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Bradley's Neurology in Clinical Practice E-Book

Bradley's Neurology in Clinical Practice E-Book

Robert B. Daroff | Joseph Jankovic | John C Mazziotta | Scott L Pomeroy

(2015)

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Book Details

Abstract

Comprehensive, easy to read, and clinically relevant, Bradley’s Neurology in Clinical Practice provides the most up-to-date information presented by a veritable "Who's Who" of clinical neuroscience. Its unique organization allows users to access content both by presenting symptom/sign and by specific disease entities—mirroring the way neurologists practice. A practical, straightforward style; templated organization; evidence-based references; and robust interactive content combine to make this an ideal, dynamic resource for both practicing neurologists and trainees.

  • Authoritative, up-to-date guidance from Drs. Daroff, Jankovic, Mazziotta, and Pomeroy along with more than 150 expert contributors equips you to effectively diagnose and manage the full range of neurological disorders.
  • Easy searches through an intuitive organization by both symptom and grouping of diseases mirrors the way you practice.
  • The latest advances in clinical neurogenetics, brain perfusion techniques for cerebrovascular disease, the relationship between neurotrauma and neurodegenerative disease, management strategies for levodopa-related complications in movement disorders, progressive neuropsychiatric disorders arising from autoimmune encephalitis, and more keep you at the forefront of your field.
  • Reorganized table of contents which includes new chapters on: Brain Death, Vegetative, and Minimally Conscious States; Deep Brain Stimulation; Sexual Dysfunction in Degenerative and Spinal Cord Disorders; Sports and Performance Concussion; Effects of Drug Abuse on the Nervous System; and Mechanisms of Neurodegenerative Disorders.

Table of Contents

Section Title Page Action Price
9780323339162v1_WEB 1
Front Cover 1
Half title page 2
Dedication 3
Bradley’s Neurology in Clinical Practice 4
Copyright Page 5
Table Of Contents 6
Foreword 10
Preface 11
List of Contributors 12
Video Table of Contents 19
I Common Neurological Problems 24
1 Diagnosis of Neurological Disease 24
Chapter Outline 24
Neurological Interview 24
Chief Complaint 25
History of Present Illness 25
Review of Patient-Specific Information 26
Review of Systems 26
History of Previous Illnesses 26
Family History 27
Social History 27
Examination 27
Neurological Examination 27
General Physical Examination 28
Assessment of the Cause of the Patient’s Symptoms 28
Anatomical Localization 28
Differential Diagnosis 29
Laboratory Investigations 29
Management of Neurological Disorders 30
Experienced Neurologist’s Approach to the Diagnosis of Common Neurological Problems 30
2 Episodic Impairment of Consciousness 31
Chapter Outline 31
Syncope 31
History and Physical Examination 31
Causes of Syncope 33
Cardiac Arrhythmias 33
Atrioventricular Block 33
Sinoatrial Block 33
Paroxysmal Tachycardia 33
Reflex Cardiac Arrhythmias 34
Decreased Cardiac Output 34
Hypovolemia 34
Hypotension 34
Cerebrovascular Ischemia 35
Metabolic Disorders 35
Miscellaneous Causes of Syncope 35
Investigations of Patients with Syncope 36
Seizures 36
History and Physical Examination 36
Absence Seizures 37
Tonic-Clonic Seizures 37
Complex Partial Seizures 37
Investigations of Seizures 37
Psychogenic (Nonepileptic) Seizures 38
Miscellaneous Causes of Altered Consciousness 38
References 39
References 40
3 Falls and Drop Attacks 41
Chapter Outline 41
Drop Attacks with Loss of Consciousness 41
Syncope 41
Seizures 42
Drop Attacks without Loss of Consciousness 42
Transient Ischemic Attacks 42
Vertebrobasilar Insufficiency 42
Anterior Cerebral Artery Ischemia 42
Third Ventricular and Posterior Fossa Abnormalities 42
Otolith Crisis 43
Falls 43
Neuromuscular Disorders and Myelopathy 43
Stroke 43
Other Cerebral or Cerebellar Disorders 43
Cryptogenic Falls in the Middle-Aged 43
Aging, Neurodegeneration, and the Neural Substrate of Gait and Balance 44
Basal Ganglia Disorders 44
Parkinson Disease. 44
Progressive Supranuclear Palsy and other Parkinsonian Syndromes. 44
Aged State 44
Summary 46
References 46
References 47
4 Delirium 49
Chapter Outline 49
Clinical Characteristics 49
Acute Onset with Fluctuating Course 49
Cognitive and Related Abnormalities 50
Attentional Deficits 50
Confusion or Disorganized Thinking 51
Altered Level of Consciousness 51
Perceptual Disturbances 51
Disturbed Sleep/Wake Cycle 51
Altered Psychomotor Activity 51
Disorientation and Memory Impairment 52
Other Cognitive Deficits 52
Behavioral and Emotional Abnormalities 52
Pathophysiology 52
Diagnosis 53
Predisposing and Precipitating Factors 53
Mental Status Examination 54
Diagnostic Scales and Criteria 54
Physical Examination 55
Laboratory Tests 55
Differential Diagnosis 55
Common Causes of Delirium 55
Metabolic Disturbances 55
Drugs 56
Infections 56
Strokes 56
Epilepsy 57
Postoperative Causes 57
Other Neurological Causes 57
Miscellaneous Causes 57
Special Problems in Differential Diagnosis 57
Prevention and Management 58
Prognosis 59
References 59
References 60
5 Stupor and Coma 62
Chapter Outline 62
Definitions 62
Conditions That May Mimic Coma 62
Approach to the Patient in Coma 63
Rapid Initial Examination and Emergency Therapy 63
Common Presentations 65
History 65
General Examination 66
Blood Pressure Evaluation 66
Hypotension. 66
Hypertension. 66
Heart Rate 66
Respiration 66
Temperature 66
General Appearance 66
Head and Neck Examination 67
Eye Examination 67
Otoscopic Examination 67
Oral Examination 67
Integument Examination 67
Examination of Lymph Nodes 67
Cardiac Examination 67
Abdominal Examination 67
Miscellaneous Examinations 67
Neurological Examination 68
State of Consciousness 68
Respiration 69
Pupil Size and Reactivity 70
Ocular Motility 71
Abnormalities in Resting Position. 72
Eye Deviation. 72
Spontaneous Eye Movements. 72
Reflex Ocular Movements. 72
Motor System 73
Coma and Brain Herniation 74
Differential Diagnosis 75
Differentiating Toxic-Metabolic Coma from Structural Coma 75
Differentiating Psychiatric Coma and Pseudocoma from Metabolic or Structural Coma 75
Helpful Laboratory Studies 76
Other Useful Studies 76
Electrocardiography 76
Neuroradiological Imaging 76
Electroencephalography 76
Evoked Potentials 77
Intracranial Pressure Monitoring 77
Prognosis 78
Nontraumatic Coma 78
Traumatic Coma 78
Persistent Vegetative State 78
Brain Death 78
References 78
References 79
6 Brain Death, Vegetative State, and Minimally Conscious States 80
Chapter Outline 80
Brain Death 80
Prolonged Disorders of Consciousness 83
Vegetative State 83
Minimally Conscious State 83
Imaging in Disorders of Consciousness 84
Decision-making and bioethics 85
References 85
References 86
7 Intellectual and Memory Impairments 87
Chapter Outline 87
Neural Basis of Cognition 87
Cerebral Cortex 87
Consciousness 88
Memory 90
Forms and Stages of Declarative Memory 90
Formation and Retrieval of Episodic Memories 91
Amnestic Syndrome 91
Syndromes of Partial Memory Loss 93
Transient Amnesia 93
Other Types of Memory (Nondeclarative or Implicit Memory) 93
Bedside Tests of Memory and Cognitive Function 94
References 95
References 96
8 Global Developmental Delay and Regression 98
Chapter Outline 98
Typical and Atypical Development 98
Child Development Concepts 98
Global Developmental Delay 98
Developmental History 98
Neurological and Other Medical History 98
Physical Examination 99
Diagnostic Testing 99
Genetic Testing 100
Neuroimaging 101
Other Tests 101
Management 103
Prognosis 103
Recurrence Risk 103
Regression 104
References 104
References 105
9 Behavior and Personality Disturbances 106
Chapter Outline 106
Frontosubcortical Circuitry 106
Assessing Behavior and Personality Disturbances in Patients with Cerebral Dysfunction 108
Classification of Neurobehavioral Symptoms and Disorders 109
Assessment of Depression 109
Assessment of Other Behavioral and Personality Disturbances 109
Behavior and Personality Disturbances Associated with Cerebral Dysfunction 110
Alzheimer Disease 110
Depression 111
Apathy 111
Aggression 111
Psychosis 111
Frontotemporal Dementia 112
Behavioral Disruption 112
Anosognosia 112
Relationship to Anatomy 113
Vascular Dementia 113
Depression 113
Additional Behavioral and Psychiatric Disorders 113
Parkinson Disease 113
Depression 114
Psychosis 114
Apathy 114
Impulse Control Disorders 114
Neuropsychiatric Effects of Deep Brain Stimulation 114
Dementia with Lewy Bodies 115
Psychosis 115
Huntington Disease 115
Depression 115
Suicide 120
Psychosis 120
Obsessive-Compulsive Traits 120
Aggression 120
Apathy 120
Tourette Syndrome 120
Multiple Sclerosis 120
Depression 120
Anxiety 121
Euphoria 121
Pseudobulbar Affect 121
Amyotrophic Lateral Sclerosis 122
Depression 122
Pseudobulbar Affect 122
Personality Change 122
Epilepsy 123
Depression 125
Anxiety 125
Psychosis 125
Aggression 126
Stroke 126
Depression 126
Pseudobulbar Affect 127
Aggression 127
Psychosis 127
Traumatic Brain Injury 127
Anosognosia 127
Depression 127
Anxiety 128
Apathy 128
Personality Change 128
References 128
References 129
10 Depression and Psychosis in Neurological Practice 133
Chapter Outline 133
Principles of Differential Diagnosis 133
Principles of Neuropsychiatric Evaluation 135
Cognitive-Affective-Behavioral Brain Behavior Relationships 136
Cortical Networks 136
Frontosubcortical Networks 138
Cerebrocerebellar Networks 139
Biology of Psychosis 139
Biology of Depression 140
Clinical Symptoms and Signs Suggesting Neurological Disease 141
Psychiatric Manifestations of Neurological Disease 141
Stroke and Cerebral Vascular Disease 141
Infectious 145
Human Immunodeficiency Virus 145
Creutzfeldt–Jakob Disease 145
Neurosyphilis 146
Metabolic and Toxic 146
Thyroid Disease 146
Wilson Disease 148
Vitamin B12 and Folic Acid Deficiency 148
Porphyrias 148
Drug Abuse 149
Systemic Lupus Erythematosus 149
Multiple Sclerosis 149
Neoplastic 151
Degenerative 151
Alzheimer Disease and Mild Cognitive Impairment 152
Frontotemporal Dementia 152
Idiopathic Parkinson Disease 153
Dementia with Lewy Bodies 153
Huntington Disease 153
Epilepsy 153
Traumatic Brain Injury 155
Depression-Related Cognitive Impairment 156
Delirium 156
Catatonia 157
Treatment Modalities 157
Electroconvulsive Therapy 160
Vagus Nerve Stimulation 160
Repetitive Transcranial Magnetic Stimulation 160
Psychiatric Neurosurgery or Psychosurgery 160
Treatment Principles 161
References 161
References 162
11 Limb Apraxias and Related Disorders 166
Chapter Outline 166
Historical Perspective 166
A Model for Praxis 167
Classification of Limb Apraxias 167
Ideomotor Apraxia, Parietal Variant 168
Ideomotor Apraxia, Disconnection Variant 168
Dissociation Apraxia 168
Ideational Apraxia 168
Conceptual Apraxia 168
Limb-Kinetic Apraxia 168
Callosal Apraxia 169
Testing for Limb Apraxias 169
Testing for Ideomotor Apraxia, Parietal and Disconnection Variants 170
Testing for Dissociation Apraxia 170
Testing for Ideational Apraxia 171
Testing for Conceptual Apraxia 171
Testing for Limb-Kinetic Apraxia 171
Testing for Callosal Apraxia 171
Pathophysiology of Limb Apraxias 171
Rehabilitation for Limb Apraxias 171
Related Disorders 172
Summary 172
References 172
References 173
12 Agnosias 174
Chapter Outline 174
Visual Agnosias 174
Cortical Visual Disturbances 174
Cortical Visual Distortions 175
Balint Syndrome and Simultanagnosia 175
Visual Object Agnosia 175
Apperceptive Visual Agnosia 175
Associative Visual Agnosia 176
Optic Aphasia 176
Prosopagnosia 177
Klüver–Bucy Syndrome 177
Auditory Agnosias 177
Cortical Deafness 177
Pure Word Deafness 178
Auditory Nonverbal Agnosia 178
Phonagnosia 178
Amusia 178
Tactile Agnosias 179
Tactile Aphasia 179
Summary 179
Acknowledgment 179
References 179
References 180
13 Aphasia and Aphasic Syndromes 181
Chapter Outline 181
Symptoms and Differential Diagnosis of Disordered Language 183
Bedside Language Examination 183
Differential Diagnosis of Aphasic Syndromes 184
Broca Aphasia 184
Aphemia 185
Wernicke Aphasia 185
Pure Word Deafness 186
Global Aphasia 186
Conduction Aphasia 186
Anomic Aphasia 187
Transcortical Aphasias 188
Subcortical Aphasias 189
Pure Alexia without Agraphia 190
Alexia with Agraphia 191
Aphasic Alexia 191
Agraphia 192
Language in Right Hemisphere Disorders 192
Language in Dementing Diseases 193
Investigation of the Aphasic Patient 194
Clinical Tests 194
Differential Diagnosis 194
Recovery and Rehabilitation of Aphasia 196
References 197
References 198
14 Dysarthria and Apraxia of Speech 200
Chapter Outline 200
Motor Speech Disorders 200
Dysarthrias 200
Apraxia of Speech 201
Oral or Buccolingual Apraxia 202
Aphemia 202
The “Foreign Accent Syndrome” 202
Acquired Stuttering 202
Opercular Syndrome 202
References 202
References 203
15 Neurogenic Dysphagia 204
Chapter Outline 204
Normal Swallowing 204
Neurophysiology of Swallowing 204
Mechanical Dysphagia 205
Neuromuscular Dysphagia 205
Oculopharyngeal Muscular Dystrophy 206
Myotonic Dystrophy 206
Other Muscular Dystrophies 206
Inflammatory Myopathies 206
Mitochondrial Disorders 207
Myasthenia Gravis 207
Neurogenic Dysphagia 207
Stroke 208
Multiple Sclerosis 209
Parkinson Disease 210
Other Basal Ganglia Disorders 210
Amyotrophic Lateral Sclerosis 211
Cranial Neuropathies 211
Brainstem Processes 211
Cervical Spinal Cord Injury 211
Other Processes 212
Evaluation of Dysphagia 212
References 213
References 214
16 Visual Loss 216
Chapter Outline 216
Pattern of Visual Loss 216
Central Visual Loss 216
Peripheral Visual Loss 216
Temporal Profile of Visual Loss 216
Sudden-Onset Visual Loss 216
Transient Monocular Visual Loss 216
Amaurosis Fugax. 216
Retinal Artery Vasospasm. 216
Angle-Closure Glaucoma. 216
Visual Loss in Bright Light. 217
Uhthoff Phenomenon. 217
Transient Visual Obscurations. 217
Other Causes of Transient Visual Loss. 217
Transient Binocular Visual Loss 217
Sudden Monocular Visual Loss without Progression 217
Sudden Binocular Visual Loss without Progression 219
Sudden Visual Loss with Progression 219
Progressive Visual Loss 219
References 220
References 221
17 Abnormalities of the Optic Nerve and Retina 222
Chapter Outline 222
Optic Nerve Anatomy and Physiology 222
The Swollen Optic Disc 222
Unilateral Optic Disc Swelling 222
Optic Neuritis 224
Ischemic Optic Neuropathy 225
Other Causes 226
Bilateral Optic Disc Swelling 229
Papilledema 229
Malignant Hypertension 231
Diabetic Papillopathy 231
Other Causes 231
Pseudopapilledema 232
Optic Disc Drusen 232
Optic Neuropathies with Normal-Appearing Optic Discs 233
Unilateral Presentations 233
Bilateral Presentations 233
Optic Neuropathies with Optic Atrophy 233
Congenital Optic Disc Anomalies 234
Tilted Optic Disc 234
Optic Nerve Dysplasia 234
Retinal Disorders 235
Retinal Arterial Disease 235
Branch Retinal Artery Occlusions and Encephalopathy (Susac Syndrome) 236
Ocular Ischemic Syndrome 236
Retinal Vein Occlusion 236
Retinal Degenerations 236
Phakomatoses 237
References 237
References 238
18 Pupillary and Eyelid Abnormalities 239
Chapter Outline 239
Pupillary Abnormalities 239
Pupil Anatomy and Neural Control 239
Normal Pupil Phenomena 239
Afferent Pathological Conditions of the Pupils 239
Efferent Pathological Conditions of the Pupils 240
Clinical Presentation and Examination 240
Anisocoria Greater in the Light 240
Postganglionic Parasympathetic Dysfunction—Tonic Pupil. 240
Preganglionic Parasympathetic Dysfunction—Oculomotor Palsy. 242
Iris Sphincter Injury and Ischemia. 243
Pharmacological Mydriasis. 243
Anisocoria Greater in the Dark 244
Horner Syndrome. 244
Anterior Uveitis (Iris Inflammation). 245
Episodic Anisocoria 246
Pupillary Light-Near Dissociation 246
Eyelid Abnormalities 246
Eyelid Anatomy and Neural Control 246
Pathological Conditions of the Eyelids 246
Clinical Presentation and Examination 246
Pathologically Widened Palpebral Fissures 247
Pathologically Narrowed Palpebral Fissures 248
Dynamic Eyelid Abnormalities 248
References 249
References 250
19 Disturbances of Smell and Taste 251
Chapter Outline 251
Anatomy and Physiology 251
Olfaction 251
Gustation 252
Chemosensory Testing 253
Disorders of Olfaction 254
Disorders of Taste 255
Clinical Evaluation of Taste and Smell 256
Treatment and Management 256
References 257
References 258
20 Cranial and Facial Pain 259
Chapter Outline 259
History 259
Types of Headaches 259
Onset of Headaches 259
Frequency and Periodicity of Episodic Headaches 260
Temporal Profile 260
Time of Day and Precipitating Factors 261
Location 261
Quality and Severity 261
Premonitory Symptoms, Aura, and Accompanying Symptoms 261
Aggravating and Mitigating Factors 262
Family History of Headaches 262
Prior Evaluation 262
Prior Treatment 262
Disability 262
Patient Concerns and Reasons for Seeking Help 262
Other Medical or Neurological Problems 262
Examination 262
Diagnostic Testing 263
Neuroimaging and Other Imaging Studies 263
Computed Tomography and Magnetic Resonance Imaging 263
Plain Radiographs and Other Imaging of the Skull, Sinuses, and Cervical Spine 264
Temporomandibular Joint/Dental Imaging Studies 264
Cerebral Angiography 264
Myelography with Computed Tomography and Radioisotope Studies for Detection of Cerebrospinal Fluid Leaks 264
Cerebrospinal Fluid Tests 264
Electrophysiological Testing 264
General Medical Tests 264
Special Examinations and Consultations 265
Further Observation 266
References 266
References 267
21 Brainstem Syndromes 268
Chapter Outline 268
Ocular Motor Syndromes 268
Combined Vertical Gaze Ophthalmoplegia 268
Upgaze Paresis (Dorsal Midbrain or Parinaud Syndrome) 269
Downgaze Paresis 269
Internuclear Ophthalmoplegia 270
Horizontal Gaze Paresis 270
Global Paralysis of Gaze 270
One-and-a-Half Syndrome 271
Syndromes Involving Ocular Motor Nuclei 271
Third Cranial Nerve Nucleus 271
Sixth Cranial Nerve Nucleus 271
Other Brainstem and Associated Syndromes 271
Diencephalic Syndrome (Russell Syndrome) 271
Thalamic Syndrome 272
Tectal Deafness 272
Foramen Magnum Syndrome 272
Syringobulbia 272
Brainstem Ischemic Stroke Syndromes 273
Thalamic Stroke Syndromes 273
Midbrain Stroke Syndromes 274
Pontine Stroke Syndromes 275
Medullary Stroke Syndromes 275
References 279
References 280
22 Ataxic and Cerebellar Disorders 281
Chapter Outline 281
Symptoms and Signs of Ataxic Disorders 281
Symptoms in Patients with Ataxia 281
Gait Disturbances 281
Limb Ataxia 281
Truncal Ataxia 281
Dysarthria and Bulbar Symptoms 281
Visual Symptoms 281
Symptoms in Sensory Ataxia 281
Neurological Signs in Patients with Cerebellar Ataxia 281
Stance and Gait 282
Limb Incoordination 282
Dysmetria. 282
Kinetic (Intention) Tremor. 282
Action Tremor. 282
Other Types of Tremor. 282
Dysdiadochokinesia. 282
Abnormalities of Muscle Tone and Strength 282
Oculomotor Disturbances 282
Disorders of Pursuit. 283
Disorders of Saccades. 283
Other Saccadic Intrusions. 283
Nystagmus. 283
Vestibulo-Ocular Reflex. 283
Speech and Bulbar Function 283
Cognitive-Affective Features 283
Neurological Signs in Patients with Sensory Ataxia 283
Diagnostic Approach to Ataxia 283
References 286
References 287
23 Diagnosis and Assessment of Parkinson Disease and Other Movement Disorders 288
Chapter Outline 288
Parkinsonism 289
Motor Abnormalities 290
Cognitive, Autonomic, and Sensory Abnormalities 291
Onset and Course 292
Examination and Clinical Signs 292
Differential Diagnosis 293
Tremor 295
Common Symptoms 295
Rest Tremor 295
Postural Tremor 296
Other Types of Tremor 296
Other Clues in the History 297
Examination 297
Dystonia 300
Common Symptoms 300
Examination 302
Chorea 302
Common Symptoms 302
Other Clues in the History 303
Examination 304
Tardive Dyskinesia 304
Ballism 305
Tics 305
Common Symptoms 306
Examination 307
Myoclonus 307
Common Symptoms 307
Examination 308
Miscellaneous Movement Disorders 309
Investigation of Movement Disorders 310
References 314
References 315
24 Gait Disorders 317
Chapter Outline 317
Physiological and Biomechanical Aspects of Gait 317
Anatomical Aspects of Gait 317
History: Common Symptoms and Associations 318
Weakness 318
Slowness and Stiffness 318
Imbalance 318
Falls 319
Sensory Symptoms and Pain 319
Urinary Incontinence 319
Cognitive Changes 319
Examination of Posture and Walking 319
Arising from Sitting 319
Stance 319
Trunk Posture 320
Postural Responses 321
Walking 321
Initiation of Gait 321
Stepping 321
Turning 321
More Challenging Tests of Walking 321
Associated and Synergistic Limb Movements While Walking 321
Motor and Sensory Examination 321
Discrepancies on Examination of Gait 322
Classification of Gait Patterns 322
Lower Level Gait Disorders 322
Myopathic Weakness and Gait 322
Neurogenic Weakness and Gait 322
Sensory Ataxia 322
Vestibular Imbalance, Vertigo, and Gait 322
Middle-Level Gait Disorders 323
Spastic Gait 323
Cerebellar Ataxia 323
Spastic Ataxia 323
Hypokinetic (Parkinsonian) Gait 324
Choreic Gait 324
Dystonic Gait 324
Mixed Movement Disorders and Gait 324
Tremor of the Trunk and Legs 325
Action Myoclonus 325
Higher Level Gait Disorders 325
Hypokinetic Higher Level and Freezing Gait Patterns 325
Elderly Gait Patterns, Cautious Gaits, and Fear of Falling 327
Perceptions of Instability and Illusions of Movement 327
Reckless Gait Patterns 327
Hysterical and Psychogenic Gait Disorders 327
Musculoskeletal Disorders and Antalgic Gait 328
Skeletal Deformity and Joint Disease 328
Painful (Antalgic) Gaits 328
References 328
References 329
25 Hemiplegia and Monoplegia 330
Chapter Outline 330
Anatomy and Physiology 330
Motor System Anatomy 330
Pyramidal Tract 330
Basal Ganglia 330
Cerebellum 330
Localization of Motor Deficits 331
Hemiplegia 331
Cerebral Lesions 331
Cortical Lesions 331
Infarction. 331
Middle Cerebral Artery. 331
Anterior Cerebral Artery. 332
Posterior Cerebral Artery. 332
Mass Lesion. 332
Subcortical Lesions 332
Infarction. 332
Lenticulostriate Arteries. 332
Thalamoperforate Arteries. 332
Demyelinating Disease. 332
Multiple Sclerosis. 332
Acute Disseminated Encephalomyelitis. 332
Progressive Multifocal Leukoencephalopathy. 332
Migraine. 333
Seizures. 333
Tumors. 333
Infections. 333
Alternating Hemiplegia of Childhood. 333
Hemiconvulsion–Hemiplegia–Epilepsy Syndrome. 333
Brainstem Lesions 333
Brainstem Motor Organization 333
Common Lesions 334
Spinal Lesions 334
Spinal Hemisection (Brown–Séquard Syndrome) 334
Transverse Myelitis 334
Spinal Cord Compression 334
Spinal Cord Infarction 334
Peripheral Lesions 335
Psychogenic Hemiplegia 335
Monoplegia 336
Cerebral Lesions 336
Infarction 336
Transient Ischemic Attack 336
Migraine 336
Seizure 336
Multiple Sclerosis 336
Tumors 336
Infections 336
Brainstem Lesions 336
Spinal Lesions 336
Peripheral Lesions 336
Pressure Palsies 336
Hereditary Neuropathy with Predisposition to Pressure Palsies. 337
Pressure Palsies in Polyneuropathy. 337
Mononeuropathies 337
Median Nerve. 337
Carpal Tunnel Syndrome. 337
Anterior Interosseus Syndrome. 338
Pronator Teres Syndrome. 338
Ulnar Nerve. 338
Radial Nerve Palsy. 338
Femoral Neuropathy. 338
Sciatic Neuropathy. 338
Peroneal (Fibular) Neuropathy. 338
Radiculopathies 338
Plexopathies 339
Brachial and Lumbar Plexitis (or Plexopathy). 339
Neoplastic Plexus Infiltration. 339
Radiation Plexopathy. 339
Plexopathy from Hematomas. 339
Plexus Trauma. 339
Thoracic Outlet Syndrome. 339
Diabetic Amyotrophy. 340
Neuronopathies 340
Monomelic Amyotrophy. 340
Poliomyelitis. 340
Multifocal Motor Neuropathy. 340
Pitfalls in the Differential Diagnosis of Hemiplegia and Monoplegia 340
Pitfalls in the Differential Diagnosis of Hemiplegia and Monoplegia 341
Focal Weakness of Apparently Central Origin 341
Focal Weakness That Appears to Be Central: Cerebral Cortex, Internal Capsule, Brainstem, or Spinal Cord? 341
Focal Weakness That Appears to Be Central: Migraine, TIA, or Seizure? 341
Weakness of the Hand and Wrist 342
Weakness in Intrinsic Muscles of the Hand: Median Nerve, Ulnar Nerve, Brachial Plexus, or Small Cerebral Cortical Lesion? 342
Weakness of the Wrist: Radial Neuropathy or Small Cerebral Cortical Infarcts? 342
Leg Weakness 342
Peroneal Nerve Palsy or Paramedian Cerebral Cortical Lesion? 342
Cauda Equina Lesion, Myelopathy, or Paramedian Cerebral Cortical Lesion? 342
References 340
References 343
26 Paraplegia and Spinal Cord Syndromes 344
Chapter Outline 344
Common Spinal Cord Syndromes 344
Spinal Shock 344
Incomplete Lesions of the Spinal Cord 344
Unilateral Transverse Lesion 344
Central Cord Syndrome 345
Anterior Spinal Artery Syndrome 345
Anterior Horn and Pyramidal Tract Syndromes 345
Combined Posterior and Lateral Column Disease 346
Characteristic Clinical Features of Lesions at Different Levels 346
Foramen Magnum and Upper Cervical Spine 346
Lower Cervical and Upper Thoracic Spine 347
Thoracic Levels 347
Conus Medullaris and Cauda Equina 347
Pain and Autonomic Dysfunction 348
Pain Syndromes 348
Local Pain 348
Projected Pain 348
Central Neurogenic Pain 348
Autonomic Dysreflexia 349
Bowel and Bladder Dysfunction 349
References 349
References 350
27 Proximal, Distal, and Generalized Weakness 351
Chapter Outline 351
Clinical Presentation by Affected Region 351
General Considerations 351
Ocular Muscles 352
Facial and Bulbar Muscles 352
Neck, Diaphragm, and Axial Muscles 352
Proximal Upper Extremity 352
Distal Upper Extremity 353
Proximal Lower Extremity 353
Distal Lower Extremity 353
Bedside Examination of the Weak Patient 353
Observation 353
Muscle Bulk and Deformities 354
Muscle Palpation, Percussion, and Range of Motion 355
Muscle Tone 355
Strength 355
Fatigue 356
Reflexes 356
Sensory Disturbances 356
Peripheral Nerve Enlargement 356
Fasciculations, Cramps, and Other Abnormal Muscle Movements 357
Functional Evaluation of the Weak Patient 357
Walking 357
Arising from the Floor 357
Stepping onto a Stool 357
Psychogenic Weakness 358
Clinical Investigations in Muscular Weakness 358
Serum Creatine Kinase 358
Electromyography 358
Muscle Biopsy 359
Genetic Testing 359
Exercise Testing 359
Differential Diagnosis by Affected Region and Other Manifestations of Weakness 360
Disorders with Prominent Ocular Weakness 360
Disorders with Distinctive Facial or Bulbar Weakness 361
Disorders with Prominent Respiratory Weakness 362
Disorders with Distinctive Shoulder-Girdle or Arm Weakness 362
Disorders with Prominent Hip-Girdle or Leg Weakness 362
Disorders with Fluctuating Weakness 363
Disorders Exacerbated by Exercise 364
Disorders with Constant Weakness 364
Acquired Disorders Causing Weakness 365
Lifelong Disorders 366
Lifelong Nonprogressive Disorders 366
Lifelong Disorders Characterized by Progressive Weakness 366
Other Conditions 367
References 367
References 368
28 Muscle Pain and Cramps 369
Chapter Outline 369
General Features of Pain 369
Muscle Pain: Basic Concepts 369
Nociceptor Terminal Stimulation and Sensitization 369
Nociceptive Axons 370
Clinical Features of Muscle Pain 372
General Features of Muscle Pain 372
Evaluation of Muscle Discomfort 372
Muscle Discomfort: Specific Causes 373
Myopathies with Muscle Pain 373
Muscle Cramps 376
Other Involuntary Muscle Contraction Syndromes 376
Myalgia Syndromes without Chronic Myopathy 376
References 377
References 378
29 Hypotonic (Floppy) Infant 379
Chapter Outline 379
Approach to Diagnosis 379
History 379
Physical Examination 379
General Features of Hypotonia 379
Localization 380
Diagnostic Studies 381
Neuroimaging 381
Electroencephalography 381
Creatine Kinase 381
Metabolic Studies 382
Nerve Conduction Studies and Electromyography 382
Muscle Biopsy 382
Nerve Biopsy 382
Genetic Testing 382
Serology 382
Specific Disorders Associated with Hypotonia in Infancy 383
Cerebral Disorders 383
Chromosomal Disorders 383
Chronic Nonprogressive Encephalopathy 383
Chronic Progressive Encephalopathy 383
Benign Congenital Hypotonia 383
Combined Cerebral and Motor Unit Disorders 383
Acid Maltase Deficiency 384
Congenital Myotonic Dystrophy 384
Infantile Facioscapulohumeral Dystrophy 384
Syndromic Congenital Muscular Dystrophies 384
Congenital Disorders of Glycosylation 384
Lysosomal Disorders 384
Infantile Neuroaxonal Dystrophy 384
Spinal Cord Disorders 384
Acquired Spinal Cord Lesions 384
Spinal Muscular Atrophy 385
Infantile Spinal Muscular Atrophy with Respiratory Distress Type 1 385
X-linked Spinal Muscular Atrophy 385
Peripheral Nerve Disorders 385
Neuromuscular Junction Disorders 386
Juvenile Myasthenia Gravis 386
Neonatal Myasthenia 386
Congenital Myasthenic Syndromes 386
Infant Botulism 386
Muscle Disorders 386
Congenital Myopathies 387
Centronuclear Myopathy. 387
Nemaline Myopathy. 387
Central Core Disease. 387
Nonsyndromic Congenital Muscular Dystrophies 387
Merosin-Deficient Congenital Muscular Dystrophy. 387
Ullrich Congenital Muscular Dystrophy. 387
Summary 387
References 387
References 388
30 Sensory Abnormalities of the Limbs, Trunk, and Face 390
Chapter Outline 390
Anatomy and Physiology 390
Peripheral Pathways 390
Spinal Cord Pathways 390
Brain Pathways 390
Brainstem 390
Thalamus 390
Cerebral Cortex 390
Sensory Input Processing 390
Approach to Localization and Diagnosis 392
Sensory Abnormalities 392
Localization of Sensory Abnormalities 392
Peripheral Sensory Lesions 393
Spinal Sensory Lesions 394
Sensory Level. 394
Dissociated Sensory Loss. 395
Suspended Sensory Loss. 395
Sacral Sparing. 395
Brainstem Sensory Lesions 395
Cerebral Sensory Lesions 396
Thalamic Lesions. 396
Cortical Lesions. 396
Common Sensory Syndromes 396
Peripheral Syndromes 396
Sensory Polyneuropathy 396
Diabetic Neuropathies 397
Small Fiber Neuropathy 397
Acquired Immunodeficiency Syndrome-Associated Neuropathies 397
Toxic Neuropathies 397
Amyloid Neuropathy 397
Proximal Sensory Loss 397
Temperature-Dependent Sensory Loss 397
Acute Inflammatory Demyelinating Polyradiculoneuropathy 397
Mononeuropathy 398
Carpal Tunnel Syndrome. 398
Ulnar Neuropathy. 398
Radial Neuropathy. 398
Radiculopathy 398
Spinal Syndromes 398
Myelopathy 398
Syringomyelia 399
Spinal Hemisection 399
Tabes Dorsalis and Related Disorders 399
Brain Syndromes 399
Thalamic Infarction and Hemorrhage 399
Thalamic Pain Syndrome (Central Post-Stroke Pain) 399
Trigeminal Neuralgia 399
Mental Neuropathy (Numb Chin Syndrome) 399
Cortical Infarction 399
Deficits of Higher Sensory Perception 399
Functional (or Psychogenic) Sensory Loss 400
Pitfalls 400
Pitfalls 401
Acute Inflammatory Demyelinating Polyneuropathy in a Patient with Known Peripheral Neuropathy 401
Myelopathy vs Midline Cerebral Lesion 401
References 400
References 402
31 Arm and Neck Pain 403
Chapter Outline 403
Clinical Assessment 403
History 403
Neurological Causes of Pain: Sites That Can Trigger Pain 403
Muscle Spasm. 403
Central Pain. 403
Nerve Root Pain. 403
Ulnar Nerve Pain. 403
Median Nerve Pain. 403
Plexus Pain. 403
Non-Neurological Causes of Neck Pain and Brachialgia 404
Examination 405
Motor Signs—Atrophy and Weakness 405
Sensory Signs 405
Tendon Reflexes 406
Pathology and Clinical Syndromes 406
Spinal Cord Syndromes 406
Intramedullary Lesions 406
Extramedullary Lesions 406
Other Cord Compression Syndromes 406
Radiculitis 407
Brachial Plexopathy 407
Brachial Neuritis (Neuralgic Amyotrophy, Parsonage-Turner Syndrome) 407
Brachial Plexopathy in Cancer Patients 407
Thoracic Outlet Syndrome 407
Suprascapular Nerve Entrapment 407
Carpal Tunnel Syndrome 407
Ulnar Entrapment at the Elbow 408
Radial Nerve–Posterior Interosseus Nerve Syndrome 408
Complex Regional Pain Syndrome 408
“In-Between” Neurogenic and Non-Neurogenic Pain Syndrome—Whiplash Injury 409
Rheumatoid Arthritis of the Spine 409
Non-Neurological Neck/Arm Pain Syndromes 409
Fibromyalgia and Myofascial Syndrome 410
Polymyalgia Rheumatica 410
Tendonitis, Bursitis, and Arthritis 410
Shoulder. 410
Elbow 410
Epicondylitis. 410
Olecranon Bursitis. 410
Wrist 410
Tendonitis. 410
Hands. 410
References 410
Further Reading 411
32 Lower Back and Lower Limb Pain 412
Chapter Outline 412
Anatomy and Physiology 412
Approach to Diagnosis of Low Back and Leg Pain 412
History and Examination 412
Differential Diagnosis of Lower Back and Leg Pain 414
Evaluation 414
Magnetic Resonance Imaging 415
Myelography and Postmyelographic Computed Tomography 415
Nerve Conduction Studies and Electromyography 415
Radiography 417
Bone Scan 417
Clinical Syndromes 417
Lower Back and Leg Pain 417
Lumbar Spine Stenosis 417
Cauda Equina Syndrome and Conus Medullaris Syndrome 417
Lumbosacral Radiculopathy 417
Arachnoiditis 418
Plexopathy 418
Neoplastic Lumbosacral Plexopathy. 418
Plexus Injury from Retroperitoneal Abscess. 418
Plexus Injury from Retroperitoneal Hematoma. 418
Leg Pain without Lower Back Pain 418
Peripheral Nerve Syndromes 418
Femoral Neuropathy. 419
Meralgia Paresthetica. 419
Sciatic Neuropathy. 419
Piriformis Syndrome. 419
Peroneal (Fibular) Neuropathy. 419
Polyneuropathy. 419
Plexopathy 420
Lumbosacral Plexitis. 420
Diabetic Amyotrophy. 420
Herpes Zoster 420
Claudication of Leg Arteries 420
Lower Back Pain without Leg Pain 420
Mechanical Lower Back Pain 420
Sacroiliac Joint Inflammation (Sacroilitis) 420
Facet Joint Pain Syndrome 420
Lumbar Spine Osteomyelitis 421
Lumbar Spine Compression 421
Lumbar Diskitis 421
Spinal epidural abscess 421
Pitfalls 421
Pitfalls 422
Low Back Pain: Disc, Tumor, Diskitis, or Epidural Abscess 422
Lower Back Pain from Intra-abdominal and Pelvic Causes 422
Lower Back and Leg Pain from Arterial Insufficiency 422
References 421
References 423
II Neurological Investigations and Related Clinical Neurosciences 425
A General Principles 425
33 Laboratory Investigations in Diagnosis and Management of Neurological Disease 425
Chapter Outline 425
Diagnostic Yield of Laboratory Tests 426
Interpretation of Results of Laboratory Investigations 426
Risk and Cost of Investigations 427
Risk-to-Benefit Analysis 427
Lumbar Puncture 427
Cerebral Arteriography 427
Brain Biopsy 427
Cost-to-Benefit Analysis 428
Prioritization of Tests 428
Reliability of Laboratory Investigations 429
Decision Analysis 429
Research Investigations and Teaching Hospitals 429
Patient Confidentiality 429
Role of Laboratory Investigations in Neurological Disease Management 429
B Clinical Neurophysiology 430
34 Electroencephalography and Evoked Potentials 430
Chapter Outline 430
Electroencephalography 430
Physiological Principles of Electroencephalography 430
Normal Electroencephalographic Activities 432
Common Types of Electroencephalographic Abnormalities 433
Focal Polymorphic Slow Activity 433
Generalized Polymorphic Slow Activity 433
Intermittent Monomorphic Slow Activity 433
Voltage Attenuation 433
Epileptiform Discharges 433
Recording Techniques 433
Clinical Uses of Electroencephalography 434
Epilepsy 435
Focal Cerebral Lesions 438
Altered States of Consciousness 439
Metabolic Encephalopathies 440
Hypoxia 440
Infectious Diseases 440
Brain Death 442
Aging and Dementia 442
Continuous EEG Monitoring in the Intensive Care Unit 442
cEEG Monitoring for Nonconvulsive Seizures 443
Electrographic Identification of Nonconvulsive Seizures 444
The “Ictal-Interictal Continuum” 444
Periodic Discharges 445
Stimulus-Induced Rhythmic, Periodic, or Ictal Discharges 445
Quantitative EEG 445
Magnetoencephalography 448
Magnetoencephalography 449
Evoked Potentials 448
Evoked Potentials 449
Visual Evoked Potentials 449
Normal Visual Evoked Potentials 449
Visual Evoked Potentials in Neurological Disease 450
Brainstem Auditory Evoked Potentials 451
Normal Brainstem Auditory Evoked Potentials 451
Brainstem Auditory Evoked Potentials in Neurological Disease 451
Somatosensory Evoked Potentials 452
Median Nerve Somatosensory Evoked Potentials 453
Posterior Tibial Nerve Somatosensory Evoked Potentials 454
Somatosensory Evoked Potentials in Neurological Disease 455
Motor Evoked Potentials and Magnetic Coil Stimulation 455
Intraoperative Monitoring 448
Intraoperative Monitoring 457
References 448
References 460
35 Clinical Electromyography 461
Chapter Outline 461
Nerve Conduction Studies 461
Principles 461
Stimulators 461
Recording Electrodes 462
Recording Procedure 462
Motor Nerve Conduction Studies 462
Sensory Nerve Conduction Studies 463
Mixed Nerve Conduction Studies 464
Segmental Stimulation in Short Increments 464
Physiological Variability and Common Sources of Error 464
Temperature 465
Age 465
Height and Nerve Segment Lengths 465
Anomalies 465
Martin–Gruber Anastomosis. 465
Accessory Deep Peroneal Nerve. 466
Pre- and Post-Fixed Brachial Plexus. 466
Riche–Cannieu Anastomosis. 466
Temporal Dispersion and Phase Cancellation 466
Intertrial Variability 466
Electrodiagnosis by Nerve Conduction Studies 466
Focal Nerve Lesions 466
Demyelinative Mononeuropathy. 468
Axon-Loss Mononeuropathy. 469
Preganglionic (Intraspinal Canal) Lesions. 469
Generalized Polyneuropathies 470
Axonal Polyneuropathies. 470
Demyelinating Polyneuropathies. 470
Needle Electromyographic Examination 471
Principles and Techniques 471
Insertional and Spontaneous Activity 471
Normal Insertional and Spontaneous Activity 471
End-Plate Noise (see Video 35.1, available at http://www.experconsult.com). 471
End-Plate Spikes (see Video 35.2, available at http://www.experconsult.com). 471
Abnormal Insertional and Spontaneous Activity 471
Prolonged Versus Decreased Insertional Activity. 471
Fibrillation Potentials (see Video 35.3, available at http://www.experconsult.com). 472
Fasciculation Potentials (see Video 35.4, available at http://www.experconsult.com). 474
Myotonic Discharges (see Video 35.5, available at http://www.experconsult.com). 474
Myokymic Discharges (see Video 35.6, available at http://www.experconsult.com). 474
Complex Repetitive Discharges (see Video 35.7, available at http://www.experconsult.com). 474
Neuromyotonic Discharges (see Video 35.8, available at http://www.experconsult.com). 474
Cramp Discharges (see Video 35.9, available at http://www.experconsult.com). 475
Voluntary Motor Unit Action Potentials 475
MUAP Morphology (see Videos 35.10, 35.11, 35.12, 35.13, available at http://www.experconsult.com) 475
Amplitude. 475
Duration. 475
Phases. 475
MUAP Stability (see Video 35.14, available at http://www.experconsult.com) 476
MUAP Firing Patterns (see Videos 35.10, 35.13, 35.15, 35.16, available at http://www.experconsult.com) 476
Electrodiagnosis by Needle Electromyography 476
Lower Motor Neuron Disorders 476
Radiculopathies. 477
Plexopathies. 477
Mononeuropathies. 477
Peripheral Polyneuropathies. 478
Anterior Horn Cell Disorders. 478
Upper Motor Neuron Lesions 479
Myopathic Disorders 479
Specialized Electrodiagnostic Studies 480
F Wave 480
A Wave 480
H Reflex 480
Blink Reflex 481
Repetitive Nerve Stimulation 481
Principles 481
Slow Repetitive Nerve Stimulation 482
Rapid Repetitive Nerve Stimulation 483
Single-Fiber Electromyography 483
Fiber Density 484
Jitter 484
References 485
References 486
36 Neuromodulation and Transcranial Magnetic Stimulation 487
Chapter Outline 487
Methods and Their Neurophysiological Background 487
Stimulation Parameters for Diagnostic Use of TMS 487
Central Motor Conduction Measurements 487
Motor Excitability Measurements 488
Motor Thresholds 488
MEP Recruitment Curve (Stimulus Response Curve; Input–Output Curve) 488
Silent Period and Long-Interval Intracortical Inhibition (LICI) 488
Short-Interval Intracortical Inhibition (SICI) and Intracortical Facilitation (ICF) 489
Short-Interval Intracortical Facilitation (SICF) 489
Short-Latency and Long-Latency Afferent Inhibition (SAI and LAI) 489
Surround Inhibition (SI) 489
Other Inhibitory Phenomena of the Motor Cortex 489
Motor Cortical Plasticity Measurements—Paired Associative Stimulation 490
Clinical Applications for Diagnostic Use of TMS 490
Movement Disorders 490
Parkinson Disease and Parkinson Plus Syndromes 490
Dystonia 491
Huntington Disease 492
Other Neurodegenerative Disorders 492
Dementia and Mild Cognitive Impairment 492
Amyotrophic Lateral Sclerosis 492
Cerebellar Disorders 492
Epilepsy and Antiepileptic Drugs 492
Stroke 492
Multiple Sclerosis (MS) 493
Migraine 493
Cervical Myelopathy and Other Spinal Cord Lesions 493
Therapeutic Applications 493
Rationale for rTMS 493
Basic Principles of rTMS 493
Current Concepts of Therapeutic Application of rTMS 494
Depression 494
Auditory Hallucinations and Negative Symptoms in Schizophrenia 494
Parkinson Disease 494
Dystonia 495
Pain and Migraine 495
Tinnitus 495
Stroke with Motor Deficits, Aphasia, and Hemispatial Neglect 495
Amyotrophic Lateral Sclerosis 495
Epilepsy 496
Conclusion and Outlook 496
References 496
References 497
37 Deep Brain Stimulation 500
Chapter Outline 500
Parkinson Disease 501
Clinical Evidence: Randomized Controlled Trials 501
Dystonia 503
Tremor 503
Neuropsychiatric Disorders 503
Tourette Syndrome 503
Depression 504
Obsessive-Compulsive Disorder 504
Drug Addiction 504
Pain 505
Epilepsy 505
Closed Loop Stimulation—The Epilepsy Experience 505
Conclusions and the Future of DBS 505
References 505
References 506
38 Intraoperative Monitoring 509
Chapter Outline 509
Introduction 509
Techniques 509
Spinal Cord Monitoring Techniques 509
Interpretation 511
Monitoring 511
Testing 511
Response to Change 511
Prediction of Deficits 511
Anesthesia 512
Clinical Settings 512
References 512
References 513
C Neuroimaging 514
39 Structural Imaging using Magnetic Resonance Imaging and Computed Tomography 514
Chapter Outline 514
Computed Tomography 514
Magnetic Resonance Imaging 515
Basic Principles 515
T1 and T2 Relaxation Times 516
Repetition Time and Time to Echo 517
Tissue Contrast (T1, T2, and Proton Density Weighting) 518
Magnetic Resonance Image Reconstruction 519
Spin Echo and Fast (Turbo) Spin Echo Techniques 521
Gradient-Recalled Echo Sequences, Partial Flip Angle 521
Inversion Recovery Sequences (FLAIR, STIR) 522
Fat Saturation 522
Echoplanar Imaging 522
Diffusion-Weighted Magnetic Resonance Imaging 523
Perfusion-Weighted Magnetic Resonance Imaging 523
Susceptibility-Weighted Imaging 523
Diffusion Tensor Imaging 524
Magnetization Transfer Contrast Imaging 524
Structural Neuroimaging in the Clinical Practice of Neurology 520
Brain Diseases 520
Brain Tumors 520
Intra-axial Primary Brain Tumors 520
Ganglioglioma and Gangliocytoma. 520
Pilocytic Astrocytomas. 527
Pleomorphic Xanthoastrocytoma. 528
Low-Grade Astrocytomas. 527
Anaplastic Astrocytoma. 529
Oligodendroglioma. 530
Oligoastrocytoma. 531
Gliomatosis Cerebri. 532
Glioblastoma Multiforme. 532
Ependymoma. 533
Lymphoma. 533
Hemangioblastoma. 534
Extra-axial Primary Brain Tumors 533
Meningiomas. 533
Schwannoma. 536
Primitive Neuroectodermal Tumor. 535
Medulloblastoma. 535
Esthesioneuroblastoma. 536
Pineoblastoma. 535
Other Pineal Region Tumors. 537
Pineocytoma. 537
Germ Cell Tumors (Germinoma). 538
Central Neurocytoma. 539
Subependymal Giant Cell Astrocytoma. 538
Choroid Plexus Papilloma. 538
Subependymoma. 539
Tumors in the Sellar and Parasellar Region 538
Pituitary Adenomas. 538
Craniopharyngioma. 540
Metastatic Tumors 542
Advanced Structural Neuroimaging for Planning of Brain Tumor Surgery. 543
Ischemic Stroke 542
Acute Ischemic Stroke. 542
Temporal Evolution of Ischemic Stroke on Magnetic Resonance Imaging 544
Acute Stroke. 544
Subacute Ischemic Stroke (1 Day to 1 Week after Onset). 547
Late Subacute Ischemic Stroke (1 to 3 Weeks after Onset). 548
Chronic Ischemic Stroke (3 Weeks and Older). 548
Stroke Etiology 548
Watershed Ischemic Stroke. 549
Ischemic Stroke of Thromboembolic Origin. 549
Lacunar Ischemic Stroke. 550
Other Cerebrovascular Occlusive Disease 548
Microvascular Ischemic White Matter Lesions, “White Matter Disease,” Binswanger Disease. 548
CADASIL. 553
Hippocampal Sclerosis. 552
Venous Stroke. 554
Cerebral Venous Sinus Thrombosis. 552
Hemorrhagic Cerebrovascular Disease 557
Hyperacute Hemorrhage (0 to 24 Hours). 558
Acute Hemorrhage (1 to 3 Days). 558
Early Subacute Hemorrhage (3 Days to 1 Week). 558
Late Subacute Hemorrhage (1 to 4 Weeks). 558
Chronic Hemorrhage (>4 Weeks). 558
Superficial Siderosis 559
Cerebral Amyloid Angiopathy 559
Infection 558
Bacterial Meningitis. 559
Cerebritis, Abscess. 559
CNS Tuberculosis. 560
Lyme Disease. 560
Cysticercosis. 560
Herpes Simplex Encephalitis. 561
Human Immunodeficiency Virus Encephalitis. 562
Cerebral Toxoplasmosis. 563
Progressive Multifocal Leukoencephalopathy. 563
Cytomegalovirus. 563
Creutzfeldt-Jakob Disease. 563
Noninfectious Encephalitis 558
Limbic Encephalitis. 563
Multiple Sclerosis and Other White Matter Diseases 558
Multiple Sclerosis. 565
Inflammatory and Noninflammatory Lesions of the Corpus Callosum. 569
Acute Disseminated Encephalomyelitis. 568
Neurosarcoidosis. 568
Leukodystrophy. 569
Krabbe Disease. 569
Metachromatic Leukodystrophy. 569
Adrenoleukodystrophy. 569
Radiation Leukoencephalopathy. 569
Posterior Reversible Encephalopathy Syndrome. 569
Central Pontine Myelinolysis. 570
Trauma 568
Traumatic Subarachnoid Hemorrhage. 568
Subdural Hemorrhage. 571
Epidural Hemorrhage. 572
Cortical Contusion. 573
Diffuse Axonal Injury. 574
Cerebral Parenchymal Hematoma. 574
Metabolic and Toxic Disorders 574
Disorders due to Ethanol. 575
Carbon Monoxide Poisoning. 575
Methanol Poisoning. 575
Wilson Disease. 575
Mitochondrial Disease. 576
Genetic and Degenerative Disorders Primarily Causing Ataxia (Cerebellar Disorders) 574
Multiple System Atrophy, Cerebellar Subtype. 576
Spinocerebellar Ataxias. 576
Friedreich Ataxia. 577
Ataxia-Telangiectasia. 577
Fragile X Premutation Syndrome. 577
Genetic and Degenerative Disorders Primarily Causing Parkinsonism or Other Movement Disorders 578
Idiopathic Parkinson Disease. 579
Progressive Supranuclear Palsy. 579
Multiple System Atrophy, Parkinsonian Variant (Striatonigral Degeneration). 580
Huntington Disease. 580
Degenerative Disorders Primarily Causing Dementia 578
Age-Related Involutional Changes. 578
Alzheimer Disease. 581
Frontotemporal Lobar Degeneration, Including Pick Disease. 581
Neurocutaneous Syndromes 581
Neurofibromatosis. 583
Hamartoma. 583
Neurofibroma. 583
Tuberous Sclerosis (Bourneville Disease). 583
Cortical Tubers. 583
Subependymal Nodules. 583
White Matter Lesions. 583
Von Hippel-Lindau Disease. 583
Sturge-Weber Syndrome. 583
Congenital Anomalies of the Brain 581
Holoprosencephaly. 586
Septo-Optic Dysplasia. 586
Dandy-Walker Malformation. 586
Agenesis of the Corpus Callosum. 586
Gray Matter Heterotopia. 586
Pachygyria, Polymicrogyria, Lissencephaly. 586
Schizencephaly. 586
Porencephaly. 586
Hydranencephaly. 586
Lipomas. 587
Non-neoplastic Congenital Cystic Lesions 581
Epidermoid. 588
Dermoid. 589
Colloid Cyst. 589
Arachnoid Cyst. 589
Dilated Virchow-Robin Spaces. 589
Choroid Fissure Cyst. 590
Choroid Plexus Cyst. 590
Ependymal Cyst. 590
Neuroglial Cyst. 590
Vascular Malformations 594
Arteriovenous Malformations. 595
Cavernous Malformation. 595
Developmental Venous Anomaly. 595
Capillary Telangiectasia. 595
Cerebrospinal Fluid Circulation Disorders 594
Obstructive, Noncommunicating Hydrocephalus. 594
Normal-Pressure Hydrocephalus. 594
Chiari Malformation. 596
Idiopathic Intracranial Hypertension (Pseudotumor Cerebri). 598
Intracranial Hypotension. 598
Orbital Lesions 597
Ocular Tumors. 598
Optic Nerve Tumors. 598
Thyroid Ophthalmopathy. 598
Optic Neuritis. 598
Orbital Pseudotumor. 600
Spinal Diseases 597
Spinal Tumors 597
Vertebral Metastases, Extradural Tumors. 597
Extramedullary Intradural Spinal Tumors. 601
Leptomeningeal Metastases. 601
Spinal Meningiomas. 601
Nerve Sheath Tumors. 602
Embryonal Tumors. 602
Intramedullary Tumors. 601
Ependymoma. 601
Astrocytoma. 601
Intramedullary Metastases. 603
Vascular Disease 603
Spinal Cord Infarction. 604
Arteriovenous Malformation. 604
Dural Arteriovenous Fistula. 604
Cavernous Malformation. 604
Infection 605
Discitis and Osteomyelitis. 605
Epidural Abscess, Paravertebral Phlegmon. 605
Noninfectious Inflammatory Disorders 605
Multiple Sclerosis. 605
Acute Disseminated Encephalomyelitis. 605
Transverse Myelitis. 605
Sarcoidosis. 608
Vacuolar Myelopathy. 608
Trauma 607
Hangman’s Fracture. 608
Odontoid Fracture. 608
Burst Fracture. 608
Jefferson Fracture. 608
Facet Joint Disruption, Traumatic Spondylolisthesis. 609
Spinal Epidural Hematoma. 609
Spinal Subdural Hematoma. 609
Spinal Subarachnoid Hemorrhage. 610
Spinal Cord Trauma. 610
Spinal Cord Injury without Radiologic Abnormality. 611
9780323339162v2_WEB 1078
Front Cover 1078
Half title page 1079
Dedication 1080
Bradley’s Neurology in Clinical Practice 1081
Copyright Page 1082
Table Of Contents 1083
Foreword 1087
Preface 1088
List of Contributors 1089
Video Table of Contents 1096
III Neurological Diseases and Their Treatment 1101
A Principles of Management 1101
53 Management of Neurological Disease 1101
Chapter Outline 1101
Principles of Neurological Management 1101
Evidence-Based Medicine in Neurology 1101
Goals of Treatment 1102
Arresting an Attack 1102
Slowing Disease Progression 1102
Relieving Symptoms 1102
Circumventing Functional Disability 1103
Principles of Symptom Management 1103
Treatment of Common Neurological Symptoms 1103
Pain 1103
Sensory Loss, Paresthesias, and Burning Pain 1103
Weakness 1103
Ataxia 1104
Slowness of Movement or Abnormal Involuntary Movements 1104
Aphasia and Dysarthria 1104
Respiratory Failure 1104
Ethical Considerations in the Treatment of Respiratory Failure. 1104
Managing Terminal Respiratory Failure. 1105
Memory Impairment and Dementia 1105
Treatment of Secondary Effects of Neurological Disease 1105
Explaining the Prognosis 1105
Palliation and Care of the Terminally Ill Patient 1106
Genetic Counseling 1106
Legal Issues 1106
Implications for Clinical Practice 1107
References 1107
References 1108
54 Principles of Pain Management 1109
Chapter Outline 1109
Definition and Challenge 1109
Anatomy and Physiology of the Pain Pathways 1109
Central Modulation of Nociception 1110
Opioid Receptors 1110
Neuronal Plasticity and Chronic Pain 1111
Multidisciplinary Approach to Pain Management 1111
Pharmacological Management of Chronic Pain 1112
Nonsteroidal Anti-inflammatory Drugs 1112
Antidepressants 1112
Anticonvulsants 1114
Systemic Local Anesthetic Therapies 1115
Topical Analgesics 1115
Muscle Relaxants 1115
N-Methyl-d-Aspartate Receptor Blockers 1115
Opioid Analgesics 1116
Interventional Pain Management 1118
Greater Occipital Nerve Block 1118
Sphenopalatine Ganglion Block for Headache and Facial Pain 1118
Gasserian Ganglion Lesions for Trigeminal Neuralgia 1119
Stellate Ganglion Block 1120
Epidural Corticosteroid Injection 1120
Lumbar Facet Joint Block 1121
Percutaneous Disk Decompression 1121
Spinal Cord Stimulation 1122
Intrathecal Drug Delivery Systems 1123
Motor Cortex Stimulation 1123
Psychological Therapy in Chronic Pain Management 1123
Rehabilitation in Chronic Pain Management 1124
Common Pain Syndromes 1124
Trigeminal Neuralgia 1124
Low Back Pain 1125
Cervicogenic Headache 1126
Complex Regional Pain Syndrome 1126
Post-Stroke Pain Syndrome 1128
Spinal Cord Injury and Pain 1128
Pain in Multiple Sclerosis 1129
Phantom-Limb Pain and Stump Pain 1129
Summary 1130
References 1130
References 1131
55 Principles of Neurointensive Care 1133
Chapter Outline 1133
Clinical Assessment of Critically Ill Neurological Patients 1133
Monitoring in the Neurointensive Care Unit 1134
Systemic Monitoring 1134
Brain Monitoring 1135
Global Brain Monitoring Techniques 1136
Intracranial Pressure Monitoring 1136
Jugular Bulb Oximetry 1137
Electroencephalography 1138
Regional/Focal Brain Monitoring Techniques 1138
Regional Cerebral Blood Flow Monitoring 1138
Transcranial Doppler Ultrasonography 1139
Evoked Potentials 1139
Local Cerebral Oxygenation Monitoring Techniques 1139
Microdialysis 1139
Principles of Managing Critically Ill Neurological Patients 1140
Analgesia and Sedation 1140
Airway and Ventilatory Assistance 1141
Pulmonary Complications 1143
Cardiovascular Care and Blood Pressure Management 1143
Acute Coronary Syndrome 1143
Cardiac Arrhythmias 1144
Congestive Heart Failure 1144
Blood Pressure Management 1144
Acute Ischemic Stroke. 1145
Intracerebral Hemorrhage. 1145
Subarachnoid Hemorrhage. 1145
Traumatic Brain Injury. 1145
Fluid and Electrolytes 1145
Nutrition and Metabolic Derangements 1146
Fever and Infections 1147
Hematological Complications 1148
References 1148
References 1149
56 Principles of Neurointerventional Therapy 1152
Chapter Outline 1152
Ischemic Stroke 1152
Acute Stroke Treatment 1152
Sonothrombolysis 1152
Endovascular Revascularization Therapy 1152
Intra-arterial Thrombolysis 1153
Mechanical Recanalization 1153
MR CLEAN, ESCAPE, EXTEND-IA, and SWIFT-PRIME Trials 1155
Angioplasty and Stenting 1156
Carotid Artery Disease 1157
Carotid Artery Angioplasty and Stenting 1157
Angioplasty and Stenting Procedure. 1157
Clinical Trials. 1157
Intracranial Atherosclerotic Disease 1159
Intracranial Angioplasty 1159
Intracranial Angioplasty and Stenting 1159
Angioplasty/Stenting Procedure. 1160
Clinical Trials. 1160
Vertebral Artery Disease 1161
Hemorrhagic Stroke 1162
Cerebral Aneurysms 1162
Ruptured Aneurysms 1162
Unruptured Aneurysms 1163
Endovascular Treatment Modalities 1163
Coil Embolization. 1163
Balloon Remodeling. 1164
Stent-Assisted Coil Embolization. 1164
Alternative Treatments 1167
Management of Cerebral Vasospasm 1168
Balloon Angioplasty 1168
Intra-arterial Vasodilators 1168
Cerebral Arteriovenous Malformations 1169
Embolization Procedure 1169
Polyvinyl Alcohol 1169
n-Butyl Cyanoacrylate 1170
Ethylene Vinyl Alcohol Copolymer 1170
Stereotactic Radiotherapy 1171
Cerebral Arteriovenous Fistulas 1171
Cranial Dural Arteriovenous Fistulas 1171
Carotid-Cavernous Fistulas 1172
Spinal Vascular Malformations 1173
Spinal Dural Arteriovenous Fistula (Type I) 1174
Glomus Arteriovenous Malformation (Type II) 1174
Juvenile/Metameric Arteriovenous Malformation (Type III) 1174
Perimedullary Arteriovenous Malformation (Type IV) 1174
Tumor Embolization 1174
Embolization Procedure 1175
Embolization Materials 1175
Vessel Selection 1176
Clinical Evidence 1176
Idiopathic Intracranial Hypertension 1176
References 1177
References 1178
57 Neurological Rehabilitation 1184
Chapter Outline 1184
Goals and Structure of Rehabilitation 1184
Aims 1184
Personnel and Strategies 1185
Physicians 1185
Rehabilitation Nursing 1185
Physical Therapists 1186
Exercise and Compensatory Functional Training. 1186
Conditioning and Strengthening. 1186
Neurophysiological Schools. 1186
Motor Learning Approaches. 1187
Task-Oriented Practice. 1187
Assistive Equipment. 1187
Occupational Therapists 1187
Speech and Cognitive Therapists 1188
Recreational Therapists 1189
Psychologists 1189
Social Workers 1189
Orthotists and Bracing 1189
Measurement Tools 1190
Organization of Services 1191
Service Provision 1191
Inpatient Rehabilitation Unit. 1191
Community-Based Services. 1192
Biological Bases for Rehabilitative Interventions 1192
Neuromedical Problems during Rehabilitation 1193
Frequency of Complications 1193
Complications in Patients with Stroke 1193
Complications in Patients with Spinal Cord Injury 1193
Complications in Patients with Traumatic Brain Injury 1194
Management of Neuromedical Problems 1194
Dysphagia 1194
Skin Ulcers 1195
Contractures 1195
Heterotopic ossification 1195
Dysautonomia 1195
Bowel and Bladder Dysfunction 1196
Fatigue 1197
Central Pain 1197
Sleep Disorders 1197
Spasticity and Upper Motor Neuron Syndrome 1197
Mechanisms. 1197
Assessments. 1197
Treatment. 1198
Physical Modalities. 1198
Pharmacotherapy. 1198
Chemical Injections. 1199
Surgical Interventions. 1199
Therapies for Impairments and Disabilities 1199
Gait Training 1199
Constraint-Induced Upper Extremity Therapy 1201
Bimanual Upper Extremity Therapy 1201
Virtual Reality Training 1201
Mirror Therapy and Imagery 1202
Instrumented Biofeedback 1202
Acupuncture 1202
Visual Field Deficits 1202
Mechanical and Robotic-Assistive Devices 1202
Upper Extremities 1202
Mobility 1202
Functional Neuromuscular Stimulation 1203
Brain Stimulation 1203
Neural Prostheses and Brain–Computer Interfaces 1204
Pharmacological Adjuncts 1204
Mobile Health and Wireless Sensing Devices 1204
Therapies for Cognitive and Behavioral Disabilities 1204
Overview of Cognitive Therapy 1204
Aphasia 1205
Treatments 1205
Pharmacological Adjuncts 1206
Outcomes 1206
Apraxia 1206
Attentional Disorders 1206
Memory Disturbances 1207
Frequency of Memory Disturbance across Diseases 1207
Treatments 1207
Pharmacological Adjuncts 1207
Outcomes 1208
Hemineglect 1208
Frequency 1208
Treatments 1209
Behavioral Disorders 1209
Affective Disorders 1210
Incidence 1210
Treatment 1210
Functional Outcomes with Rehabilitation 1210
Stroke 1210
Ambulation 1211
Self-Care Skills 1211
Spinal Cord Injury 1211
Traumatic Brain Injury 1212
Parkinson Disease 1212
Multiple Sclerosis 1212
Other Diseases 1213
Aging with Neurological Disabilities 1213
Future Directions 1213
References 1213
References 1214
B Neurological Complications of Systemic Disease 1216
58 Neurological Complications of Systemic Disease 1216
Chapter Outline 1216
Cardiac Disorders and the Nervous System 1217
Cardiogenic Embolism 1217
Syncope 1217
Cardiac Arrest 1218
Complications of Cardiac Catheterization and Surgery 1218
Neurological Complications of Medication 1219
Infective Endocarditis 1219
Diseases of the Aorta 1220
Aortic Aneurysms 1220
Aortitis 1220
Coarctation of the Aorta 1221
Subclavian Steal Syndrome 1221
Complications of Aortic Surgery 1221
Connective Tissue Diseases and Vasculitides 1221
Polyarteritis Nodosa, Churg-Strauss Syndrome, and Overlap Syndrome 1221
Giant Cell Arteritis 1222
Granulomatosis with Polyangiitis 1222
Isolated Angiitis of the Nervous System 1223
Rheumatoid Arthritis 1223
Systemic Lupus Erythematosus 1223
Sjögren Syndrome 1223
Progressive Systemic Sclerosis 1224
Behçet Disease 1224
Relapsing Polychondritis 1224
Respiratory Diseases 1224
Hypoxia 1224
Hypercapnia 1224
Hypocapnia 1224
Systemic Inflammatory Response Syndrome 1224
Sarcoidosis 1225
Hematological Disorders with Anemia 1226
Megaloblastic Anemia 1226
Sickle Cell Disease 1226
Thalassemias 1226
Acanthocytic Syndromes 1226
Proliferative Hematological Disorders 1226
Leukemias 1226
Plasma Cell Dyscrasias 1226
Myelomatosis 1226
Waldenström Macroglobulinemia 1227
Monoclonal Gammopathy of Undetermined Significance 1227
Amyloidosis 1227
Cryoglobulinemia 1227
Lymphoma 1228
Polycythemia 1228
Hemorrhagic Diseases 1228
Hemophilia 1228
Other Hemorrhagic Disorders 1228
Disseminated Intravascular Coagulation 1228
Thrombocytopenia 1228
Thrombotic Thrombocytopenic Purpura 1228
Iatrogenic Hemorrhagic Disorders 1228
Antiphospholipid Antibody Syndromes 1229
Liver Disease 1229
Portal-Systemic Encephalopathy 1229
Chronic NonWilsonian Hepatocerebral Degeneration 1229
Liver Transplantation 1230
Pancreatic Encephalopathy 1230
Gastrointestinal Diseases 1230
Gastric Surgery 1230
Small-Bowel Disease 1230
Celiac Disease 1230
Whipple Disease 1230
Renal Failure 1231
Overview of Related Neurological Complications 1231
Neurological Complications of Dialysis 1231
Neurological Complications of Renal Transplantation 1232
Electrolyte Disturbances 1232
Sodium 1232
Potassium 1232
Calcium 1232
Magnesium 1233
Pituitary Disease 1233
Pituitary Adenomas 1233
Cushing Disease and Syndrome 1233
Hypopituitarism 1234
Diabetes Insipidus 1234
Thyroid Disease 1234
Hyperthyroidism 1234
Hypothyroidism 1234
Hashimoto Thyroiditis 1234
Parathyroid Disease 1235
Hyperparathyroidism 1235
Hypoparathyroidism 1235
Adrenal Glands 1235
Pheochromocytoma 1235
Addison Disease 1235
Diabetes Mellitus 1235
Peripheral Nervous System 1235
Central Nervous System 1235
Hypoglycemia 1236
References 1236
References 1237
59 Neurological Complications of Systemic Disease: 1238
Chapter Outline 1238
Cardiac Disorders and the Nervous System 1238
Congenital Heart Disease 1238
Cerebral Dysgenesis and Malformations 1238
Chromosomal and Genetic Disorders 1238
Neurological Complications Unrelated to Intervention and Cardiac Surgery 1239
Cerebrovascular Accidents in Uncorrected Congenital Heart Disease 1239
Brain Abscess 1239
Infective Endocarditis 1239
Cognitive Impairment 1239
Neurological Complications of Intervention and Cardiac Surgery 1240
Cardiac Transplantation 1240
Acquired Heart Disease 1241
Connective Tissue Diseases and Vasculitides 1241
Polyarteritis Nodosa 1241
Kawasaki Disease 1241
Henoch-Schönlein Purpura 1242
Takayasu Arteritis 1242
Churg-Strauss Syndrome 1242
Juvenile Rheumatoid Arthritis 1242
Systemic Lupus Erythematosus 1242
Granulomatosis with Polyangiitis (Wegener) 1243
Behçet Disease 1244
Sjögren Syndrome 1244
Primary Angiitis of the Central Nervous System 1244
Respiratory Disorders 1244
Periodic Breathing and Apnea 1244
Bronchopulmonary Dysplasia 1244
Cystic Fibrosis 1245
Sarcoidosis 1245
Hypertension 1245
Hematological Disorders 1246
Hemolytic Disease of the Newborn and Kernicterus 1246
Hemorrhagic Disease of the Newborn 1246
Neonatal Polycythemia 1246
Sickle Cell Disease 1246
Hemophilia 1247
Thrombotic Thrombocytopenic Purpura 1247
Hemolytic-Uremic Syndrome 1248
Gastrointestinal Disorders 1248
Hepatic Encephalopathy 1248
Liver Transplantation 1248
Endocrine Disorders 1249
Thyroid Disorders 1249
Hypothyroidism 1249
Hyperthyroidism 1249
Parathyroid Disorders 1249
Adrenal Gland Dysfunction 1250
Addison Disease 1250
Cushing Syndrome 1250
Excess Mineralocorticoid Secretion 1250
Pituitary Disorders 1250
Diabetes Mellitus 1250
Renal Disorders 1251
Renal Failure 1251
Complications of Dialysis 1251
Headaches 1251
Dialysis Disequilibrium Syndrome 1251
Progressive Dialysis Dementia 1251
Renal Transplantation 1251
References 1252
References 1253
C Trauma of the Nervous System 1255
60 Basic Neuroscience of Neurotrauma 1255
Chapter Outline 1255
Experimental Models of Traumatic Brain Injury 1255
Percussion Concussion 1255
Acceleration Concussion 1256
Military Models 1256
In Vitro Models 1256
Neuronal Damage after Traumatic Brain Injury 1256
Temporal Patterns of Neuronal Death 1256
Selective Neuronal Vulnerability 1257
Progressive Damage 1257
Secondary and Repetitive Damage 1258
Axonal and Dendritic Injury 1258
Importance of Gender 1259
Basic Mechanisms of Injury 1260
Primary Injury Mechanisms 1260
Secondary Injury Mechanisms 1260
Therapeutic Interventions Directed Against Pathophysiological Processes 1261
Glutamate Antagonists 1261
Free Radical Scavengers 1261
Neurotrophic Factors 1261
Protection by Nitric Oxide-Related Species 1261
Inflammation 1262
Antiapoptotic Agents 1262
Therapeutic Hypothermia 1262
Recovery of Function 1263
Environmental Enrichment 1263
Reparative and Transplantation Strategies 1263
Summary and Future Directions 1264
References 1264
References 1265
61 Sports and Performance Concussion 1266
Chapter Outline 1266
Epidemiology 1266
Definition 1266
Pathophysiology 1266
Neurometabolic Cascade 1266
Cerebral Blood Flow 1267
Axonal Dysfunction 1267
Synaptic Plasticity and Neural Activation 1267
Diagnosis 1267
In-Office/Outpatient Evaluation 1268
Neuropsychological Testing 1268
Neuroimaging 1268
Acute and Subacute Management 1268
Return to School, Work, or Cognitive Activity 1269
Return to Physical Activity 1269
Risk Involved with Premature Return to Participation 1270
Long-Term Effects 1270
Post-Concussion Syndrome (PCS) 1270
Chronic Traumatic Encephalopathy 1271
When to Retire an Athlete 1271
Prevention 1272
References 1272
References 1273
62 Craniocerebral Trauma 1275
Chapter Outline 1275
Epidemiology 1275
Classification 1275
Mechanism 1275
Injury Severity 1276
Morphology 1277
Skull Fractures 1277
Intracranial Injuries 1277
Pathophysiolgy 1280
Treatment 1281
Evaluation 1281
Management of Diffuse Injuries 1281
Management of Focal Injuries 1286
Late Complications 1287
Outcome 1287
Future of TBI 1288
References 1288
References 1289
63 Spinal Cord Trauma 1291
Chapter Outline 1291
Epidemiology 1291
Pathophysiology 1291
Clinical Presentation 1293
Spinal Cord Injury Syndromes 1293
Central Cord Syndrome 1293
Anterior Cord Syndrome 1294
Posterior Column Syndrome 1294
Brown–Séquard Syndrome 1294
Cervicomedullary Syndrome 1295
Conus Medullaris Syndrome 1295
Cauda Equina Syndrome 1296
Transient Spinal Cord Syndromes 1296
Spinal Shock 1296
Mechanisms and Types of Injuries 1297
Cervical Spine Fractures 1297
Atlanto-occipital Dissociation 1297
Occipital Condyle Fractures 1297
Atlantoaxial Injuries 1297
Subaxial Cervical Spine Injuries 1298
Thoracolumbar Injuries 1299
Penetrating Spinal Cord Injuries 1299
Management of Acute Spinal Cord Injuries 1299
Management in the Field 1299
Initial Hospital Assessment 1300
Radiographic Evaluation 1300
Current Guidelines 1300
Plain Radiography 1300
Computed Tomography 1301
Magnetic Resonance Imaging 1301
SCIWORA 1301
Treatment of Spinal Cord Injuries in the Acute Setting 1301
Cardiovascular Management in the Intensive Care Unit Setting 1301
Respiratory Management in the Intensive Care Unit Setting 1302
Medical Management 1303
Nutritional Support 1304
Stabilization and Support 1304
Nonsurgical Management 1304
Surgical Management 1305
Long-Term Management of Spinal Cord Injuries 1306
Spinal Cord Injury and Bladder Function 1306
Spinal Cord Injury and Bowel Function 1308
Delayed Post-traumatic Spinal Cord Syndromes 1309
Post-traumatic Syringomyelia 1309
Neuropathic Pain 1309
Spasticity 1310
Autonomic Dysreflexia 1311
Deep Vein Thrombosis and Thromboembolism in Spinal Cord Injury 1312
Future Directions 1312
References 1313
References 1314
64 Trauma of the Nervous System: Peripheral Nerve Trauma 1317
Chapter Outline 1317
Anatomy of the Spinal Nerves of the Peripheral Nervous System 1317
Axon 1317
Peripheral Nerve Trunks 1318
Classification of Nerve Trauma 1319
Peripheral Nerve Degeneration and Regeneration 1319
Segmental Demyelination 1319
Wallerian Degeneration 1320
Distal Segment Changes 1320
Proximal Segment 1320
Nerve Regeneration 1320
Mechanisms of Traumatic Nerve Injury 1321
Compression 1321
Stretch and Traction 1321
Laceration 1322
Crush 1322
Gunshot 1322
Radiation 1322
Cold Injury 1322
Electrical 1323
Injection 1323
Evaluation of Nerve Trauma 1323
Clinical and Electrodiagnostic Examination 1323
Imaging Studies 1324
Surgical Repair of Nerve Trauma 1325
Indications for and Timing of Surgical Repair 1325
Intraoperative Nerve Monitoring 1327
Surgical Techniques 1328
Neurolysis (External and Internal) 1328
Primary Neurorrhaphy 1328
Nerve Grafting 1329
Nerve Transfer 1330
Management of Neuropathic Pain Associated with Nerve Trauma 1331
Pharmacological Options 1331
Interventional Strategies 1331
Movement Disorders after Nerve Trauma 1331
Future Directions in Treatment of Nerve Trauma 1333
References 1333
References 1334
D Vascular Diseases of the Nervous System 1336
65 Ischemic Cerebrovascular Disease 1336
Chapter Outline 1336
Epidemiology and Risk Factors 1336
Risk Factors for Stroke 1337
Heredity and Risk of Stroke 1337
Common Modifiable Risk Factors 1337
Other Risk Factors for Stroke 1340
Pathophysiology of Cerebral Ischemia 1340
Pathology of Ischemic Stroke 1341
Clinical Syndromes of Cerebral Ischemia 1341
Transient Ischemic Attacks 1341
Carotid Artery System Syndromes 1343
Carotid Artery Syndromes 1343
Middle Cerebral Artery Syndromes 1343
Syndromes of the Anterior Cerebral Artery and Related Blood Vessels 1344
Lacunar Syndromes 1344
Vertebrobasilar System Syndromes 1345
Posterior Cerebral Artery Syndromes 1346
Syndromes of Thalamic Infarction 1348
Watershed Ischemic Syndromes 1348
Diagnosis and Treatment of Threatened Ischemic Stroke 1348
Large-Artery Atherothrombotic Infarctions 1350
Small-Vessel or Penetrating Artery Disease 1351
Cardiogenic Embolism 1351
Nonatherosclerotic Vasculopathies 1354
Dissections 1354
Trauma 1356
Radiation Vasculopathy 1356
Moyamoya 1356
Fibromuscular Dysplasia 1358
Inflammatory Vasculitides 1358
Infections and Stroke 1359
Drug Abuse and Stroke 1359
Stroke and Systemic Vasculitides 1360
Migraine and Stroke 1362
Inherited and Miscellaneous Disorders 1363
Hypercoagulable Disorders 1365
Primary Hypercoagulable States 1365
Secondary Hypercoagulable States 1367
Sickle Cell Disease 1370
Thrombotic Thrombocytopenic Purpura 1370
Infarcts of Undetermined Cause 1370
Essential Investigations for Patients with Threatened Strokes 1370
Neuroimaging 1371
Cardiac Evaluation of the Stroke Patient 1372
Cerebral Angiography 1373
Preventing Stroke Recurrence: Medical Therapy 1374
Platelet Antiaggregants 1374
Oral Anticoagulants 1375
Treatment of Acute Ischemic Stroke 1377
Heparins and Heparinoids 1377
Thrombolytic Therapy 1377
Defibrinogenating and Hemorheological Agents 1382
Neuroprotective Agents 1382
Hypothermia 1383
Surgical Therapy 1382
Symptomatic Carotid Artery Stenosis 1382
Asymptomatic Carotid Artery Stenosis 1384
Stenting of the Carotid Artery and Other Cervicocerebral Vessels 1385
General Management of Acute Ischemic Stroke 1386
Cerebral Venous Thrombosis 1388
References 1393
References 1394
66 Intracerebral Hemorrhage 1399
Chapter Outline 1399
Mechanisms of Intracerebral Hemorrhage 1399
Hypertension 1399
Vascular Malformations 1399
Intracranial Tumors 1400
Bleeding Disorders, Anticoagulants, and Fibrinolytic Treatment 1400
Cerebral Amyloid Angiopathy 1402
Granulomatous Angiitis of the Central Nervous System and Other Vasculitides 1403
Sympathomimetic Agents 1405
Hemorrhagic Infarction 1406
Head Trauma 1406
Clinical Features of Intracerebral Hemorrhage 1406
Putaminal Hemorrhage 1407
Caudate Hemorrhage 1409
Thalamic Hemorrhage 1409
Lobar Hemorrhage 1409
Cerebellar Hemorrhage 1412
Pontine Hemorrhage 1412
Mesencephalic Hemorrhage 1412
Medullary Hemorrhage 1414
Intraventricular Hemorrhage 1414
Treatment of Intracerebral Hemorrhage 1414
General Management of Intracerebral Hemorrhage 1414
Initial Evaluation 1414
General Measures for Prevention of Further Elevation of Intracranial Pressure 1416
Specific Treatment of Increased Intracranial Pressure 1416
Choice between Medical and Surgical Therapy in Intracerebral Hemorrhage 1416
Hemostatic Therapy of Intracerebral Hemorrhage 1417
References 1417
References 1419
67 Intracranial Aneurysms and Subarachnoid Hemorrhage 1421
Chapter Outline 1421
Epidemiology 1421
Associated Conditions 1421
Pathophysiology 1422
Saccular Aneurysm 1422
Fusiform Aneurysm 1423
Dissecting Aneurysm 1423
Natural History of Intracranial Aneurysms 1423
Imaging Modalities and Diagnosis 1425
Subarachnoid Hemorrhage 1425
Symptoms 1428
Treatment of Incidental Intracranial Aneurysms 1428
Open Surgical Treatment 1429
Endovascular Treatment 1429
Management of Subarachnoid Hemorrhage 1431
Endovascular and Surgical Treatment of Ruptured Aneurysms 1431
Vasospasm and Delayed Cerebral Ischemia 1432
References 1433
References 1434
68 Stroke in Children 1436
Chapter Outline 1436
Stroke and the Developing Cerebrovascular System 1436
Epidemiology 1436
Full-Term and Near-Term Neonates 1436
The General Population of Children 1436
High-Risk Subgroups 1436
Presentations 1438
Etiology 1439
Cardiac 1439
Hematological 1439
Trauma and Vascular Compression 1439
Infection 1440
Vascular Malformations/Vasculopathy/Migraine 1440
Drugs/Toxins 1440
Metabolic 1441
Gender and Ethnicity 1441
Genetic 1441
Differential Diagnosis 1442
Evaluation 1442
History and Physical 1442
Imaging Studies 1444
Coagulation Workup 1444
Cardiac Evaluation 1444
Other Studies 1445
Treatment 1445
The Acute Period and Initiating Chronic Therapy 1445
Additional Issues in Chronic Therapy 1445
Other Issues 1446
Summary 1446
References 1446
References 1447
69 Spinal Cord Vascular Disease 1450
Chapter Outline 1450
Vascular Anatomy of the Spinal Cord 1450
Vascular Anatomy of the Spinal Cord 1451
Intrinsic Spinal Cord Arterial Supply 1451
Venous Drainage 1451
Spinal Cord Infarction 1450
Presentation and Initial Course 1450
Examination Findings 1450
Investigations 1450
Course and Prognosis 1453
Causes of Spinal Cord Infarction 1454
Treatment 1455
Spinal Vascular Malformations 1455
Distribution and Prevalence 1455
Clinical Presentation and Course 1455
Investigations 1456
Treatment 1457
Spinal Cavernous Malformation 1458
Spinal Hemorrhage 1458
Subarachnoid Hemorrhage 1458
Hematomyelia 1458
Spinal Epidural and Subdural Hemorrhage 1458
References 1459
References 1460
70 Central Nervous System Vasculitis 1461
Chapter Outline 1461
Types of Central Nervous System Vasculitis 1461
Isolated Central Nervous System Vasculitis 1461
Clinical Findings 1461
Pathology 1461
Laboratory Findings 1462
Approach to Diagnosis 1462
Therapy 1462
Central Nervous System Vasculitis Associated with Other Disorders 1463
Cutaneous Herpes Zoster Infection 1463
Drug Abuse 1463
Lymphoma 1463
Amyloid 1463
References 1463
References 1464
E Cancer and the Nervous System 1465
71 Epidemiology of Brain Tumors 1465
Chapter Outline 1465
Classification 1465
Descriptive Epidemiology 1465
Incidence 1465
Mortality and Prognostic Factors 1466
Gender and Race 1466
Temporal Trends 1467
Geographical Trends and Migrant Studies 1467
Primary Central Nervous System Lymphoma 1467
Analytical Epidemiology 1467
Study Designs 1467
Methodological Challenges 1468
Radiation 1468
Ionizing Radiation 1468
Electromagnetic Field Radiation 1468
Radiofrequency and Cellular Telephone 1468
Occupational Studies 1469
Head Trauma 1469
N-Nitroso Compounds 1469
Vitamins 1470
Tobacco and Alcohol 1470
Tea and Coffee 1470
Infections, Allergies and Immunity 1470
Genetic Syndromes 1471
Genetic Susceptibility 1472
Summary 1472
References 1472
References 1473
72 Pathology and Molecular Genetics 1475
Chapter Outline 1475
General Principles of Nervous System Tumor Biology 1475
History of Nervous System Tumor Classification Schemes 1475
General Histopathological Features and Techniques 1476
General Histopathological Features 1476
Anaplasia 1477
Tumor Grading 1477
Palisading 1477
Rosettes 1477
Desmoplasia 1477
Microvascular Proliferation 1477
Frozen Sections and Touch Imprints/Smears 1477
Electron Microscopy (Ultrastructural Pathology) 1478
Immunohistochemistry 1478
Glial Markers 1478
Neuronal Markers 1478
Epithelial Markers 1479
S100 Protein 1479
Other Useful Immunomarkers 1479
Methods of Assessing Cell Proliferation 1479
Molecular Diagnostics 1479
Primary Neuroepithelial Tumors 1479
Diffuse Astrocytoma (WHO Grade II–III) 1480
Oligodendroglioma (WHO Grade II–III) 1480
Oligoastrocytoma (WHO Grade II–III) 1481
Glioblastoma (WHO Grade IV) 1481
Molecular Characteristics of Diffuse Gliomas 1481
Circumscribed (“Favorable Prognosis”) Neuroepithelial Tumors 1482
Pilocytic Astrocytoma (WHO Grade I) 1483
Pleomorphic Xanthoastrocytoma (WHO Grade II or III) 1483
Subependymal Giant Cell Astrocytoma (WHO Grade I) 1483
Ganglioglioma/Gangliocytoma (WHO Grade I or II) 1484
Dysembryoplastic Neuroepithelial Tumor (WHO Grade I) 1484
Other Glioneuronal Tumors 1484
Central Neurocytoma (WHO Grade II) 1485
Ependymoma (WHO Grade II or III) 1485
Myxopapillary Ependymoma (WHO Grade I) 1485
Subependymoma (WHO Grade I) 1486
Choroid Plexus Tumors 1486
Choroid Plexus Papilloma (WHO Grade I) 1486
Choroid Plexus Carcinoma (WHO Grade III) 1486
Embryonal Tumors/Primitive Neuroectodermal Tumors 1487
Medulloblastoma (WHO Grade IV) 1487
Atypical Teratoid/Rhabdoid Tumor (WHO Grade IV) 1488
Meningeal/Extra-axial Tumors 1488
Meningioma (WHO Grade I) 1488
Atypical Meningioma (WHO Grade II) 1488
Anaplastic Meningioma (WHO Grade III) 1489
Molecular Features of Meningioma 1489
Hemangiopericytoma and Solitary Fibrous Tumor (WHO Grade II or III) 1489
Nerve Sheath Tumors 1490
Schwannoma (Neurilemoma) (WHO Grade I) 1490
Neurofibroma (WHO Grade I) 1490
Miscellaneous Tumors 1490
Central Nervous System Lymphoma 1490
Germ Cell Tumors 1491
Hemangioblastoma (WHO Grade I) 1492
Craniopharyngioma (WHO Grade I) 1492
Epidermoid and Dermoid Cysts 1492
Neuroenteric, Colloid, and Rathke Cleft Cysts 1492
Lipomas 1493
Metastatic Tumors 1493
Acknowledgments 1493
References 1493
References 1494
73 Clinical Features of Brain Tumors and Complications of Their Treatment 1496
Chapter Outline 1496
Clinical Features of Brain Tumors 1496
Overview of Symptoms 1496
Specific Symptoms and Signs 1496
Focal Symptoms 1496
Focal Motor Symptoms. 1496
Ataxia. 1496
Aphasia. 1497
Visual Problems. 1497
Seizures. 1497
Generalized Symptoms 1497
Headaches. 1497
Nausea and Vomiting. 1498
Syncope. 1498
Mental Status and Behavioral Changes. 1498
Complications of Brain Tumor Treatment 1498
Surgery 1498
Radiation 1499
Chemotherapy and Biologic Therapy 1499
References 1499
References 1500
74 Primary Nervous System Tumors in Adults 1501
Chapter Outline 1501
General Aspects 1501
Established Treatment Strategies 1501
Surgery 1501
Radiation Therapy 1502
Conventional Fractionated Radiotherapy. 1502
Brachytherapy. 1502
Sensitization of Tumor Cells to Ionizing Radiation. 1502
Stereotactic Radiosurgery Techniques. 1502
Chemotherapy 1503
Standard Cytotoxic Chemotherapy. 1503
Delivery Strategies. 1503
New Treatment Strategies 1503
Management of Specific Brain Tumors 1507
Neuroepithelial Tumors 1507
Astrocytic Tumors 1507
Noninfiltrative Tumors 1507
Pilocytic Astrocytoma. 1507
Pleomorphic Xanthoastrocytoma. 1507
Subependymal Giant Cell Astrocytoma. 1508
Neuroepithelial Tumors of Unknown Origin: Chordoid Glioma of the Third Ventricle. 1508
Infiltrative Low-Grade Astrocytomas. 1508
High-Grade Astrocytomas 1509
Anaplastic Astrocytoma. 1509
Glioblastoma Multiforme. 1509
Others 1510
Gliomatosis Cerebri. 1510
Oligodendroglial Tumors 1510
Oligodendroglioma and Oligoastrocytoma. 1510
Anaplastic Oligodendroglioma and Anaplastic Oligoastrocytoma. 1510
Ependymal Tumors 1510
Ependymoma and Anaplastic Ependymoma. 1510
Subependymoma. 1510
Choroid Plexus Tumors 1510
Choroid Plexus Papilloma and Carcinoma. 1510
Neuronal and Mixed Neuronal-Glial Tumors 1511
Ganglioglioma and Gangliocytoma. 1511
Central Neurocytoma. 1511
Pineal Parenchymal Tumors 1511
Peripheral Neuroblastic Tumors 1511
Esthesioneuroblastoma. 1511
Embryonal Tumors 1511
Medulloblastoma. 1511
Supratentorial Primitive Neuroectodermal Tumors. 1512
Tumors of Cranial and Peripheral Nerves 1512
Schwannoma and Neurofibroma 1512
Malignant Peripheral Nerve Sheath Tumor 1513
Meningeal Tumors 1513
Meningioma 1513
Hemangiopericytoma 1514
Neuraxis Tumors Derived from the Hematopoietic System 1514
Primary Central Nervous System Lymphoma 1514
NonAIDS Related Disease. 1514
AIDS-Related PCNSL. 1515
Germ Cell Tumors 1515
Germinoma 1515
Nongerminomatous Germ Cell Tumors 1515
Tumors of the Sellar Region 1515
Craniopharyngioma 1515
Pituitary Adenoma 1516
References 1516
References 1517
75 Primary Nervous System Tumors in Infants and Children 1520
Chapter Outline 1520
Pediatric Primary Nervous System Tumors 1520
Embryonal Tumors 1520
Medulloblastoma and Central Nervous System Primitive Neuroectodermal Tumor 1520
Background 1520
Etiology 1520
Clinical Presentation 1522
Diagnosis 1522
Management 1522
Prognosis 1524
Atypical Teratoid/Rhabdoid Tumor 1524
Background 1524
Clinical Presentation 1524
Diagnosis 1524
Management and Prognosis 1524
Astrocytic Tumors 1525
Pilocytic Astrocytoma 1525
Background 1525
Clinical Presentation 1525
Diagnosis 1525
Management 1526
Prognosis 1526
Optic Pathway Glioma 1526
Background 1526
Clinical Presentation 1526
Diagnosis and Management 1527
Prognosis 1527
Subependymal Giant Cell Astrocytoma 1527
Background 1527
Clinical Presentation 1527