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NEUROLOGICAL
PRACTICE
This page intentionally left blank
NEUROLOGICAL
PRACTICE
SECOND EDITION
Consulting Editor

Noshir H Wadia
MD, FRCP, FAMS, FNA, FASc, D Sc (Hons)
Director Emeritus, Department of Neurology
Jaslok Hospital and Research Centre, Mumbai
Former Professor of Neurology, Consultant Neurologist for Life

Editor
The J J Group of Hospitals and Grant Medical College
Mumbai, INDIA

Satish V Khadilkar
MD, DM, DNBE, FIAN
Professor and Head
Department of Neurology
Grant Medical College and Sir J J Group of Hospitals
Mumbai
Consultant Neurologist
Bombay Hospital Institute of Medical Sciences
Mumbai, INDIA

ELSEVIER
A division of
Reed Elsevier India Private Limited
Neurological Practice, 2e
Wadia & Khadilkar

© 2015 Reed Elsevier India Private Limited.

Second Edition 2015

First Edition 2005

All rights reserved. No part of this publication may be reproduced or transmitted in any form or by any means, electronic
or mechanical, including photocopying, recording, or any information storage and retrieval system, without permission in
writing from the Publisher.

This book and the individual contributions contained in it are protected under copyright by the Publisher (other than as may
be noted herein).

ISBN: 978-81-312-3755-7
eISBN: 978-81-312-3874-5
epub ISBN: 978-81-312-3848-6

Notices

Knowledge and best practice in this field are constantly changing. As new research and experience broaden our
understanding, changes in research methods, professional practices, or medical treatment may become necessary.
Practitioners and researchers must always rely on their own experience and knowledge in evaluating and using
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 Dedication

Studying neurological diseases in India for over

five decades gave him a unique perspective of the
disease spectrum encountered in India and he often
pointed out differences in the disease patterns during
his teaching classes. In the late 90s, he felt the need
to put these aspects of Indian neurology in one place,
in the form of a book and the first edition of the book
Neurological Practice: An Indian Perspective was
conceived. With years of persistent efforts, the first
edition was published in 2005. The book then, was
the single composite source of information on Indian
Neurology.
Since the publication of the first edition, a decade
has gone by and further information on neurological
diseases seen in India has accumulated. Dr Wadia
considered it appropriate to publish the second edi-
tion of the book, updating new information and de-
leting diseases having less relevance to current times.
While he chose to be called the Consulting Editor,
Dr N H Wadia has been a pioneer in studies of neuro- he has been fully active throughout the preparations
logical diseases in India. He has had a very distin- of this edition. He carefully decided the topics, com-
guished, multifaceted career as a clinical neurologist, municated his thoughts to authors and avidly went
academic neurologist, a researcher and a teacher par through the manuscripts as they came in. His sug-
excellence to generations of neurologists in India. He gested modifications have enhanced the usefulness
has published seminal articles on a variety of neuro- of the information.
logical disorders; to name a few, craniovertebral anom- This second edition is a tribute to the persistent
alies, tuberculosis of the CNS, nutritional disorders efforts of Dr Noshir H Wadia towards the goal of
of the nervous system, inherited ataxias, Enterovirus documenting information on neurological disorders
70 Disease, neurocysticercosis, etc. seen in India.
This page intentionally left blank
 Contributors

Aggarwal Annu dnb, mrcp Iype Thomas md, dm

Wilson Disease Clinic Professor and Head
Kokilaben Dhirubhai Ambani Hospital and Department of Neurology
Medical Research Institute Government Medical College
Mumbai, INDIA Thiruvananthapuram, INDIA

Alladi Suvarna md, dm J Kalita md, dm

Professor Professor
Department of Neurology Department of Neurology
Nizam’s Institute of Medical Sciences Sanjay Gandhi Postgraduate Institute
Punjagutta, Hyderabad, INDIA of Medical Sciences
Lucknow, INDIA
Bhatt Mohit md, dm
Director Karmarkar Vikram ms, dnb
Department of Neurosciences Department of Neurosurgery
Centre for Brain and Nervous System Bombay Hospital Institute of Medical Sciences
Kokilaben Dhirubhai Ambani Hospital and Mumbai, INDIA
Medical Research Institute
Mumbai, INDIA Kaul Subhash md, dm, fian
Professor and Head
Cherian Ajit md, dm Department of Neurology
Professor Nizam’s Institute of Medical Sciences
Department of Neurology Punjagutta, Hyderabad, INDIA
Government Medical College
Thiruvananthapuram, INDIA Khadilkar Satish md, dm, dnbe, fian
Professor and Head
Deopujari CE ms, mch, msc Department of Neurology
Professor and Head, Department Grant Medical College and Sir J J Group
of Neurosurgery, of Hospitals
128, MRC wing First Floor, Mumbai
Bombay Hospital Institute of Medical Consultant Neurologist
Sciences, Mumbai Bombay Hospital Institute of Medical Sciences
Mumbai, INDIA
Gururaj md, dm
Professor Krishnamoorthy ES md
Department of Epidemiology The TS Srinivasan Institute
Centre for Public Health Chennai, INDIA
National Institute of Mental Health
and Neurosciences
Bangalore, INDIA

vii
viii CONTRIBUTORS

Lalkaka JA md, dm Shankar S K md, fams, fnasc, ficp

Professor Emeritus Professor, Neuropathology and
Department of Neurology Principal Co-ordinator
Bombay Hospital Institute of Medical Sciences Human Brain Tissue Repository (Human Brain
Mumbai, INDIA Bank)
Neurobiology Research Centre
Mahadevan Anita md National Institute of Mental Health and
Professor Neurosciences
Department of Pathology Bangalore, INDIA
National Institute of Mental Health
and Neurosciences Shukla Rakesh md, dm, mnams
Bangalore, INDIA Professor
Department of Neurology
Misra UK md, dm King George’s Medical University
Professor and Head Lucknow, INDIA
Department of Neurology
Sanjay Gandhi Postgraduate Institute Singh Gagandeep md, dm
of Medical Sciences Professor and Head
Lucknow, INDIA Department of Neurology
Dayanand Medical College
Misra V md Ludhiana, INDIA
The TS Srinivasan Institute
Chennai, INDIA Singhal BS md, frcp (lond), frcp (edin), fams
Director
Netravati M md Department of Neurology
Professor Bombay Hospital Institute of Medical Sciences
Department of Neurology Mumbai, INDIA
National Institute of Mental Health
and Neurosciences Sinha Sanjib md
Bangalore Professor
Department of Neurology
Pradhan S md, dm, fams, fnasc, ficp National Institute of Mental Health
Professor and Neurosciences
Department of Neurology Bangalore, INDIA
Sanjay Gandhi Postgraduate Institute
of Medical Sciences Srivatsa VG md
Lucknow, INDIA The TS Srinivasan Institute
Chennai, INDIA
Ravishankar K md
A-64, Kalpataru Residency Tripathi Manjari md, dm
Sion, Mumbai, INDIA Professor
Department of Neurology
Satishchandra P md All India Institute of Medical Sciences
Director/Vice Chancellor New Delhi, INDIA
National Institute of Mental Health
and Neurosciences
Bangalore, INDIA
 CONTRIBUTORS ix

Udani Vrajesh md Wadia NH md, frcp, fams, fna, fasc,

Consultant, Child Neurology and Epilepsy d sc (hons)
PD Hinduja National Hospital and Medical Director Emeritus
Research Centre Department of Neurology
Mumbai Jaslok Hospital and Research Centre
Honorary Asst Professor Mumbai 400 026
Department of Paediatrics and Neurology Former Professor of Neurology, Consultant
Grant Medical College and JJ Group Neurologist for Life
of Hospitals The J J Group of Hospitals and Grant Medical
Mumbai College
Honorary Consultant Mumbai, INDIA
Department of Child Neurology
Bai Jerbai Wadia Hospital for Children
Mumbai, INDIA
This page intentionally left blank
 Preface to the Second Edition

It was the end of one of the Sunday sessions were unaware of the work done in their own
of ‘Continuums’ in April 2012, and we were departments and generally, the importance of
walking out of the auditorium, when Dr N H knowing local conditions was not well per-
Wadia mentioned to me that he was consider- ceived. As years pass by and bulk of Indian
ing revision of his book Neurological Prac- information increases, it will become increas-
tice: An Indian Perspective and would like to ingly important to have access to it in a single
go ahead with the task, if a helpful pair of place, such as this book.
hands was available. Having dabbled a little The preparation of the second edition took
in the editorial jobs with the Annals of Indian almost 18 months. During this time, we worked
Academy of Neurology and Association of from my office, Dr Wadia’s office and at other
Physicians of India, I felt ready to help him times, at his home. While I took on the respon-
and we started the preparation of the second sibility of communication, liaison, and editing
edition. the chapters, I was guided by Dr Wadia’s
One question repeatedly kept surfacing in depth of knowledge, persistence and attention
discussions with colleagues, as to whether to details. In spite of health concerns, he ac-
there was a need for a book like this, in this era, tively participated in the editorial process,
where knowledge is available at the fingertips making suggestions to improve the contents.
to all, as and when required. We decided to do It has been a very enjoyable experience for me
a small experiment and gave a seminar topic to work alongside him and to delve in nuances
on Subacute Sclerosing Panencephalitis to our of the art of writing, editing and compiling
postgraduates at the Grant Medical College information.
and saw the result after a week’s preparation. We have been fortunate to have very good
Comparing what was found on the net with contributors for this edition, some new and some
what was in the first edition of the book, it was others from the previous edition. They have all
clear that all the information in the book could done a commendable task of rewriting or revis-
not be accessed by the net, and also, a lot of ing the chapters, taking care to include all Indian
information has gathered after the first edition information on the subject. The book deals with
came to be. Doing the same exercise with areas 16 important areas of neurological practice in
of stroke and epilepsy, it was obvious that the India. Some topics do not find a place in this edi-
new Indian information needed to be collated tion, as some diseases seem to have gone away
in the second edition of the book, for ease of or become infrequent and for the present, prob-
retrieval and as supplementary information to ably not much relevant. The readers are directed
the standard textbooks. to the first edition for the details of exposition of
During my experiences as examiner for DM these topics, though brief accounts appear in the
Neurology in various parts of the country, it introduction of this edition. Two new chapters
was disturbing to realize that the DM post- namely Headache and Brain Tumours have been
graduates do not seem to consider it important added, making this edition more relevant to the
to be updated with Indian information on neu- current issues in neurological practice in our
rological diseases. In some instances, they country.

xi
xii PREFACE TO THE SECOND EDITION

I am indebted to the many individuals who I do hope that the readers find this edition
participated in more ways than one, in this useful in their practice of Neurology.
project. I would like to thank my wife Suvarna
and my children, Ankeeta and Rahul, for their Satish V Khadilkar
understanding and cooperation during the
many hours I spent in the preparation of the
book.
 Preface to the First Edition

It may well be asked why a book entitled important information has been gathered,
“Neurological Practice” is written when there demanding this somewhat oversized book and
are more comprehensive volumes on neurology many contributors, some of whom have added
already available. The answer is in the subtitle much to the medical literature through their
– an Indian perspective. Very early in my prac- observations and writings.
tice in the late 1950s, I was struck by the Within the covers of this book are included
contrast in the general pattern of neurological disorders as varied as common infections of the
diseases from what I had seen in Britain during nervous system, a unique adult poliomyelitis
my training days. When I saw a patient with following acute haemorrhagic conjunctivitis,
clinical manifestations of a cerebral tumour and diseases caused by iodine and protein energy
made a diagnosis of a glioma, more often than deficiencies, hot water epilepsy, newly identi-
not a tuberculoma turned up at surgery, and fied hereditary ataxias, fluorosis, lathyriasis,
there were no investigations available to pre- snake and shell fish poisoning, sequelae of
dict the nature of the tumour; this demanded congenital atlanto-axial dislocation, a megalen-
devising a new approach in the management of cephalic leukodystrophy and much else that
such patients. Soon other neurological disor- affects the nervous system of Indians. There is
ders appeared such as I had not seen frequently; also reference to prevalence data of well-known
and I did not see others, like multiple sclerosis, diseases like epilepsy, stroke, dementia, move-
so common in the West. And over the time it ment disorders, comparing it with reports from
became increasingly clear that even the most elsewhere, which may provide clues to better
exhaustively written volumes on neurology did understanding of these diseases.
not and possibly could not deal sufficiently Hopefully then, this book will find a place on
well with diseases of much interest and concern the book shelves of students and those who
to peoples outside the western world. This did have to treat Indians at home or abroad. And
not mean that such texts were irrelevant for perhaps even be useful for their subcontinental
populations beyond their compass; it only indi- neighbours.
cated the need for supplementary books, cover- Having said all this, it is with much pleasure
ing newly gathered information essential for that I thank all those who have helped me to put
treatment of locally more prevalent diseases. this book together. I owe an immense debt of
This, I felt was more so for populous countries gratitude to Dr Piroja Wadia, my often unappre-
like India where one-fifth of the world’s popu- ciated and silent partner in much of my work
lation reside and whose diaspora has spread far and research, without whose encouragement
and wide, carrying their diseases with them at this book would not have been written.
least in the immediate term. I cannot thank enough the trustees and
This, I hope is one such book. administration of the Jaslok Hospital and
Some 30 years ago when I first thought of Research Centre, Mumbai, where I continue to
writing a book, it would have been less than half practice, for providing me with every facility I
this size and probably written entirely by me. required and bearing the considerable expenses
But in the ensuing years much interesting and which came along with it.

xiii
xiv PREFACE TO THE FIRST EDITION

I will be considerably remiss if I did not put the text understanding the not easy task that I
on record what I owe to all those who were had undertaken. It has given me much pleasure
with me at the JJ Hospital where I spent a large collaborating with them.
part of my making day in the first 25 years of Finally, my apologies as no book can cover
my career. The administration, the patients every subject. My colleagues and I have tried to
whose illnesses I tended to, and who were will- gather as much of the important Indian litera-
ing subjects of my observations, the medical ture on neurology as possible, but inevitably
students, the residents and research assistants, there will be some authors or contributions not
my colleagues, especially the late Dr Darab mentioned, or reference to their publication not
Dastur, the nursing and other staff, each in included. My apologies to them; I hope they will
their own way energised me to do more than understand.
what I was capable of. I also recognise that important neurological
I recognise and appreciate the effort of the disorders like headache, myasthenia gravis,
contributors who have so fulsomely given their have not found a place. But I had to keep the
time and skill to contribute chapters to this book size of the book within limits and only include
and who have respected my editorial privileges those diseases where sufficient published infor-
without demur. I have taken help from my mation was available. Perhaps another volume
departmental colleagues and other staff when- can always be added by a kindred soul, which
ever I needed, which I sincerely acknowledge. could also include diseases managed mostly by
Asha Padiyar, my principal secretary has neurosurgeons.
laboured through many hours typing repeated My earnest hope is that successive editions of
drafts, correcting manuscripts and as far as this book will expand its scope and continue to
possible the reference lists. She was joined later reflect the increasing contributions of Indian
by Jasmin Daruwalla and my clinical assistant, Neurology to world literature through observa-
Dr Annu Aggarwal. I am grateful to them. tions and research carried out by Indians in India.
Elsevier Science readily agreed to publish this
book and patiently waited for me to complete Noshir H Wadia
 Acknowledgements

Dr Satish Khadilkar thanks Dr Chetan Choudhari Public Health, NIMHANS for all help and sup-
and Dr Ahshish Bhutada for their invaluable help port in literature pooling and review.
during the editing of manuscripts. Dr Wadia Dr Mohit Bhat and Dr Annu Aggarwal thank
thanks Ms Asha Padiar for the secretarial help. Dr Uday Muthane and Dr Noshir Wadia for
Dr Subhash Kaul and Dr Suvarna Alladi their inputs from the first edition.
thank Shailaja Mekala for technical assistance Dr Chandrashekhar Deopujari and Dr Vikram
and support in editing the manuscript. Karmarkar thank Drs Vishwaraj Rath and
Dr Satishchandra and Dr Gururaj acknowl- Shraddha Maheshwari for their assistance in
edge the help and support of Dr Senthil Amudan, preparing the manuscript.
Assistant Professor of Epidemiology, Centre for

xv
This page intentionally left blank
 Brief Contents

╇ 1.╇ Acute Viral Encephalitis╅ 25 10.╇ Hereditary Ataxias╅ 261

╇ 2.╇ Subacute and Chronic Viral Infections of Nervous 11.╇ Diseases of Central Nervous System Myelin╅ 293
System and Prion Diseases╅ 57 12.╇ Craniovertebral Anomalies with Particular Emphasis
╇ 3.╇ Neurotuberculosis╅ 117 on Congenital Atlantoaxial Dislocation╅ 309
╇ 4.╇ Neurocysticercosis╅ 137 13.╇ Peripheral Neuropathies╅ 347
╇ 5.╇ Cerebrovascular Diseases╅ 171 14.╇ Disorders of the Muscles╅ 385
╇ 6.╇ Epilepsies╅ 195 15.╇ Paediatric Neurology╅ 429
╇ 7.╇ Headache╅ 209 16.╇ Tumours of Central Nervous System╅ 469
╇ 8.╇ Dementias╅ 219
╇ 9.╇ Movement Disorders╅ 241

xvii
This page intentionally left blank
 Detailed Contents

Contributors╅ vii 2.╇ Japanese Encephalitis╅ 29

2.1╇ Introduction╅ 29
Preface to the Second Editionâ•… xi
2.2╇ History and Epidemiology (India)╅ 30
Preface to the First Edition╅ xiii 2.3╇ Seasonal Variation╅ 30
Acknowledgements╅ xv 2.4╇ Vector╅ 31
2.5╇ The JE Virus╅ 32
Introduction 2.6╇ Pathogenesis╅ 32
KHADILKAR SV 2.7╇ Immunology╅ 33
2.8╇ Pathology╅ 33
1.╇ Infections╅ 1 2.9╇ Clinical features╅ 35
1.1╇ Viral╅ 1 2.10╇ Investigations╅ 38
1.2╇ Prion Diseases╅ 2 2.11╇ Management╅ 41
1.3╇ Mycobacterial╅ 2 2.12╇ Prognosis and sequelae╅ 41
1.4╇ Parasitic╅ 3 2.13╇ Prevention╅ 42
2.╇ Epilepsy╅ 3 3.╇ Dengue╅ 43
3.╇ Cerebrovascular Diseases╅ 3 3.1╇ Dengue Virus╅ 44
4.╇ Movement Disorders╅ 4 3.2╇ Pathogenesis╅ 44
5.╇ Headaches╅ 5 3.3╇ Clinical Features╅ 45
6.╇ Brain Tumours╅ 6 3.4╇ Neurological Manifestations of Dengue╅ 46
7.╇ Dementias╅ 6 3.5╇ Diagnosis╅ 48
8.╇ Hereditary Ataxias╅ 6 3.6╇ Management╅ 48
9.╇ Diseases of Central Nervous System Myelin╅ 7 3.7╇ Outcome╅ 48
10.╇ Congenital Craniovertebral Anomalies╅ 8 3.8╇ Prevention╅ 49
11.╇ Peripheral Neuropathies╅ 9 4.╇ Chikungunya╅ 49
12.╇ Myopathies╅ 10 4.1╇ Epidemiology╅ 49
13.╇ Paediatric Neurology╅ 10 4.2╇ Clinical Picture╅ 49
14.╇ Infectious Diseases╅ 11 4.3╇ Neurological Manifestations in Newborns╅ 50
15.╇ Malnutrition╅ 12 4.4╇ Autopsy╅ 51
16.╇ Environmental Diseases╅ 13 4.5╇ Treatment╅ 51

1.╇ Acute Viral Encephalitis 2.╇ Subacute and Chronic Viral Infections
MISRA UK, KALITA J of Nervous System and Prion Diseases
ANITA MAHADEVAN, SANJIB SINHA, NETRAVATHI M,
1.╇ Herpes Simplex Encephalitis╅ 25 SATISHCHANDRA P, SHANKAR SK
1.1╇ Epidemiology╅ 25
1.2╇ Latency and Activation╅ 26 1.╇ Introduction╅ 57
1.3╇ Pathogenesis╅ 26 2.╇ Neurobiology of HIV/AIDS╅ 57
1.4╇ Clinical Presentation╅ 27 2.1╇ Introduction╅ 57
1.5╇ Investigations╅ 27 2.2╇ Epidemiology╅ 58
1.6╇ Treatment╅ 28 2.3╇ HIV Subtypes in India╅ 58
1.7╇ Outcome╅ 29 2.4╇ Neurological Manifestations of HIV╅ 59

xix
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