Table of Contents

 

 

 

 

Preface

vii

 

Acknowledgments

ix

 

List of Abbreviations

xi

1

Basics of Metabolism and Pediatric Neurology

1

 

Spectrum of Vitamin B12-Requiring Disorders

2

 

Carbohydrate Metabolism: Inherited Muscle Glycogenosis

3

 

Mitochondrial Metabolism

4

 

Lipid Metabolism

5

 

Purine Metabolism Disorders

6

 

Peroxisomal Disorders

11

 

Lysosomel 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-Deficit Hyperactivity Disorder

30

 

Floppy Infant Syndrome

31

 

Microcephaly

33

 

Macrocephaly

35

 

Glutaric Aciduria Type I

36

 

Biotinidase Deficiency

37

 

Mitochondrial Diseases

38

 

Mitochondria! 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

2

Stroke, Migraine, and Epilepsy

48

 

Stroke

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 Myocionic 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

108

 

Cerebrospinal 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 Leukoencephaiopathy

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

134

 

Manganese 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

155

 

Cerebellar Ataxias

155

 

Inherited Ataxias with Identifiable 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

 

Dystonie

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

210

 

Behavioral 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): Office Assessment of Frontal Function

230

7

Neuro-Ophthalmology and Neuro-Otology

231

 

Transient Monocular Visual Loss

232

 

Permanent Monocular Visual Loss

233

 

Binocular Visual Loss

235

 

Cerebral Visual Loss

236

 

Anisocoria

237

 

Diptopia

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

 

Ménière’s Disease

250

 

Cerebellar Ataxia with Bilateral Vestibulopathy

251

8

Demyelinating Diseases, Neuro-Oncology and Disorders of Neural Tube Closure and Other Congenital Malformations

252

 

Multiple 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

283

 

Brachial 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-Barré Syndrome

291

 

Chronic Inflammatory 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

 

Scapuioperoneal Dystrophy

318

 

Oculopharyngeal Muscular Dystrophy

318

 

Inflammatory 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

331

 

Depression

3

 

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