The Integrated Nervous System: A Systematic Diagnostic Case-Based Approach, Second Edition

363 Pages·2017·73.16 MB·English

by  Walter J. Hendelman

SECOND EDITION
THE INTEGRATED
NERVOUS SYSTEM
A SYSTEMATIC DIAGNOSTIC
CASE- BASED APPROACH
http://taylorandfrancis.com
SECOND EDITION
THE INTEGRATED
NERVOUS SYSTEM
A SYSTEMATIC DIAGNOSTIC
CASE-BASED APPROACH
WALTER J. HENDELMAN, MD, CM
University of Ottawa
Ontario, Canada
PETER HUMPHREYS, MD, FRCP(C)
Children’s Hospital of Eastern Ontario
Ottawa, Canada
CHRISTOPHER R. SKINNER, MD, FRCP(C)
University of Ottawa
Ontario, Canada
CRC Press
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CONTENTS
Preface................................................................................................................................................................................vi
Organization of the Book and Integration with the Web Site ..........................................................................................viii
Acknowledgements ..............................................................................................................................................................x
Authors ...............................................................................................................................................................................xi
Introduction ......................................................................................................................................................................xiii
Section 1 The Basics of Neurological Problem Solving
1 Synopsis of the Nervous System ....................................................................................................................................3
2 Neurological Examination ..........................................................................................................................................24
3 Clinical Problem Solving ............................................................................................................................................44
Section 2 Applying the Basics to Clinical Cases
4 Fifi ................................................................................................................................................................................72
5 Cletus ...........................................................................................................................................................................92
6 Ernesto .......................................................................................................................................................................111
7 Bernie .........................................................................................................................................................................131
8 Etienne .......................................................................................................................................................................157
9 Cheryl .........................................................................................................................................................................178
10 Patty ........................................................................................................................................................................200
11 Didi ..........................................................................................................................................................................223
12 Armand ....................................................................................................................................................................243
13 Mickey .....................................................................................................................................................................261
Section 3 Supplementary Considerations: Rehabilitation and Ethics
14 Neurorehabilitation ..................................................................................................................................................285
15 Ethics and Neurology ...............................................................................................................................................296
Glossary ...........................................................................................................................................................................307
Annotated Bibliography ...................................................................................................................................................319
Answers to Chapter Questions .........................................................................................................................................327
Index ................................................................................................................................................................................336
v
PREFACE
You might be wondering why this textbook about neurology and neurologic problem-solving, using a case-based
approach, is called the INTEGRATED Nervous System.
If you have ever attended an orchestral concert playing classical music, perhaps a symphony by Beethoven or
Brahms or Stravinsky, you would have heard, and perhaps seen, the marvelous synchronization of the various musical
instruments. It is incredible how the music is created as each member of the group starts or stops playing according to
the musical score, resulting in the sound that we hear. Perhaps, you are fortunate enough to actually play in an orches-
tra or band and are part of that experience. Next, you might think about yourself, the listener. After all, your brain
receives just sound waves via a complex series of connections. Just where in the brain is ‘the music centre’? Finally,
you might contemplate the genius of the composers whose brains produced this complex combination of sounds, the
music we hear.
So it is with our brains. No part of the brain acts alone. All its component parts participate in a wondrous synchro-
nization of thought/feeling/sensation/motor activity that characterizes our daily behaviour. You almost need to ‘dissect’
behaviour to recognize the contribution of the component parts. The mature nervous system integrates information from
different sensory modalities, amalgamates experiences and information from the past and correlates it with the present
context, resulting in responses (verbal and/or motor) that are (hopefully) appropriate for the situation.
The integration that we the authors are striving to achieve with this book is the approach that you as a non-neurologist
will need in order to weave together your knowledge of neuroanatomy with the art and science of clinical neurology.
We, the coauthors of this book, include a neuroanatomist (WH), an adult neurologist (CS) and a pediatric neurologist
(PH), all of whom have been involved in teaching a combined neuroscience and neurology course to second-year medi-
cal students. This is a five-week course, case-based, with lectures, organized on the principle of adult learning in small
groups, with expert tutors (almost all practicing neurologists). Clinical disease entities (e.g. multiple sclerosis) are used
as the ‘problems’ in the course.
One of the real challenges in a course that includes both neuroscience and neurology is the enormous scope of the
subject matter; this leads to significant information overload when the process is compressed into a short period of block
learning. A by-product of this compressed learning process has been the observation by both clinicians that most of their
students – members of their tutor groups who ‘knew’ their material and passed the course, often with a good grade –
when returning as clinical clerks or while taking a neurology elective, were unable to use the knowledge they once had
mastered to solve clinical problems at the bedside.
This lack of integration of basic science and clinical information has been postulated as the basis of a syndrome
called neurophobia, which apparently can affect one of every two medical students. This lack of ability to reason
through clinical problems results in anxiety and dislike of the subject matter and, eventually, negative sentiments
about and even fear of neurology (Jozefowicz, 1994; Fantaneanu et al., 2014). This book has been created to integrate
these two worlds and to overcome this pedagogical deficit, using a problem-based approach with clinical disease enti-
ties. Our objective is to bridge the gap between the book and the bedside, in other words between the classroom and
the clinic.
The second edition of this book has been revised and edited keeping you, the learner, in mind. We have rewritten and
updated the text, as well as many of the tables. To help you understand the neurological disorders that you will encoun-
ter in this text and the challenges that patients and relatives face when such disorders occur, we have created a fictional
‘person’ for each of the clinical chapters as well as for the cases that are found on the accompanying Web site, called
‘e-cases’, with each of these chapters. The most significant addition is the abbreviated presentation of the history and
neurologic examination of all the e-cases (now numbering over 50) within the text at the end of each of the clinical chap-
ters; along with this, we have created ‘maps’ – a visual representation of the clinical motor, sensory and reflex findings.
We hope that this inclusion will encourage the student to go to the Web site where each of the cases is presented in detail
and where the ‘expert’ discusses the reasoning for the localization and etiological diagnosis.
We believe that this book and the associated Web site will be of practical value to all the professionals who deal with
people who have neurological conditions, not only medical students and residents. This includes physiatrists (rehabilita-
tion medicine specialists), physiotherapists, occupational therapists and speech therapists, and nurses who specialize in
the care of neurological patients. We think that this text will also be of value for family physicians and specialists in
vi
Preface vii
internal medicine and pediatrics, all of whom must differentiate between organic pathology of the nervous system and
other conditions.
Dr. Walter J. Hendelman
Dr. Peter Humphreys
Dr. Christopher Skinner
Faculty of Medicine
University of Ottawa
Ottawa, Canada
REFERENCES
Fantaneanu, T.A. et al. Neurophobia inception. Can J Neurol Sci 2014. 41: 421–429.
Jozefowicz, R.F. Neurophobia: The fear of neurology among medical students. Arch Neurol 1994. 51: 328–329.
ORGANIZATION OF THE BOOK
AND INTEGRATION WITH THE WEB SITE
The aim of this book is to enable you, the learner, to use your knowledge of the nervous system combined with a neuro-
logically based, problem-solving clinical reasoning approach to neurology to help in the diagnosis and treatment, in the
broadest sense, of those who suffer from a neurological disease or injury. We hope that this approach meets with success,
insofar as it leads to an improvement in the diagnosis and care of persons afflicted by neurological problems.
To help you understand the neurological disorders that you will encounter in this text and the challenges that patients
and relatives face when such disorders appear, we have created fictional persons with names. We meet the first of these
people in the Introduction, which lays out the complexity of the nervous system and its capacity to multitask. The intro-
duction exposes the reader to neuroanatomical pathways, which, by the end of the book, you should be quite familiar with
in terms of both their function and their importance in neurology.
The first section is called ‘The Basics of Neurological Problem Solving’. Chapter 1 is a review of clinically relevant
neuroanatomy, enough to set the stage in terms of the basic knowledge of the nervous system needed for this book. The
second chapter is devoted to the clinical neurological examination and the integration of the information garnered in
terms of the normal functioning nervous system, for example, the assessment of reflex activity. Chapter 3 introduces the
student to neurological clinical reasoning for the purpose of localizing the disease or lesion within the nervous system
and determining the aetiology, the pathophysiological mechanism of disease. Patients with neurological problems present
great challenges to their physicians (as well as to their relatives and caregivers) to accurately and completely gather the
information required to make a working diagnosis and plan of investigation based on a single clinic visit. The approach
used in this chapter and its accompanying worksheets is designed to provide students and non-neurologist clinicians with
practical guidelines and tools with respect to diagnosis for the full range of neurological problems seen by a neurological
generalist. This approach is applied throughout the book.
The next section is called ‘Applying the Basics to Clinical Cases’. The succeeding 10 chapters deal with important
specific clinical diseases or syndromes that have afflicted our fictional individuals, each with the focus on a different
component of the nervous system, starting with the peripheral nervous system. The following chapters ‘ascend’ – spinal
cord, brainstem, all the way to the cortex, with the last chapter in this section devoted to behavioural issues. For each of
these cases, the history is presented, followed by the findings of the neurological examination. Additional neuroanatomi-
cal, neurophysiological and neurochemical information is added, where required, as it pertains to the clinical condition
discussed in the chapter. Notwithstanding the information given, it is suggested that students review their knowledge
of neuroanatomy, neurophysiology, neuropharmacology and neuropathology, using other resource books (given in the
suggested readings and references sections at the end of each chapter and in the Annotated Bibliography at the end of
the book). In each of these chapters, there is an application of the process of neurological reasoning to narrow the pos-
sibilities of where the lesion is located. This is followed by a systematic analysis in order to determine what disease (or
diseases) should be considered. Relevant selected investigations are then presented and the results discussed. Finally, the
diagnosis is made, with its prognosis, and an outline of the appropriate management is given, ending with the outcome
of the case.
The text illustrations have been prepared with an emphasis on the functioning nervous system. In addition to neuro-
anatomical drawings related to the cases and tables with relevant clinical data, there are figures illustrating neuro-
physiological concepts, clinical findings (such as radiographic images and electroencephalograms) and microscopic
neuropathological images. Again, the information is described in the context of the disease presented in that chapter. The
glossary of terms also emphasizes clinical terminology.
The third section, named ‘Supplementary Considerations’, presents additional insights written by guest authors,
Dr. Anna McCormick, a physiatrist (a specialist in physical medicine rehabilitation), and Dr. Robert Nelson, a senior neu-
rologist with expertise in ethics. Both discuss other important dimensions of neurological problems: rehabilitation and
ethics. Currently, rehabilitation has much to offer for those afflicted by disease or injury of the nervous system; there is
now a certain air of hopefulness that there can be recovery of function following an insult to the nervous system, in adults
as well as in children. The ethical principles and reasoning on the basis of which decisions (sometimes quite unique) are
taken in neurological cases are presented in the context of an inherited disease of the nervous system.
There is a Web site associated with this textbook: http://www.integratednervoussystem.com.
viii
Organization of the Book and Integration with the Web Site ix
This Web site contains the worksheets that have been developed to apply the clinical reasoning approach to neuro-
logical problem solving and the e-cases that are now part of each of the clinical chapters. The e-cases enlarge the scope of
the book by adding additional commonly seen neurological diseases for each level of the nervous system. These are pre-
sented in a more sequential fashion, although once having learned the analytic approach, it is hoped (expected) that you,
the learner, will work through each clinical case on your own, using the worksheets, before reading the case evolution,
investigations and resolution. It is highly recommended that you, the non-neurologist student/learner, apply this approach
when confronted with a neurological patient. The authors want you to learn how to think like a neurologist. In addition,
the Web site has all the illustrations found in the book, with animation added to assist in the understanding of some reflex
circuits and various pathways. It also includes the glossary. There is also a learning module to assist you, as a non-expert,
in understanding neuroimaging, how the various modes of computed tomography and magnetic resonance imaging assist
in localizing a lesion and defining the likely etiology. The Web site will also be utilized to provide updates on the cases
presented as well as new cases, so it may be wise to check it periodically.
We sincerely hope that our system will work for you and wish you every success in the diagnosis and management
of your neurological patients.