Neurology Study Guide - part 1 pps

Neurology Study GuideOral Board Examination Review Teresella Gondolo, MD... Neurology study guide : oral board examination review / Teresella Gondolo.. The idea of a book to help neurolo

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.

ISBN 0-387-95565-8 Printed on acid-free paper.

䉷 2005 Springer ScienceⳭBusiness Media, Inc.

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 ⳭBusiness 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 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, are 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 in the United States of America (APEX/EB)

SPIN 10890928 springeronline.com

9 8 7 6 5 4 3 2

The idea of a book to help neurologists prepare for the oral part of the Neurology BoardExamination stemmed from numerous exchanges with colleagues on how they preparedfor this important exam Nobody seemed to have the magic formula to maximize theirchances of passing and there were wide disparities of opinion on what they considered thebest preparation Some recommendations were based on often inaccurate impressions,others were the distorted product of their stressful experience while taking the test On onething everyone seemed to agree: There is not a single book available that systematicallyaddresses the specifics of this crucially important test

The task was daunting because the scope of knowledge required to pass the test is aswide as the field of clinical neurology itself To make it relevant to the experience of thetest it was clear that a good preparation needed to be based on practical advice on thetechnical 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, thisbook 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’sstructure, its etiquette, and preparation Particular emphasis is placed on reasons for failingthe exam Part 2 concerns itself with the adult and pediatric vignettes part of the oralBoard Each vignette is presented in a format similar to the one candidates find at theexam The case is then comprehensively formulated with a differential diagnosis, mostlikely diagnosis, and treatment recommendations Where relevant, potential pitfalls, dosand don’ts, musts and shoulds, and frequently asked questions complement the casediscussion

TERESELLAGONDOLO, MD

What many Neurology residents do not realize is that they are preparing for the oral boardexamination every day Presentations at rounds, at conferences, and even informal discus-sions regarding differential diagnosis and potential treatment plans are the “stuff” of theoral boards Anxiety about the boards, however, is common to almost all trainees And isdoesn’t seem to get better even with increasing clinical experience One reason for thisanxiety is that the Boards are shrouded by a veil of anecdotal experiences and myth, passeddown 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 newlyminted Neurologists

There is no magic formula for passing—solid training, broad experience and clear ing are all basic requirements But a prescription for failure is a lack of preparation, whichideally includes not only knowledge of Neurology, but also an understanding of what isexpected by the examiners The exam structure provides relatively little time to presentoneself (to a group of strangers, no less) as a competent and caring physician Preparingfor this interaction is essential Many training programs have instituted mock oral boardexamination in order to better prepare trainees specifically for the exam A formal syllabusfor this exercise has been lacking

think-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 duringclinical Neurology training Here Dr Gondolo outlines clearly the structure of the oralboard exam, including information on the examiners themselves: who are they and whatare their expectations? This is also a guidebook of “dos” and “don’ts” for the exam processthat should be taken seriously For example, dress in a businesslike fashion, get sufficientsleep before the exam, never argue with your examiner, and (when possible) focus yourdiscussion on topics for which you have significant knowledge

The section on “Reasons for Failing” provides useful test-taking tips even for the smartestand 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 andevery 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 informationthat could be important in convincing the examiner of your competence Perhaps the mostimportant 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 neverjump to a trivial diagnostic conclusion without first systematically excluding the moreserious considerations

Part II is a concise and sensible study guide of Neurologic disorders and treatments thatserves as a review for board examinees, but also as a teaching tool and reference guide formore junior trainees and medical students The case studies presented are typical of thosethat may be encountered during the Vignette portion of the exam, and thus are good toolsfor study

What advice can I provide for the Neurologist about the take the oral board exams? Preparewell, play to your strengths, be considerate of your patients and your examiners, and get agood 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 PathologyDirector, Neurology Residency Training Program

Emory University School of Medicine

Atlanta, Georgia

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

Part 1

4 1 General Information

the night before What little information you will retain

will not offset the effects that the loss of sleep will have

on your performance It is better to go to sleep at your

regular time, making sure that you have taken the

appro-priate steps to wake up on time the next morning In

ad-dition to setting an alarm, arrange to have a wake-up call,

or for someone to wake you up All the knowledge and

preparation will not help you if you get to the exam

burned out, overwhelmed and out of focus

The Day You Arrive

You will get to your hotel the day before the test After

checking in, your next step is to proceed to the

registra-tion area, where you will receive a packet containing all

the instructions on part 2, including a booklet, timetable,

location of the exam, bus schedule, and name of the team

leader who will administer your exam It is absolutely

essential that you familiarize yourself with the hotel’s

lay-out, and the place and time of departure of the buses

Although private transportation is certainly an option, it

is less advisable than the official transportation, which is

very efficient and certain to get to the destination on time

A general orientation will go over the details and

logis-tics Once you have taken care of registration and

orien-tation you will have some time before you have dinner

and retire Spending the evening in the hotel lobby may

not be the best idea Examiners and examinees are

ac-commodated in the same hotel Therefore, it is likely to

see examiners in the hotel lobby They are easily

recog-nizable for their red and white badges It is best to avoid

socialization between examiners and examinees, to avoid

uncomfortable situations and any possible appearance of

impropriety

If you hook up with some colleagues for dinner, it is

better to avoid the topic of the exam as much as possible

Beware of the anxiety-inducing doomsayers and

obses-sional types who may significantly reduce your chances

of getting a good night’s sleep

The Examiners

Structure of the Examining Team

Since you will be evaluated by a team comprised of

sev-eral people, it may be helpful to understand how this team

is formed and which function each team person has The

American Board of Psychiatry and Neurology (ABPN)

has a highly structured higherarchy consisting of eight

teams headed by a Board Director

The team leader works with four senior examiners who

have considerable experience, sit in several exams at a

time, and help settle scoring controversies between the

two examiners, should they arise The director himself

may join the seniors during the evaluation to give the

examiners his attendance card and to assess the ers The exams themselves are administered by two ex-aminers Primary examiners are volunteers who haverequested to be examiners and submitted their qualifica-tions In addition to their qualifications, examiners areselected for geographic criteria, which allows the Board

examin-to save on expenses Examiners are paid a per diem andtheir expenses are reimbursed

Training of Examiners

New examiners and old ones who have not examined fortwo years or more are trained the day before for half aday by experienced examiners and sometimes directors.During such training, examiners are instructed in detailabout specific elements of the exams, minimum require-ments for a passing grade, examples of conditionalscores, and attitude to keep during the exam Tapes andmock exams can also be part of the training Fairness tothe applicant in the examining process and its evaluation

is stressed and examiners are taught to keep a neutralattitude throughout the experience to avoid giving can-didates a false impression about their performance Pit-falls are discussed: lecturing or teaching is discouraged,

as is giving feedback to the candidate

The examiners are also taught not to be hostile, castic, or condescending, and not to dwell on what thecandidate does not know They are directed not to askonly questions pertaining to their area of expertise Fi-nally, they are reminded to be mindful of the anxiety fac-tor during the examination, both on their approach to thecandidate and to the grading

sar-How Examiners Plan for the Session

The two examiners agree in advance on who should takethe lead in asking questions Though often only a silentobserver, the second examiner may ask questions as well.The Board takes great care to assure the highest standard

of fairness during the exam To that end, new examinersare coupled with more seasoned ones the during their firstexperience as examiners Examiners are also given theopportunity of experiencing different partners as they aresystematically rotated in their pairings during the twodays of the exam To further ensure unbiased and fair testconditions, examiners must report to the team leader thenames of candidates they personally know Similarly, in-formation about the candidate’s background and educa-tional institution are purposely kept out of the process,again to avoid any undue influence on the course of theexam and evaluation

In preparation for the exam, examiners spend a fewhours going over the vignettes and discussing signs,symptoms, localization, and differential diagnosis, ensur-