Neurologic Disease: A Modern Pathophysiologic Approach to Diagnosis and Treatment

261 Pages·2016·34.42 MB·English

by  Michael Andrew Meyer (auth.)

Michael Andrew Meyer
Neurologic
Disease
A Modern
Pathophysiologic
Approach to Diagnosis
and Treatment
123
Neurologic Disease

Michael Andrew Meyer
Neurologic Disease
A Modern Pathophysiologic
Approach to Diagnosis
and Treatment
Michael Andrew Meyer, M.D.
Department of Neurology
Guthrie Clinic
Corning , NY , USA
ISBN 978-3-319-39579-1 ISBN 978-3-319-39581-4 (eBook)
DOI 10.1007/978-3-319-39581-4
Library of Congress Control Number: 2016943316
© Springer International Publishing Switzerland 2016
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Pref ace
We all want to know this answer: How does our brain work?
“…I am conscious… but how?” is a question every person asks at some
time…
“… I think, therefore I am” said Descartes in the seventeenth century—but
how does the brain actually think?
H ow does a highly interconnected mass of nerve cells process informa-
tion, and simultaneously is aware of itself and surroundings in the form of
consciousness?
As neurologists, we unfortunately see patients slip away into a world of
unconsciousness from which they may never return, despite our best efforts
to save them. We can defi ne what is lost and how—we know that the absence
of higher cortical functions leaves patients in an irretrievable state of brain
death with only the most primitive brain stem and/or spinal refl exes, but how
exactly does a viable network of interconnected areas of cerebral cortex gen-
erate consciousness and meaningful human existence?
“… I think, therefore I am.” These seventeenth-century words of Descartes
remind us how the brain defi nes who we are. Without proper functioning of
the brain, our very sense of who we are can disappear—without a functioning
brain, there is no quality of life, and we have come to measure life as just that.
For the unfortunate ICU patient who has suffered a prolonged cardiac arrest
and then revived, a pumping heart alone, without the brain being active and
living as well, offers no life value.
This book examines all diseases of the nervous system and explores the
central and peripheral nervous system in health and disease and the rapid
diagnosis and treatment of an illness that affects the patient in profound ways
and can make the difference between having a good long quality of life or
being paralyzed and bedridden with diminished quality of life. In neurologic
emergency cases, it is important to remember that the brain needs oxygen
continuously and any deprivation of the circulation can lead to lifelong dis-
ability. Therefore, the astute physician must be aware of the clues to form a
proper diagnosis and the subsequent need to fi nd and implement therapy as
soon as possible. The fundamentals of neuroscience are emphasized here as
the root of knowledge for clinical neurology—once the basic science is
grasped, the pathophysiology behind clinical neurology becomes readily
apparent.
T his book is also written to inspire the physician who wants to fi nd new
cures for untreatable illnesses—many illnesses in neurology are not treatable
v
vi Preface
at the current time, and an exciting fi eld lies before the inspired neurologist to
fi nd cures to these devastating illnesses. In this regard, the inspired neurolo-
gist is invited to learn as much as possible about the molecular working of the
nervous system, with the hopes that genetic therapies can intervene with the
disease process.
A lthough this book is intended to train the next generation of physicians,
it hopefully will also guide other established physicians in understanding the
complexities of neurological disease as many may not be specialists in neu-
rology. The book is written from the perspective of an initial inquiry into
symptoms and goes forward from a simple set of symptoms into the actual
possibilities and how a diagnosis is linked to a possible treatment plan that
could be formed.
The approach to diagnosis is timeless and involves not only skills acquired
by physicians through years of training but what could arguably be the most
important skill of all, good, basic communication. The doctor and the patient
must communicate effectively: the patient must clearly communicate their
problem to the best of their ability, and the doctor must carefully listen and
ask the right questions. Frequently patients with neurological disease may
simply not be able to give any history. Consider the patient arriving in the
emergency room with a sudden occlusion of the main artery leading to the
left side of the brain where language takes place. In such a case, a left middle
cerebral artery occlusion will leave the patient speechless and powerless on
the right side of the body—history from another person who witnessed the
event is critical in this case to know the time of onset—is this of great impor-
tance when considering intravenous medications to break up the clot (tPA
must be used within 180 min of symptom onset). At the other end of the time
spectrum, the patient may have incurred a slowly progressive aphasia—clues
and details from others are essential in the classifi cation of the problem as
being likely of a degenerative nature. Without a clear and accurate history, the
neurologist may only have a few clues to the underlying problem.
Although the approach to diagnosis in taking a careful history and physi-
cal examination may be timeless and involve skills doctors have employed
for hundreds of years, the technology with which we explore these diseases
has evolved in a dramatic way that is constantly being updated, as is the
ever- expanding breadth of knowledge we have about neurological disease
mechanisms.
Corning, NY Michael Andrew Meyer, M.D.
Contents
1 Introduction to Differential Diagnosis in Neurology ................. 1
1.1 General Principles ................................................................ 1
1.2 Organizational Principles ..................................................... 2
1.2.1 Neurological Diagnosis ......................................... 5
1.3 Systematic Approach to Neurological Problems ................. 6
1.3.1 Diagnosis by Time and Space Factors ................... 8
1.3.2 Diagnosis: Signs and Symptoms ........................... 9
1.3.3 Organizational Overview of the Text .................... 10
1.3.4 Neurologic Disease Is Very Prevalent ................... 11
1.3.5 Data for USA in 2005 ............................................ 11
1.3.6 Neurological Emergencies .................................... 11
References ....................................................................................... 13
2 Cellular and Subcellular Elements of the Nervous System ....... 15
References ....................................................................................... 30
3 Stroke ............................................................................................. 31
3.1 Introduction .......................................................................... 31
3.2 General Facts About Stroke ................................................. 31
3.3 Establishing a Stroke Diagnosis .......................................... 31
3.4 Historical Aspects of Cerebrovascular Disease ................... 33
3.5 Modern Concepts in Cerebrovascular
Anatomy and Disease .......................................................... 33
3.6 Acute Management Issues with ICH ................................... 41
3.7 Cerebral Aneurysms ............................................................. 43
3.7.1 Hypertension-Related Intracranial Hemorrhage ... 45
3.7.2 Intracranial Hemorrhage: Relation
to Thrombocytopenia ............................................ 46
3.7.3 Facts on TIA .......................................................... 53
3.8 Thrombolytic Therapy ......................................................... 55
3.9 Clinical Analysis by Time and Space Methodology ............ 56
3.9.1 Details of the Ninds Stroke Protocol ..................... 58
3.10 Stroke in Young Adults ........................................................ 59
3.11 IV tPA Contraindicated with Anticoagulation ..................... 62
3.11.1 Common Normal Developmental Variations ........ 62
3.12 New Developments in Acute Stroke Care ........................... 63
3.13 Stroke in Special Populations .............................................. 64
vii
viii Contents
3.14 Seasonal Fluctuations in Stroke Mortality ........................... 66
3.15 Circadian Variation in the Timing of Stroke Onset.............. 67
3.15.1 CT Angiography in the Management
of Carotid Disease ................................................. 67
3.15.2 Circle of Willis Is Crucial in Maintaining
Flow in Setting of Unilateral ICA Disease ............ 69
3.16 Functional Recovery After Stroke: New Insights
Using MRI and PET ............................................................ 77
References ....................................................................................... 79
4 Epilepsy .......................................................................................... 83
4.1 Introduction .......................................................................... 83
4.2 Historical Aspects of Epilepsy ............................................. 83
References ....................................................................................... 105
5 Demyelinating Diseases of the Peripheral and Central
Nervous System: Guillain–Barre and Multiple Sclerosis .......... 107
5.1 Introduction .......................................................................... 107
5.2 Guillain–Barre Syndrome or Acute Infl ammatory
Demyelinating Polyneuropathy ........................................... 107
5.3 Multiple Sclerosis ................................................................ 111
5.4 Leukodystrophies and Other Diseases
of the White Matter .............................................................. 120
References ....................................................................................... 125
6 The Hippocampus ......................................................................... 127
6.1 Introduction .......................................................................... 127
6.1.1 Clinical Case Examples ......................................... 138
References ....................................................................................... 140
7 Alzheimer’s Disease ...................................................................... 143
7.1 Clinical and Radiographic Aspects
of Alzheimer’s Disease ........................................................ 152
References ....................................................................................... 158
8 Neurodegenerative Diseases ......................................................... 161
References ....................................................................................... 174
9 Diseases of Skeletal Muscle .......................................................... 177
9.1 Introduction .......................................................................... 177
References ....................................................................................... 186
10 Diseases of the Spinal Cord .......................................................... 187
References ....................................................................................... 194
11 The Cerebellum ............................................................................. 197
11.1 Introduction .......................................................................... 197
References ....................................................................................... 205
12 The Visual Cortex ......................................................................... 207
12.1 Migraine and the Occipital Cortex ...................................... 214
References ....................................................................................... 218
Contents ix
13 Infections of the Nervous System ................................................. 219
References ....................................................................................... 228
14 Neoplastic Diseases ........................................................................ 229
14.1 Radiation Necrosis ............................................................... 235
14.2 Genetic Defects and Primary Neoplasms ............................ 235
References ....................................................................................... 243
15 Traumatic and Toxic Effects on the Nervous System ................ 245
15.1 Toxins and the Nervous System: Alcohol, Mercury,
Lead, Medications ................................................................ 247
References ....................................................................................... 251
16 Conclusions .................................................................................... 253
16.1 Introduction .......................................................................... 253
References ....................................................................................... 254
Index ....................................................................................................... 255