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A DICTIONARY OF NEUROLOGICAL SIGNS THIRD EDITION A DICTIONARY OF NEUROLOGICAL SIGNS THIRD 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. 123 Andrew J. Larner MA MD MRCP (UK) DHMSA Walton Centre for Neurology & Neurosurgery Lower Lane L9 7LJ Liverpool, UK ISBN 978-1-4419-7094-7 e-ISBN 978-1-4419-7095-4 DOI 10.1007/978-1-4419-7095-4 Springer New York Dordrecht Heidelberg London Library of Congress Control Number: 2010937226 © Springer Science+Business Media, LLC 2001, 2006, 2011 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, LLC, 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 dissimilar 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 proprietary 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 on acid-free paper Springer is part of Springer Science+Business Media (www.springer.com) To Sue Nil satis nisi optimum CECILY: ...Dr Chasuble is a most learned man. He has never written a single book, so you can imagine how much he knows. Oscar Wilde The importance of being earnest: 1899, Act III The tendency to appear exact by disregarding the complexity of the factors is the old failing in our medical history. J. Curnow Cited in: Critchley M, Critchley EA. John Hughlings Jackson. Father of English Neurology. New York: Oxford University Press, 1998: 106 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, neuroimag- ing 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 compre- hensive, 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 - ix - PREFACE TO THIRD EDITION To paraphrase John Hughlings Jackson (1835–1911), clinical phenomena are experiments on the nervous system made by disease. Neurological signs might therefore be (loosely) characterized as the ‘dependent variables’ of the experi- ments wrought by neurological disease. Observing or eliciting these signs may therefore give insight into neurological disease processes. (Details of neurolog- ical disorders mentioned in passing in this book may be found elsewhere, in a companion volume.1) However, as mentioned in the preface to the first edition of this book,2 the accuracy and precision of most neurological signs remain to be defined at the level of traditional probabilistic parameters (specificity, sensitivity, positive and negative predictive values, likelihood ratios, etc.), perhaps because this undertaking is a less alluring prospect for research than, say, neuroimaging and neurogenetics. Thankfully, the clinical examination still has some supporters (not merely apologists), and neurological signs feature prominently amongst the core competencies.3 Clinicians, as opposed to academics, may be ideally placed to undertake the aforementioned studies in the context of their day-to-day clinical practice. A.J. Larner REFERENCES 1. Larner AJ, Coles AJ, Scolding NJ, Barker RA. The A-Z of neurological practice. A guide to clinical neurology (2nd edition). London: Springer, in press. 2. Larner AJ. A dictionary of neurological signs. Clinical neurosemiology. Dordrecht: Kluwer, 2001: xii. 3. Verghese A, Horwitz RI. In praise of the physical examination. BMJ 2009; 339: b5448. - xi - ACKNOWLEDGEMENTS In preparing this third edition, particular thanks are due to friends and colleagues who have commented on the earlier editions, namely (in alphabetical order) Alasdair Coles, Anu Jacob, Alex Leff, Miratul Muqit, Parashkev Nachev, and Sivakumar Sathasivam. At Springer, I am grateful for support and encour- agement received from Paula Callaghan, Lindsey Reilly, Brian Belval, Brian O’Connor, Richard Lansing, and Manika Power. All errors and shortcomings whichremainareentirelymyownwork. - xiii - CONTENTS Foreword to the First Edition by Martin N. Rossor ix Preface to Third Edition xi Acknowledgements xiii A: Abadie’s Sign to Autotopagnosia 1 B: Babinski’s Sign to Butt-First Manoeuvre 55 C: Cacogeusia to Czarnecki’s Sign 71 D: Dalrymple’s Sign to Dystonia 101 E: Ear Click to Eyelid Apraxia 125 F: Face–hand Test to Funnel Vision 135 G: Gag Reflex to Gynaecomastia 155 H: Habit Spasm to Hypotropia 165 I: Ice Pack Test to Iridoplegia 193 J: Jacksonian March to Junctional Scotoma, Junctional Scotoma of Traquair 199 K: Kayser–Fleischer Rings to Kyphoscoliosis 203 L: Lagophthalmos to Luria Test 207 M: Macrographia to Myotonia 215 N: Narcolepsy, Narcoleptic Syndrome to Nystagmus 237 - xv - O: Obscurations to Overflow 247 P: Pagophagia to Pyramidal Signs, Pyramidal Weakness 259 Q: Quadrantanopia to Quadrupedalism 303 R: Rabbit Syndrome to Ross’s Syndrome 305 S: Saccades to Synkinesia, Synkinesis 319 T: ‘Table Top’ Sign to Two-Point Discrimination 343 U: Uhthoff’s Phenomenon to Utilization Behaviour 355 V: Valsalva Manoeuvre to Vulpian’s Sign 359 W: Waddling Gait to Wry Neck 369 X: Xanthopsia to Xerophthalmia, Xerostomia 377 Y: Yawning to Yo-Yo-Ing 379 Z: Zeitraffer Phenomenon to Zoom Effect 381 - xvi - A Abadie’s Sign Abadie’s sign is the absence or diminution of pain sensation when exerting deep pressure on the Achilles tendon by squeezing. This is a frequent finding in the tabes dorsalis variant of neurosyphilis, i.e. with dorsal column disease. Cross Reference Argyll Robertson pupil Abdominal Paradox - see PARADOXICAL BREATHING Abdominal Reflexes Both superficial and deep abdominal reflexes are described, of which the super- ficial (cutaneous) reflexes are the more commonly tested in clinical practice. A wooden stick or pin is used to scratch the abdominal wall, from the flank to the midline, parallel to the line of the dermatomal strips, in upper (supraum- bilical), middle (umbilical), and lower (infraumbilical) areas. The manoeuvre is best performed at the end of expiration when the abdominal muscles are relaxed, since the reflexes may be lost with muscle tensing; to avoid this, patients should lie supine with their arms by their sides. Superficial abdominal reflexes are lost in a number of circumstances: • Normal ageing; • Obesity; • Following abdominal surgery; • Following multiple pregnancies; • In acute abdominal disorders (Rosenbach’s sign). However, absence of all superficial abdominal reflexes may be of localizing value for corticospinal pathway damage (upper motor neurone lesions) above T6. Lesions at or below T10 lead to selective loss of the lower reflexes with the upper and middle reflexes intact, in which case Beevor’s sign may also be present. All abdominal reflexes are preserved with lesions below T12. Abdominal reflexes are said to be lost early in multiple sclerosis, but late in motor neurone disease, an observation of possible clinical use, particularly when differentiating the progressive lateral sclerosis variant of motor neurone disease from multiple sclerosis. However, no prospective study of abdominal reflexes in multiple sclerosis has been reported. Reference Dick JPR. The deep tendon and the abdominal reflexes. Journal of Neurology, Neurosurgery and Psychiatry 2003; 74: 150–153. Cross References Beevor’s sign; Upper motor neurone (UMN) syndrome A.J. Larner, A Dictionary of Neurological Signs, 1 DOI 10.1007/978-1-4419-7095-4_1, C Springer Science+Business Media, LLC 2011 A Abducens (VI) Nerve Palsy Abducens (VI) Nerve Palsy Abducens (VI) nerve palsy causes a selective weakness of the lateral rectus mus- cle resulting in impaired abduction of the eye, manifest clinically as diplopia on lateral gaze, or on shifting gaze from a near to a distant object. Abducens (VI) nerve palsy may be due to: • Microinfarction in the nerve, due to hypertension, diabetes mellitus; • Raised intracranial pressure: a ‘false-localizing sign’, possibly caused by stretching of the nerve in its long intracranial course over the ridge of the petrous temporal bone; • Nuclear pontine lesions: congenital, e.g.