England Dr B. Barac, Zagreb, Croatia Dr H. Barnett, London, Ontario, Canada *Dr R.W. Beck, Tampa, FL, USA *Dr W. Bell, Iowa City, lA, USA *Dr D.F. Benson, Los Angeles, CA, USA Dr A. Beraldelli, Rome, Italy *Dr J. Berger, Miami, FL, USA Dr N.E. Bharucha, Bombay, India Dr K.L. Bick, Washington, DC, USA Dr C.D. Binnie, London, England *Dr P. Black, Boston, MA, USA Dr J.P. Blass, New York, NY, USA *Dr J. Blavis, New York, NY, USA Dr J. Bogousslavsky, Lausanne, Switzerland *Dr W.G. Bradley, Miami, FL, USA *Dr B. Brookes, Madison, WI, USA *Dr J. Bruni, Mississauga, Ontario, Canada *Dr L. Caplan, Boston, MA, USA Dr P. Casaer, Louvain, Belgium Dr J.-P. Castel, Bordeaux, France *Dr M. Cohen, Buffalo, NY, USA *Dr M. Cole, Mayfield Heights, OH, USA *Dr P. Cooper, London, Ontario, Canada Dr R. Currier, Jackson, MS, USA *Dr D. Dalessio, La Jolla, CA, USA *Dr A. Damasio, Iowa City, lA, USA Dr J.P. Dartigues, Bordeaux, France *Dr J. Daube, Rochester, MN, USA Dr D .E. Deisenhammer, Vienna, Austria *Dr R. DeLorenzo, Richmond, VA, USA *Dr S. Diamond, Chicago, IL, USA *Dr R. Dorwart, Burlington, VT, USA *Dr D. Drachman, Worcester, MA, USA Dr C. Dravet, Marseilles, France *Dr F.E. Dreifuss, Charlottesville, VA, USA *Dr R. Duvoisin, New Brunswick, NJ, USA viii ACKNOWLEDGEMENTS *Dr P. Dyck, Rochester, MN, USA *Dr A.G. Engel, Rochester, MN, USA *Dr O.B. Evans, Jackson, MS, USA *DrS. Fahn, New York, NY, USA Dr N. Fejerman, Buenos Aires, Argentina *Dr R. Feldman, Boston, MA, USA *Dr G. Fenichel, Nashville, TN, USA *Dr P. Finelli, Providence, RI, USA *Dr R.A. Fishman, San Francisco, CA, USA Dr S. Flache, Geneva, Switzerland Dr D. Gardner-Medwin, Newcastle-upon-Tyne, England Dr C. Goetz, Chicago, IL, USA *Dr M. Gomez, Rochester, MN, USA *Dr B. Griggs, Rochester, MN, USA *Dr C. Gross, Burlington, VT, USA *Dr A. Harding, London, England Dr 0. Henriksen, Sandvika, Norway Dr P. Henry, Bordeaux, France Dr A. Huber, Zurich, Switzerland *Dr J.T. Hughes, Oxford, England *Dr C. J ablecki, San Diego, CA, USA Dr P. Jallon, Geneva, Switzerland Dr J. Jancovic, Houston, TX, USA *Dr P.J. Janetta, Pittsburgh, PA, USA Dr F.R. Jeri, Lima, Peru *Dr R.T. Johnson, Baltimore, MD, USA *Dr B. Katzman, La Jolla, CA, USA Dr J. Kesselring, Bad Ragaz, Switzerland *Dr A.