A DICTIONARY OF NEUROLOGICAL SIGNS

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A DICTIONARY

OF NEUROLOGICAL SIGNS

SECOND EDITION

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A DICTIONARY OF NEUROLOGICAL SIGNS

SECOND EDITION

A.J. LARNER

MA, MD, MRCP(UK), DHMSA Consultant Neurologist

Walton Centre for Neurology and Neurosurgery, Liverpool Honorary Lecturer in Neuroscience, University of Liverpool

Society of Apothecaries’ Honorary Lecturer in the History of Medicine, University of Liverpool

Liverpool, U.K.

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A.J. Larner, MA, MD, MRCP(UK), DHMSA Walton Centre for Neurology and Neurosurgery Liverpool, UK

Library of Congress Control Number: 2005927413 ISBN-10: 0-387-26214-8

ISBN-13: 978-0387-26214-7 Printed on acid-free paper.

All rights reserved. This work may not be translated or copied in whole or in part without the written permission of the publisher (Springer Science+Business Media, Inc., 233 Spring Street, New York, NY 10013, USA), except for brief excerpts in connection with reviews or scholarly analysis. Use in connection with any form of information storage and retrieval, electronic adaptation, computer software, or by similar or dis- similar methodology now known or hereafter developed is forbidden.

The use in this publication of trade names, trademarks, service marks, and similar terms, even if they are not identified as such, is not to be taken as an expression of opinion as to whether or not they are subject to propri- etary rights.

While the advice and information in this book are believed to be true and accurate at the date of going to press, neither the authors nor the editors nor the publisher can accept any legal responsibility for any errors or omissions that may be made. The publisher makes no warranty, express or implied, with respect to the material contained herein.

Printed in the United States of America. (SPI/EB) 9 8 7 6 5 4 3 2 1

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To Philippa, Thomas, and Elizabeth

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“ ... there are many works useful and even necessary, which require no genius at all; and dictionary making is one of these.”

James Burnet, Lord Monboddo.

Of the origin and progress of language: 1773-1792: V, 273

“I know ... that Writers of Travels, like Dictionary-Makers, are sunk into Oblivion by the Weight and Bulk of those who come after, and therefore lie uppermost.”

Jonathan Swift Gulliver’s Travels: 1726 FM.qxd 9/28/05 11:10 PM Page vii

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FOREWORD TO THE FIRST EDITION

Neurology has always been a discipline in which careful physical examination is paramount. The rich vocabulary of neurology replete with eponyms attests to this historically. The decline in the importance of the examination has long been predicted with the advent of more detailed neuroimaging. However, neuroimaging has often provided a surfeit of information from which salient features have to be identified, dependent upon the neurological examination. A dictionary of neurological signs has a secure future.

A dictionary should be informative but unless it is unwieldy, it cannot be comprehensive, nor is that claimed here. Andrew Larner has decided sensibly to include key features of the history as well as the examination. There is no doubt that some features of the history can strike one with the force of a physical sign. There are entries for

“palinopsia” and “environmental tilt” both of which can only be elicited from the history and yet which have considerable significance.

There is also an entry for the “head turning sign” observed during the history taking itself as well as the majority of entries relating to details of the physical examination.

This book is directed to students and will be valuable to medical students, trainee neurologists, and professions allied to medicine.

Neurologists often speak in shorthand and so entries such as

“absence” and “freezing” are sensible and helpful. For the more mature student, there are the less usual as well as common eponyms to entice one to read further than the entry which took you first to the dictionary.

Martin N. Rossor Professor of Clinical Neurology National Hospital for Neurology and Neurosurgery Queen Square London

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PREFACE TO THE SECOND EDITION

As in the first edition, the belief in signs as signifiers underpins this text. The aim is to be true to the “methode anatomo-clinique” pio- neered in neurology by Charcot,¹but also integrating, where possible, data from newer sources such as neuroimaging and neurogenetics.

Certain omissions in the first edition have become evident to me, necessitating a second edition (moreover, according to Michael Holroyd, “Collectors like first editions, authors like second editions”).

Just under 700 entries have now expanded to a little under 1000. Most signs should be elicitable with the kit typically carried by neurologists.² New features include a greater emphasis on change in signs with ageing and more medical history. Perspective on which signs are “really important” has been addressed elsewhere.³ Details of neurological conditions associated with the various neurological signs are not dis- cussed in any depth. Readers are encouraged to consult appropriate texts, for one of which the author has a particular, and hopefully excusable, bias.⁴

Neurological signs, like neurological diagnoses, are medical constructs and, hence, cultural artefacts liable to change with time. Hence, all definitions are seen as provisional rather than fixed. Systematic studies which “operationalize” signs, both how to elicit them and how to rate responses,⁵ alone will define their utility in terms of sensitivity and specificity.

A.J. Larner

REFERENCES

1. Goetz CG, Bonduelle M, Gelfand T. Charcot: constructing neurology.

Oxford: OUP, 1996

2. Warner GTA. A typical neurological “case”. Practical Neurology 2003; 3: 220-223

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3. Larner A, Niepel G, Constantinescu C. Neurology. In: Ali N (ed.).

Alarm bells in medicine. Oxford: Blackwell, 2005: 73-77

4. Barker RA, Scolding NJ, Rowe D, Larner AJ. The A-Z of neurologi- cal practice. A guide to clinical neurology. Cambridge: CUP, 2004 5. Franssen EH. Neurologic signs in ageing and dementia. In: Burns A

(ed.). Ageing and dementia: A methodological approach. London:

Edward Arnold, 1993: 144-174

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PREFACE TO THE FIRST EDITION

In writing a book devoted to neurological signs and their meaning, it is not my intention to undervalue in any way the skill of neurological history taking. This remains the key element of the doctor-patient encounter both in the neurological clinic and on the ward, and is clearly crucial in order to formulate diagnostic hypotheses, guide clinical examination, and help decide on the nature of the pathological process (if one is present). However, having sat through several thou- sand neurological consultations, I do not subscribe to the view that all one need do is listen carefully and the patient will “tell you the diagnosis”, although this may happen on rare (and often memorable) occa- sions. Clearly, history taking is not simply a passive recording of symptoms (“what the patient complains of”), but also an active process of seeking information of possible diagnostic significance through appropriate questions; this might be called the “historical examination”. This latter facet of history taking, much the more diffi- cult skill to learn, may disclose certain neurological signs which are not available to physical examination (principally in the sensory domain, but also intermittent motor phenomena). Hence, my use of the term

“sign” in this book is a broad one, encompassing not only findings in physical examination (its traditional use) but also from focused history taking. My operational definition of sign is therefore simply a “signi- fier”, in the sense of phenomena of semiologic value, giving information as to anatomical location and/or pathological cause.

Most neurological textbooks adopt an approach which is either symp- tom-based, beginning with what the patient complains of and then offering a structured differential diagnosis; or disease-based, assuming that a diagnosis has already been established. Although such texts are of great value, it seems to me that this does leave a place for a book devoted to neurological signs. Signs, elicited in either the historical or neurological examination, bridge the gap between the patient’s symptoms, and the selection of appropriate investigations to confirm or refute the exam- iner’s diagnostic formulations and thus establish a diagnosis.

Although it has been mooted whether the dramatic technological advances in neurological practice, for example in neuroimaging, might render neurological examination redundant, others maintain the cen- tral importance of neurological examination in patient management.^1,2

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It will come as little surprise to the reader that I am emphatically of the latter persuasion. However, this book does not aim to be a handbook of neurological examination technique (one reason for the absence of pictures), or neurological investigation, many excellent examples of which already exist. Rather, it seeks to elucidate the interpretation of neurological signs (“neurosemiology”): their anatomical, physiolog- ical, and pathological significance (where these are known). It should be added quickly that this is not to suggest that neurological signs are peculiarly objective (as some systems of clerking might suggest): as with all clinical observations, neurological signs are subject to both inter- and intra-observer variation and are biased by prior knowledge of the history and other examination findings.^3-5As with other ele- ments of clinical examination, relatively little study of the accuracy and precision of neurological signs has been undertaken; a methodology to remedy this situation has been suggested.⁶It is hoped that the current work might encourage more such studies. To those who might suggest that, in an age of molecular genetics, such an undertaking is passé, and rather nineteenth-century in its outlook, I would argue that precision in the definition of clinical signs is of relevance if meaning- ful genotype/phenotype correlations are to be established.

An attempt has been made to structure the entries in this volume in the following way:

● a definition of the sign, or the common usage of the term (sub- types italicized);

● a brief account of the clinical technique required to elicit the sign;

● a description of other neurological signs which may accompany the index sign (cross referenced as appropriate).

Where known, there is appended:

● a brief account of the neuroanatomical basis of the sign;

● an explanation, where possible, of the pathophysiological and/or pharmacological basis of the sign;

● the neuropathological basis of sign;

● a differential diagnosis of the commonest clinical diseases causing or associated with the sign (bulleted);

● brief details of specific treatments of these disorders, if available.

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Using this schema, it will hopefully prove possible to integrate clinical phenomenology with the underlying neuroscience (anatomy, physiol- ogy, and pathology) in an accessible manner which will facilitate assimilation by the reader. Clearly not all these factors are known or applicable for every sign, and hence definitions vary quite considerably in length, the longer entries generally being for signs of greater clinical importance. Salient references from the primary and secondary litera- ture are given, particularly for the more uncommon signs, for those wishing to pursue topics further. Entries are cross-referenced to other relevant signs.

Clearly such an undertaking cannot hope to be (and does not claim to be) comprehensive, such is the diversity of neurological function.

Moreover, the limitations of my personal clinical experience means that selections are inevitably somewhat arbitrary, precluding (at the very least!) inclusion of signs familiar in pediatric neurological practice. Dermatological signs of potential neurological relevance have also been largely overlooked, and after much consideration “bruit” has been omitted. Nonetheless, it is hoped that this book will be of use to all students of neurology, both undergraduate and postgraduate, both dedicated neurology trainees and those required, perhaps against their personal inclinations, to develop some familiarity with neurology for examination purposes (e.g. candidates for the MRCP). It may also serve as a book of reference for more experienced clinicians. Since the majority of patients with neurological symptoms and signs in the United Kingdom are currently seen by general practitioners and general physicians, a situation which is likely to persist for some time, if not indefinitely,⁷it is very much hoped that these groups will also find the book of use, as indeed may members of ancillary professions: nurs- ing, physiotherapy, speech and language therapy, occupational therapy, radiography.

The definitions given are not conceived of as in any way immutable.

Language, after all, is plastic with respect to meaning and usage, and my aim is certainly not to “fix” the language. Nor do I suppose, despite my indebtedness to many distinguished colleagues, that I have been free from errors, all of which are my own doing. I shall be happy to hear from those who find errors, disagree with my suggested definitions, or feel that important signs have been omitted.

A.J. Larner

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REFERENCES

1. Ziegler DK. Is the neurologic examination becoming obsolete?

Neurology1985; 35: 559

2. Caplan LR. The effective clinical neurologist. Oxford: Blackwell Scientific 1990

3. Stam J, van Crevel H. Reliability of the clinical and electromyo- graphic examination of tendon reflexes. Journal of Neurology 1990; 237: 427-431

4. Maher J, Reilly M, Daly L, Hutchinson M. Plantar power: repro- ducibility of the plantar response. BMJ 1992; 304: 482

5. Hansen M, Sindrup SH, Christensen PB, et al. Interobserver vari- ation in the evaluation of neurological signs: observer dependent factors. Acta Neurologica Scandinavica 1994; 90: 145-149 6. McAlister FA, Straus SE, Sackett DL, on behalf of the CARE-

COAD1 Group. Why we need large, simple studies of the clinical examination: the problem and a proposed solution. Lancet 1999;

354: 1721-1724

7. Neurology in the United Kingdom: Towards 2000 and beyond.

London: Association of British Neurologists 1997

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ACKNOWLEDGMENTS

In preparing this second edition, particular thanks are due to friends and colleagues who commented on the first edition, namely (in alphabetical order) Alasdair Coles, Simon Kerrigan, Paul Jarman, Alex Leff, Dora Lozsadi, Michael and Sally Mansfield, Miratul Muqit, and Kathryn Prout. Thanks are also due to Dr.

J.R. Ponsford for a helpful review of the book (Brain 2003; 126:

508-510). All the errors and shortcomings which remain are entirely my own work.

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CONTENTS

Foreword to the First Edition by Martin N. Rossor ix

Preface to the Second Edition xi

Preface to the First Edition xiii

Acknowledgments xvii

A: Abadie’s Sign to Autotopagnosia 1

B: Babinski’s Sign to “Butt-First Maneuver” 50

C: Cacogeusia to Czarnecki’s Sign 64

D: Dalrymple’s Sign to Dystonia 87

E: Ear Click to Eyelid Apraxia 108

F: “Face-Hand Test” to Funnel Vision 116

G: Gag Reflex to Gynecomastia 133

H: Habit Spasm to Hypotropia 141

I: Ice Pack Test to Iridoplegia 168

J: Jacksonian March to Junctional Scotoma,

Junctional Scotoma of Traquair 174

K: Kayser-Fleischer Rings to Kyphoscoliosis 178 L: Lagophthalmos to Lower Motor

Neurone (LMN) Syndrome 182

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M: Macrographia to Myotonia 190 N: Narcolepsy, Narcoleptic Syndrome to Nystagmus 210

O: Obscurations to Overflow 219

P: Pagophagia to Pyramidal Signs, Pyramidal

Weakness 231

Q: Quadrantanopia to Quadriparesis, Quadriplegia 268

R: Rabbit Syndrome to Rubral Tremor 269

S: Saccades to Synkinesia, Synkinesis 281

T: “Table Top” Sign to Two-Point Discrimination 302 U: Uhthoff’s Phenomenon to Utilization Behavior 312

V: Valsalva Maneuver to Vulpian’s Sign 316

W: Wadding Gait to Wry Neck 324

X: Xanthopsia to Xerophthalmia, Xerostomia 330

Y: Yawning to Yo-yo-ing 331

Z: Zooagnosia 332

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