Neurology Study Guide
Neurology Study Guide
Oral Board Examination Review
Teresella Gondolo, MD
Library of Congress Cataloging-in-Publication Data Gondolo, Teresella.
Neurology study guide : oral board examination review / Teresella Gondolo.
p. ; cm.
Includes bibliographical references and index.
ISBN 0-387-95565-8 (s/c : alk. paper) 1. Neurology—Examinations, questions, etc. 2.
Physicians—Licenses—United States—Examinations—Study guides.
[DNLM: 1. Neurology—Examination Questions. WL 18.2 G637n 2005] I.
Title.
RC346.G655 2005 616.8⬘076—dc21 2002029449
ISBN 0-387-95565-8 Printed on acid-free paper.
䉷 2005 Springer ScienceⳭBusiness Media, Inc.
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Preface
The idea of a book to help neurologists prepare for the oral part of the Neurology Board Examination stemmed from numerous exchanges with colleagues on how they prepared for this important exam. Nobody seemed to have the magic formula to maximize their chances of passing and there were wide disparities of opinion on what they considered the best preparation. Some recommendations were based on often inaccurate impressions, others were the distorted product of their stressful experience while taking the test. On one thing everyone seemed to agree: There is not a single book available that systematically addresses the specifics of this crucially important test.
The task was daunting because the scope of knowledge required to pass the test is as wide as the field of clinical neurology itself. To make it relevant to the experience of the test it was clear that a good preparation needed to be based on practical advice on the technical aspects of the exam as well as on the proper attitude in taking it. Moreover, filling a void in the current neurology literature, an adequate preparation had to be based on cases and their discussion on evidence-based clinical literature.
Although primarily conceived for neurologists preparing for part 2 of the exam, this book intends to provide interesting case-based material to practicing adult and child neu- rologists, educators, academicians, supervisors, residents, and medical students.
The book is divided into two parts. Part 1 is devoted to practical tips on the exam’s structure, its etiquette, and preparation. Particular emphasis is placed on reasons for failing the exam. Part 2 concerns itself with the adult and pediatric vignettes part of the oral Board. Each vignette is presented in a format similar to the one candidates find at the exam. The case is then comprehensively formulated with a differential diagnosis, most likely diagnosis, and treatment recommendations. Where relevant, potential pitfalls, dos and don’ts, musts and shoulds, and frequently asked questions complement the case discussion.
TERESELLAGONDOLO, MD
Foreword
What many Neurology residents do not realize is that they are preparing for the oral board examination every day. Presentations at rounds, at conferences, and even informal discus- sions regarding differential diagnosis and potential treatment plans are the “stuff” of the oral boards. Anxiety about the boards, however, is common to almost all trainees. And is doesn’t seem to get better even with increasing clinical experience. One reason for this anxiety is that the Boards are shrouded by a veil of anecdotal experiences and myth, passed down with a variable degree of embellishment and probably a lot of inaccuracy. In fact, they are a highly structured and practical exercise in assuring the basic competence newly minted Neurologists.
There is no magic formula for passing—solid training, broad experience and clear think- ing are all basic requirements. But a prescription for failure is a lack of preparation, which ideally includes not only knowledge of Neurology, but also an understanding of what is expected by the examiners. The exam structure provides relatively little time to present oneself (to a group of strangers, no less) as a competent and caring physician. Preparing for this interaction is essential. Many training programs have instituted mock oral board examination in order to better prepare trainees specifically for the exam. A formal syllabus for this exercise has been lacking.
In this book Dr. Gondolo provides that syllabus, with a clear description of what to expect when taking the boards, and practical guidelines for how (and how not) to approach the exam. Examinees should pay careful attention to Part I, the part not covered during clinical Neurology training. Here Dr. Gondolo outlines clearly the structure of the oral board exam, including information on the examiners themselves: who are they and what are their expectations? This is also a guidebook of “dos” and “don’ts” for the exam process that should be taken seriously. For example, dress in a businesslike fashion, get sufficient sleep before the exam, never argue with your examiner, and (when possible) focus your discussion on topics for which you have significant knowledge.
The section on “Reasons for Failing” provides useful test-taking tips even for the smartest and most accomplished Neurologists. Dr. Gondolo reminds us that the approach to a “case”
in Neurology should always be structured and organized. Follow this path with each and every case: 1) localization, 2) differential diagnosis, 3) diagnostic workup, and 4) manage- ment plans. Straying from this path puts you at risk for overlooking essential information that could be important in convincing the examiner of your competence. Perhaps the most important function of the Boards is discussed under the heading of “The safety factor.”
First and foremost, the examiner is charged with the task of weeding out unsafe practi- tioners. Think carefully before suggesting a diagnostic test that may be risky, and never jump to a trivial diagnostic conclusion without first systematically excluding the more serious considerations.
Part II is a concise and sensible study guide of Neurologic disorders and treatments that serves as a review for board examinees, but also as a teaching tool and reference guide for more junior trainees and medical students. The case studies presented are typical of those that may be encountered during the Vignette portion of the exam, and thus are good tools for study.
What advice can I provide for the Neurologist about the take the oral board exams? Prepare well, play to your strengths, be considerate of your patients and your examiners, and get a good night’s rest. You’ve trained long and hard for this moment—make the most of it!
JONATHAND. GLASS, M.D.
Professor of Neurology and Pathology Director, Neurology Residency Training Program Emory University School of Medicine Atlanta, Georgia
Contents
Preface... v
Part 1 1 General Information ... 3
The Candidate ... 3
Presenting Yourself ... 3
The Day You Arrive... 4
The Examiners ... 4
Structure of the Examining Team ... 4
Training of Examiners ... 4
How Examiners Plan for the Session... 4
Grading ... 5
Your Interaction with the Examiners ... 5
The Anxiety Factor ... 6
Hints for Dealing with Anxiety ... 6
Information on the Board ... 6
2 The Live Patient Examination ... 9
The Room ... 9
The Time Factor: 30-Minute History and Neurological Examination ... 9
The Tool Box (Your Medical Instruments)... 10
The Patient ... 10
Differential Diagnosis and Discussion of Treatment Options ... 10
Your Interaction with the Patient ... 10
The Difficult Patient... 11
The 30-Minute Neurological History And Examination... 12
The Art of History Taking ... 12
The Neurological Examination ... 12
The 30-Minute Case Discussion and Additional Questions ... 13
3 The Vignette ... 15
Adult Vignettes... 15
How to Approach a Vignette ... 15
Adult Vignette Topics (Varies) ... 16
The Last Ten Minutes... 17
The Candidate Without a Clue ... 18
The Pediatric Vignette... 19
Age Categories... 19
4 How to Prepare for the Exam... 21
Courses ... 21
Books ... 22
Practice ... 22
5 Reasons for Failing ... 25
A Candidate’s Story ... 25
viii Contents
The Safety Factor... 26
The Information Factor ... 26
The Differential Diagnosis Factor ... 26
Localization of the Lesion ... 26
Determining the Temporal Factor ... 26
Poor Planning and Treatment ... 26
The Variability Factor ... 27
Part 2 6 Peripheral Nervous System Disorders ... 31
Motor Neuron Disease ... 31
Amyotrophic Lateral Sclerosis ... 31
Progressive Muscular Atrophy ... 32
Bulbar Palsy ... 34
Kennedy’s Syndrome ... 35
Peripheral Neuropathies ... 36
Guillain-Barre´ Syndrome ... 36
Chronic Inflammatory Demyelinating Polyradiculoneuropathy... 38
Miller Fisher Syndrome ... 40
Disorders of the Neuromuscular Junction ... 42
Botulism ... 42
Lambert-Eaton Myasthenic Syndrome ... 43
Myasthenia Gravis ... 45
Brachial Plexopathy ... 47
Femoral Neuropathy... 48
Postpartum Plexopathy ... 48
Mononeuritis Multiplex ... 49
Inflammatory Myopathies... 50
Polymyositis ... 50
Dermatomyositis... 52
Inclusion Body Myositis ... 52
7 Cerebrovascular Disorders ... 57
Sinus Thrombosis ... 57
Sagittal Sinus Thrombosis ... 57
Subarachnoid Hemorrhage... 59
Cerebellar Hemorrhage ... 61
Cerebal (Lobar) Hemorrhage... 62
Posterior Circulation Disorders ... 63
Wallenberg’s Syndrome ... 63
Vertebrobasilar Artery Syndrome... 64
Proximal Basilar Artery Occlusion... 65
Posterior Cerebral Artery Infarction ... 66
Weber’s syndrome ... 67
Carotid Artery Disease... 67
Carotid Artery Dissection ... 68
Carotid Cavernous Fistula ... 69
Temporal Arteritis ... 69
Stroke Therapy ... 70
8 Movement Disorders ... 75
Multiple System Atrophy ... 75
Progressive Supranuclear Palsy... 76
Wilson’s Disease... 77
Contents ix
Parkinson’s Disease ... 78
Dementia with Lewy Bodies ... 81
Pick’s Disease and Frontotemporal Dementia ... 82
Huntington’s Disease ... 83
9 Tumors ... 87
Pineal Tumors ... 87
Acoustic Neuroma... 88
Pituitary Adenoma ... 89
Pseudotumor Cerebri ... 89
Limbic Encephalitis... 91
Meningeal Carcinomatosis ... 92
Paraneoplastic Cerebellar Degeneration... 93
Olfactory Groove Meningioma ... 94
10 Infections ... 97
Herpes Simplex Encephalitis... 97
Herpes Zoster Vasculitis ... 98
Progressive Multifocal Leukoencephalopathy ... 100
Creutzfeld-Jacob Disease... 101
Lyme Disease... 102
Neurocysticercosis ... 104
Cytomegalovirus Polyradiculopathy ... 105
Parasitic Infections ... 106
HTLV-1 Myelopathy ... 107
11 Headache and Facial Pain ... 111
Painful Ophthalmoplegia ... 111
Subdural Hematoma ... 113
Migraine Headache ... 114
Cluster Headache ... 117
Trigeminal Neuralgia ... 118
Facial Palsy ... 119
12 Adult Seizures, Neuro-otology, and Sleep Disorders... 123
New Onset Seizures in Adults ... 123
Temporal Lobe Epilepsy... 124
Status Epilepticus in Adults ... 126
Meniere Syndrome ... 127
Narcolepsy... 129
13 Multiple Sclerosis ... 133
Multiple Sclerosis ... 133
Optic Neuritis... 136
Syringomyelia and Syringobulbia ... 137
Transverse Myelitis ... 139
14 Neurological Complications of Systemic Disorders ... 141
Wegener’s Granulomatosis... 141
Neurological Complications of Rheumatoid Arthritis... 142
Neurological Complications of Malabsorption ... 143
Neuroleptic Malignant Syndrome... 144
Vitamin B12Deficiency ... 145
Neurological Complications of Diabetes... 146
x Contents
15 Toxic and Metabolic Disorders ... 149
Wernicke-Korsakoff Syndrome ... 149
Delirium Tremens ... 150
Toxemia of Pregnancy ... 150
16 Pediatric Epilepsy ... 153
Neonatal Seizures ... 153
Infantile Spasms and Tuberous Sclerosis ... 155
Absence Seizures ... 157
Febrile Seizures ... 159
Juvenile Myoclonic Epilepsy... 160
Lennox-Gastaut Syndrome... 161
Benign Childhood Epilepsy with Centrotemporal Spikes ... 163
Status Epilepticus... 164
17 Pediatric Brain Tumors ... 167
Brainstem Glioma ... 167
18 Pediatric Neuromuscular Disorders... 169
Hypotonic Infant... 169
Spinal Muscular Atrophy Type 1 ... 171
Spinal Muscular Atrophy Type 2 ... 171
Spinal Muscular Atrophy Type 3 ... 171
Muscular Dystrophies ... 171
Duchenne’s Muscular Dystrophy ... 171
Other Muscular Dystrophies ... 173
Dermatomyositis ... 174
Infantile Botulism ... 175
Neonatal Transient Myasthenia Gravis ... 176
Charcot-Marie-Tooth Disease ... 176
Facioscapulohumeral Muscular Dystrophy... 178
Myotonic Dystrophy... 179
Periodic Paralysis... 180
Fabry’s Disease ... 181
Metabolic Myopathies... 182
McArdle’s Disease ... 182
Acid Maltase Deficiency ... 183
19 Mitochondrial Disorders... 187
Mitochondrial Encephalomyopathy ... 187
Sleep Disorders: Kleine-Levin Syndrome ... 188
Congenital Defects: Mobius’ Syndrome ... 189
20 Pediatric Ataxia ... 191
Ataxia-Telangiectasia... 191
Friedreich’s Ataxia ... 192
Posterior Fossa Tumor as Cause of Chronic Ataxia ... 193
Acute Ataxia ... 194
21 Pediatric Cerebrovascular Disorders ... 197
Paradoxical Emboli ... 197
Homocystinuria ... 199
Cerebral Hemorrhage... 200
Acute Hemiplegia ... 201
Subdural Hematoma ... 202
Contents xi
Headache... 203
Basilar Migraine... 203
Ophthalmoplegic Migraine ... 204
22 Pediatric Neurocutaneous Disorders ... 207
Neurofibromatosis... 207
23 Pediatric Movement Disorders... 209
Huntington’s Disease ... 209
Sydenham’s Chorea... 210
Dystonia Musculorum Deformans ... 212
Tic Disorders ... 213
24 Pediatric Neurometabolic Disorders... 217
Tay-Sachs Disease... 217
Krabbe’s Disease ... 219
Metachromatic Leukodystrophy... 220
Neuronal Ceroid Lipofuscinosis... 221
Adrenoleukodystrophy ... 222
25 Pediatric Infections... 225
Subacute Sclerosing Panencephalitis... 225
Gradenigo’s Syndrome... 226
Neonatal Meningitis ... 226
Index ... 229