Concise Neurology

CONTENTS
Preface vii
Acknowledgments ix
List of Abbreviations xi
1
Basics of Metabolism and Pediatric Neurology ..............................................1Spectrum of Vitamin B
Carbohydrate Metabolism: Inherited Muscle Glycogenosis 3
Mitochondrial Metabolism 4
Lipid Metabolism 5
Purine Metabolism Disorders 6
Peroxisomal Disorders 11
Lysosomal Disorders: Sphingolipidoses 12
Lysosomal Disorders: Mucopolysaccharidoses 14
Porphyrias 15
Dopamine and Catecholamine Metabolic Disorders 16
Norepinephrine System 17
Serotonin System 18
Electrolyte Derangements 19
Voltage-Gated Channels (Ionic Interaction) 24
Gamma-Aminobutyric Acid 27
Glutamate Receptors 28
Autism Spectrum Disorders 29
Attention-Defi cit Hyperactivity Disorder 30
Floppy Infant Syndrome 31
Microcephaly 33
Macrocephaly 35
Glutaric Aciduria Type I 36
Biotinidase Defi ciency 37
Mitochondrial Diseases 38
Mitochondrial Encephalomyopathy Syndromes 39
Maternally Inherited Leigh Syndrome and Related Disorders 40
Primary White Matter Disorders 41
Primary Gray Matter Disorders 43
Symmetrical Bilateral Signal Abnormalities within the Basal Ganglia 44
Angelman Syndrome 45
Neurocutaneous Syndromes (Phakomatoses) 46
12-Requiring Disorders 2 2
Stroke, Migraine, and Epilepsy .........................................................................48Stroke 49
Emergency Treatment of Ischemic Stroke 53
Antiplatelet Drugs 54
Carotid Endarterectomy 56
Transient Ischemic Attack 57
Subarachnoid Hemorrhage 58
Intracerebral Hemorrhage 59
Increased Intracranial Pressure 60
Patent Foramen Ovale 61
Neurovascular Syndromes 62
Thalamic Syndromes 64
CADASIL 70
Important Stroke Mimickers 72
Thrombotic Microangiopathies 74
Disseminated Intravascular Coagulation 75
Epilepsy 76
Generalized Frontally Dominant Slow (3.5 Hz) Spike and Wave 77
Centrotemporal Spikes and Waves 77
Febrile Seizures 78
Antiepileptic Agents 79
Mechanisms of Action of AEDs 80
Surgery for Epilepsy 82
Status Epilepticus 83
Epilepsy in Women 84
Neonatal and Pediatric EEG 85
Brainstem Auditory Evoked Potentials 87
Visual Evoked Potentials 88
Somatosensory Evoked Potentials 88
Myoclonic Encephalopathies 90
Atypical Progressive Myoclonic Encephalopathy 91
Myoclonus and Renal Failure 92
Epileptic Encephalopathies with Myoclonus 93
Sleep Disorders 94
Coma 98
Persistent Vegetative State 99
Brain Death 100
Migraine and Other Headaches 101
Selected Secondary Headaches 107
3
Infectious Diseases ..........................................................................................108Cerebrospinal Fluid 109
Viral Encephalitis 111
Imaging of Viral Encephalitis 112
Aseptic Meningitis Syndrome 115
Postencephalitic Parkinsonism 116
HIV/AIDS-Related Infectious Disorders 117
Neuroimaging of Abscesses 119
Neurosyphilis 120
Lyme Disease (Neuroborreliosis) 121
Progressive Multifocal Leukoencephalopathy 122
Parasitic Diseases of the Nervous System 123
Neurosarcoidosis 125
Whipple Disease 126
Subacute Sclerosing Panencephalitis 127
Prion Diseases 128
Pathology of Infectious Diseases 130
Pathology of Fungal Infections 131
Pathology of Prion Diseases 133
4
Neurotoxicology ................................................................................................134Manganese Toxicity 135
Botulism 136
Nonbotulism Causes of Toxic Weakness 137
Envenomations 138
Lead Poisoning 139
Mercury Intoxication 140
Toxic Gases 142
Toxic Solvents and Other Chemicals 143
Marine Biotoxins 144
Substance Abuse Disorders 145
Alcohol and Sedatives Abuse and Withdrawal 146
Alcoholism 147
Psychostimulants: Cocaine and Amphetamine 150
Marijuana, Hallucinogens, and PCP 151
Opioids Intoxication and Withdrawal 152
Toxic Leukoencephalopathies 153
Neuroleptic Malignant Syndrome 154
5
Movement Disorders ........................................................................................155Cerebellar ataxias 155
Inherited Ataxias with Identifi able Biochemical Errors 156
Hereditary Ataxias: Autosomal Dominant Inheritance 157
Episodic Ataxias 159
Hereditary Ataxias: Autosomal Recessive Inheritance 160
Hereditary Ataxias: X-Linked Inheritance 164
Congenital Ataxic Disorders 166
Acquired Ataxias: Selected Disorders 168
Neurodegeneration with Brain Iron Accumulation 169
Acanthocytosis-Related Neurologic Disorders 170
Dystonia 171
Parkinson Disease 178
Imaging in Parkinson's Disease and Other Parkinsonisms 180
Levodopa-induced Dyskinesias 183
Tremor in Conditions Other Than PD 187
Progressive Supranuclear Palsy 189
Multiple System Atrophy 191
Autonomic Dysfunction in Neurodegenerative Diseases 193
Corticobasal Degeneration 194
Machado-Joseph Disease 195
Other Parkinsonian Disorders 196
Pathology of Tauopathies 198
Pathology of Synucleinopathies 199
Chorea 200
Huntington Disease 201
Sydenham Disease 202
Restless Leg Syndrome (Ekbom Syndrome) 203
Paroxysmal Dyskinesias 204
Selected Facial Disorders 206
Myoclonus 207
Wilson Disease 208
Tourette Syndrome 209
6
Behavioral Neurology ......................................................................................210Behavioral Neuroanatomy 211
Amnesia 213
Memory Primer 214
Agnosias 215
Apraxias 216
Aphasias 217
Acquired Dyslexia and Peripheral Dyslexia 218
Neglect 219
Delirium or Acute Confusional State 220
Alzheimer's Disease 221
Mild Cognitive Impairment 224
Vascular Dementia 225
Dementia with Lewy Bodies (DLB) 226
Frontotemporal Lobar Degeneration 227
Focal Atrophy Syndromes in Dementias 229
Frontal Assessment Battery (FAB): Offi ce Assessment of Frontal Function 230
7
Neuro-ophthalmology and Neuro-otology ...................................................231Transient Monocular Visual Loss 232
Permanent Monocular Visual Loss 233
Binocular Visual Loss 235
Cerebral Visual Loss 236
Anisocoria 237
Diplopia 239
Disorders of Saccades 241
Nystagmus 242
Selected Cerebellar, Pontine, and Midbrain Oculomotor Syndromes 244
Giant-Cell Arteritis 245
Tolosa-Hunt Syndrome 246
Dizziness: Vertigo and Lightheadedness 247
Benign Paroxysmal Positional Vertigo 248
Meniere's Disease 250
Cerebellar Ataxia with Bilateral Vestibulopathy 251
8
Neural Tube Closure and Other Congenital Malformations .......................252
Demyelinating Diseases, Neuro-oncology and Disorders ofMultiple Sclerosis 253
Posterior Reversible Encephalopathy Syndrome 261
Autoimmune Channelopathies 263
Autoimmune Encephalopathies 264
Brain Tumors 265
Circumscribed Astrocytomas 266
Diffuse Astrocytomas 267
Oligodendrogliomas 268
Ependymal Tumors 269
Neuronal Tumors 270
Mixed Ganglioneuronal Tumors 271
Primitive Neuroectodermal Tumors 272
Meningeal and Mesenchymal Tumors 273
Primary Central Nervous System Lymphoma 274
Pineal Tumors and Keratin-Containing Masses 275
Paraneoplastic Syndromes of the CNS 277
Paraneoplastic Syndromes of the PNS 278
Disorders of Neural Tube Closure and Other Congenital Malformations 280
9
Neuromuscular Disorders ...............................................................................283Brachial Plexopathies 284
Brachial Neuropathies 285
Assessment of Hand Weakness 287
Lumbar Plexus 288
Sacral Plexus 288
Tibial Nerve 289
Peroneal Nerve 289
Assessment of Foot Drop 290
Guillain-Barre Syndrome 291
Chronic Infl ammatory Demyelinating Polyneuropathy 292
Myasthenia Gravis 293
Amyotrophic Lateral Sclerosis (Motor Neuron Disease) 294
Spinal Cord Pathologies per Compartment 297
Neurogenic Bladder 299
Axonal Polyneuropathies 301
Complex Regional Pain Syndrome 303
Neurogenic Orthostatic Hypotension 304
Autosomal Dominant Recurrent Hereditary Neuropathies 305
Paraproteinemic Neuropathies 308
Multiple Myeloma 310
Amyloid Neuropathies 311
Peripheral Nerve Tumors 312
Muscular Dystrophies: X-Linked Dystrophinopathies 313
Limb-Girdle Muscular Dystrophies 314
Congenital Muscular Dystrophies 315
Distal Myopathies 316
Myotonic Dystrophy 317
Facioscapulohumeral Dystrophy 318
Scapuloperoneal Dystrophy 318
Oculopharyngeal Muscular Dystrophy 318
Infl ammatory Myopathies 319
Metabolic Myopathies 321
Episodic Muscle Weakness 322
Familial Periodic Paralysis: Clinical Recognition 323
Progressive or Stable Muscle Weakness 324
Mitochondrial Myopathies 325
Other Myogenic Causes of Cramps and Myalgia 326
Neurogenic Causes of Cramps and Myalgia 327
Congenital Myopathies 328
Malignant Hyperthermia Syndrome 329
Critical Illness-Related Neuropathy and Myopathy 330
10
Psychiatry ...........................................................................................................331Depression 332
Bipolar Disorder 335
Anxiety Disorders 336
Obsessive-Compulsive Disorder 338
Personality Disorders 339
Eating Disorders 340
Schizophrenia and Other Psychoses 341
Delusions 343
Neuroleptic-Induced Movement Disorders 344
Somatoform Disorders 345
Dissociative Disorders 347
Suggested References for Further Reading 348
Index 359