Swaiman's Pediatric Neurology: Principles and Practice, 2-Volume Set, 5e (Swaiman, Pediatric Neurology)

Cover......Page 1
4-u1.0-B978-1-4377-0435-8..00117-7..DOCPDF......Page 2
Front matter......Page 3
Dedication......Page 5
Copyright......Page 6
Preface to the First Edition......Page 7
Preface to the Fifth Edition......Page 8
Contributors......Page 10
Acknowledgments......Page 17
General Aspects of the Patient's Neurologic History......Page 18
Deep Tendon Reflexes......Page 32
Optic Nerve: Cranial Nerve II......Page 34
Oculomotor, Trochlear, and Abducens Nerves: Cranial Nerves III, IV, and VI......Page 35
Facial Nerve: Cranial Nerve VII......Page 37
Glossopharyngeal and Vagus Nerves: Cranial Nerves IX and X......Page 38
Sensory System......Page 39
Muscle Testing......Page 40
Gait evaluation......Page 49
Muscle Fiber Types......Page 50
Intussusception......Page 212
Mechanisms of Neurologic Injury with Extracorporeal Membrane Oxygenation......Page 459
Genetics......Page 1026
Application of Receiver Operating Characteristic Curves to Determining Thresholds......Page 52
Fragile X Syndrome......Page 509
X-Inactivation......Page 54
Testing......Page 1925
Developmental Reflexes......Page 55
Reflex Anoxic Seizures......Page 58
General Considerations......Page 59
Observation......Page 60
Developmental Reflexes......Page 62
Generalized Epilepsy with Febrile Seizures Plus (GEFS+)......Page 64
Hypogonadotropic Hypogonadism Associated with Multiple Hypothalamic/Pituitary Hormone Deficiencies......Page 1397
Anatomy and Embryology......Page 66
Clinical Laboratory Tests......Page 1450
Spinal and bulbar muscular atrophy (kennedy disease)......Page 1737
Nocturnal Polysomnography......Page 769
Moyamoya disease and syndrome......Page 1655
Clinical Features......Page 151
Acquired Brain Injury......Page 2134
Mumps......Page 2119
Visual Sequelae......Page 1095
Laboratory tests......Page 381
Pathology......Page 77
Molecular Pathogenesis......Page 1819
Inheritance Patterns......Page 78
Benign Familial Infantile Seizures......Page 977
Clinical Characteristics, Clinical Laboratory Tests, and Diagnosis......Page 278
Dystrophin-Glycoprotein Complex......Page 1718
Sarcoglycans......Page 195
Experimental Therapeutics in Myotonic Dystrophy......Page 1920
Cockayne's Syndrome and Trichothiodystrophy......Page 1277
Progression of Neonatal Brain Injury......Page 84
Early Post-Traumatic Seizures......Page 1377
Sequelae of Inflicted Head Injury......Page 85
Neuroimaging......Page 1378
Sickle cell disease......Page 1283
Pathology......Page 742
Muscle biopsy......Page 1473
Water Intoxication, Hyponatremia, and Brain Edema......Page 1183
Chronic Inflammatory Demyelinating Polyradiculoneuropathy......Page 86
Prenatal Assessment of Normal Development of the Corpus Callosum......Page 88
Reorganization of Neuronal Connections......Page 203
Treatment Decision Support......Page 89
Interoperability......Page 2169
Type iii spinal muscular atrophy......Page 1150
Management......Page 452
Gene-Diet Interactions in Neural Tube Defects: Role of Metabolism of Folic Acid and Other Nutrients......Page 92
Epidemiology......Page 558
Functional Hypogonadotropic Hypogonadism......Page 503
Arteriopathies......Page 93
Increased Excitatory Neurotransmission......Page 379
Pulmonary involvement......Page 1827
Overlap syndromes......Page 1117
Dynamic Changes in Cerebral Metabolism......Page 1339
Phenytoin......Page 1069
Cerebral Blood Flow: Hyperemia? Hypoperfusion?......Page 745
Rubella......Page 413
Acute Renal Failure......Page 713
Computed Tomography......Page 95
Treatment......Page 1912
Disorders of Receptive and Expressive Language......Page 360
Intracranial Hypertension Related to Compromise of Autoregulation......Page 1439
Clinical Biochemistry......Page 1309
Hyperkalemic Periodic Paralysis......Page 97
Specific Agents......Page 1130
Eyelid abnormalities: ptosis......Page 98
Neurometabolic retinal dysfunction......Page 99
Pseudopapilledema......Page 100
Optic atrophy......Page 101
Cerebral Vision Impairment......Page 102
Transient Episodic Vision Loss in Children......Page 103
Astrocytoma......Page 106
Pathology......Page 1178
Orthopedic Surgery......Page 611
Impairment of Consciousness with Activated Mental State......Page 1068
Motor Unit......Page 108
Seizure Classification: International League Against Epilepsy......Page 1001
Classification of Hearing Losses......Page 110
Other Mendelian Focal Epilepsies......Page 621
Juvenile Idiopathic Arthritis (Chronic Arthropathies)......Page 772
Campylobacter jejuni......Page 1315
Delayed Recovery of Consciousness......Page 1641
Uremic Encephalopathy......Page 111
Blood-brain barrier disruption......Page 1409
Duration of Observation Periods......Page 112
Pathogenesis......Page 1727
Combination Vaccines and Additives......Page 534
Storage disorders......Page 1411
Hepatitis......Page 777
Initial Assessment, Imaging and Surgical Intervention......Page 1442
Etiology......Page 115
Lead......Page 726
Thromboembolic cardiogenic stroke......Page 2021
Clinical features......Page 1519
Treatment......Page 1870
Summary......Page 925
Diabetes Mellitus......Page 119
Cerebroretinal Microangiopathy with Calcifications and Cysts......Page 1290
Lightning and Electrical Injuries......Page 120
Positron Emission Tomography......Page 648
Clinical Manifestations......Page 1689
Volatile Solvents and Propellants......Page 1201
Neurologic Prognosis After Cardiac Arrest......Page 121
Mitochondrial fatty acid oxidation defects......Page 1140
22q11.2 Deletion Syndrome......Page 568
Types of Hearing Tests......Page 122
Formal behavioral audiometry......Page 123
Acoustic Immittance and Middle Ear Reflexes......Page 124
Electrocochleography and Cochlear Potentials......Page 125
Brainstem Auditory-Evoked Responses/Auditory Brainstem Responses (BAER/ABR)......Page 126
Miscellaneous Blood, Urine, and Other Tests......Page 127
Psychologic Evaluation......Page 128
Brain, Language, and Intellectual Consequences of Auditory Deprivation......Page 129
Cochlear Implants......Page 130
Education......Page 131
Consequences of Hearing Loss for Overall Development......Page 133
Conflicts of Interest......Page 134
Introduction......Page 135
Resolution of Temporary Dysfunction......Page 2125
Delayed or Arrested Puberty......Page 136
Incidence......Page 393
Neural Systems for Reading......Page 704
Pathogenesis and Pathologies......Page 138
Clinical Features......Page 454
Developmental Abnormalities......Page 1249
Tourette's Syndrome/Tourette's Disorder......Page 865
Clinical Features......Page 1338
Autoimmune Myasthenia Gravis......Page 1797
Intestinal Pseudo-Obstruction......Page 353
Recurrent Vertigo......Page 140
Dystonic Postures......Page 980
Clinical laboratory tests......Page 1829
Neuromuscular Transmission......Page 1721
Varicella......Page 2120
Syringomyelia......Page 141
Gamma Efferent System......Page 562
Complications......Page 488
Acute encephalopathy......Page 142
Clinical Features of Autonomic Dysfunction......Page 143
Clinical Disorders of Taste and Smell......Page 144
Fucosidosis......Page 148
Perspectives......Page 794
Demographics and Epidemiology of MS in Children......Page 559
Bell's palsy......Page 1826
Rabies Vaccine......Page 876
Autosomal-Dominant and Autosomal-Recessive Myotonia Congenita......Page 150
Antibodies......Page 275
Tonic Postures......Page 412
Renal Diseases Secondarily Affecting the Nervous System......Page 153
Differential Diagnosis......Page 990
Sudden Infant Death Syndrome......Page 1308
Hypothalamic Disorders of Appetite Regulation and Energy Balance......Page 1946
Acknowledgments......Page 156
Spinal Fluid Examination......Page 157
Type i spinal muscular atrophy......Page 1730
Epilepsies of the Neonatal Period......Page 975
Severe Myoclonic Epilepsy in Infancy......Page 160
Duchenne Muscular Dystrophy......Page 295
Types of Vaccines......Page 307
Vaccines Composed of Whole-Killed Organisms......Page 1717
Subacute Sensory Neuronopathy......Page 163
Glucose......Page 164
Research......Page 1188
Intermediate Filaments......Page 166
Pediatric Neuroimaging......Page 168
Disruption of Brain Programming......Page 277
Anatomy......Page 734
Magnetic Resonance Imaging......Page 174
Diagnostic Evaluation......Page 766
Related Morbidity and Mortality......Page 1040
Neurotransmitters......Page 1275
Arterial Circulation: Anatomy and Vascular Patterns......Page 1644
Clinical Features......Page 918
Pathogenesis......Page 1337
Magnetic Source Imaging......Page 198
Management......Page 1857
Treatment......Page 1866
Gastroesophageal Reflux......Page 1259
ALG12-CDG (Ig)......Page 286
Pediatric Neurophysiologic Evaluation......Page 202
Embryologic Development......Page 1951
Assessing Causality......Page 515
Structure and Function of DNA......Page 281
Comprehensive Pediatric Rehabilitation Programs......Page 204
Dysferlin......Page 206
Opsoclonus-Myoclonus-Ataxia Syndrome......Page 1187
Neurologic Complications of Congenital Heart Disease Prior to Anatomic Intervention......Page 209
Audiological Disorders......Page 427
Spastic Hemiplegia......Page 1053
Behavioral Disturbances......Page 530
Biochemistry and molecular genetics......Page 218
Physiology......Page 2140
Magnetic Resonance Imaging......Page 219
Localizing Symptoms......Page 220
Clinical Features......Page 994
Contractures......Page 221
Tuberous Sclerosis Complex......Page 752
Molecular Genetics......Page 1729
Photic Stimulation......Page 222
Drowsy Patterns......Page 223
Prognosis......Page 313
Mumps, Measles, and Rubella Vaccine and Autism......Page 2122
Postoperative Morbidity......Page 1599
Intracranial Hemorrhage......Page 1008
Mycophenolate Mofetil......Page 225
Clinical Characteristics, Clinical Laboratory Tests, and Diagnosis......Page 1547
White-Matter Contusional Tears......Page 1267
Transcranial magnetic stimulation......Page 900
Adult Cerebral Form of Adrenoleukodystrophy......Page 416
Cocaine......Page 227
Palliative Care......Page 797
Type III......Page 1768
Drugs......Page 2054
Neurologic Abnormalities in Primary Biliary Cirrhosis......Page 2086
Spikes and Sharp Waves......Page 233
Antidepressants......Page 234
Hypsarrhythmia......Page 235
Ribose-5-phosphate isomerase deficiency......Page 1142
X-linked muscular dystrophies......Page 236
Adverse Drug Reactions to Antiepileptic Drugs......Page 906
Pathology......Page 237
Laboratory Testing......Page 816
Multiple Independent Spike Foci......Page 238
Limb-girdle muscular dystrophy 1a (myotilinopathy)......Page 1843
Treatment of Psychiatric Disorders......Page 819
Genetics and Pathophysiology......Page 1145
Vitamin A intoxication......Page 1295
Febrile Seizures......Page 239
Determination of Brain Death......Page 240
Outcome......Page 1674
Clinical Laboratory Tests......Page 1695
Nitrofurantoin......Page 1209
Degenerative Diseases......Page 241
Acquired Arteriopathies......Page 1678
Pediatric Neurointensive Care......Page 243
Facioscapulohumeral Muscular Dystrophy......Page 574
Tick-Borne Flaviviruses......Page 244
Evoked Potential Analysis......Page 245
Auditory-Evoked Potentials......Page 246
Electroretinography......Page 248
Somatosensory-Evoked Potentials......Page 249
Event-Related Potentials......Page 250
Introduction......Page 251
Laboratory Testing......Page 253
Tic Phenomenology......Page 1107
Asperger's Disorder......Page 256
The Enteric Nervous System......Page 2069
Influenza Virus Vaccine......Page 1024
Guillain-barré syndrome......Page 269
Brain Stimulation and Adaptive Plasticity......Page 258
Topiramate......Page 892
Clinical Features and Management of Hyperprolactinemia......Page 259
Clinical Features......Page 260
Mechanisms Underlying Functional Recovery in the Nervous System......Page 261
Stimulants......Page 914
Pelizaeus-Merzbacher Disease......Page 1272
Ethics of Care......Page 1375
Management......Page 1541
Cerebral Palsy......Page 270
Other neonatal risk factors......Page 273
Physiology of Absence Epilepsy......Page 274
Symptoms and Signs in Older Children......Page 485
Paraneoplastic Encephalitides......Page 1640
Abnormalities of Galactose Metabolism......Page 616
References......Page 0
Orthostatic Intolerance/Postural Tachycardia Syndrome......Page 954
Individual Inflammatory and Cell Signaling Molecules......Page 279
Downregulation of Astrocytic Glutamate Transporters......Page 610
Duties as a Neurologist......Page 1401
Selective Dorsal Rhizotomy......Page 1491
Febrile Seizures and Subsequent Epilepsy......Page 282
Ion Channel Configuration Favors Depolarization in Early Life......Page 284
Dysphagia......Page 2006
Paroxysmal Depolarization Shift......Page 965
SOX10-Associated Disorders......Page 285
Genetics......Page 287
Chromosomal disorders......Page 1961
Clinical features......Page 289
Smallpox......Page 1340
Escherichia coli......Page 773
Infant Botulism......Page 291
Epilepsy of Infancy with Migrating Focal Seizures......Page 1004
Plexopathies......Page 796
Clinical Characteristics......Page 293
Epidemiology......Page 823
Hypothalamic/Pituitary Disorders of Pubertal Development......Page 496
Clinical Features......Page 1273
Volume of Distribution......Page 297
Citrullinemia Type II or Citrin Deficiency......Page 421
Disorders Associated with Gastrointestinal Disease......Page 308
Ethical Responsibilities......Page 1151
Magnetic resonance spectroscopy imaging......Page 298
Disorders of Prolactin Secretion......Page 644
Anatomic Gastrointestinal Disorders......Page 1451
Mortality in Children with Epilepsy......Page 958
Perfusion Imaging......Page 301
Pulmonary Issues......Page 2136
Enteric Infections......Page 896
Clinical features......Page 1867
Other Viral Myositides......Page 306
Hereditary sensory and autonomic neuropathies......Page 919
Galactokinase Deficiency......Page 481
Neurologic Conditions Associated with Congenital Heart Disease......Page 1590
Introduction......Page 741
Function......Page 309
Prader-Willi Syndrome......Page 310
Lamotrigine......Page 1070
Neuroprotection......Page 312
Cerebrospinal Fluid......Page 1516
Types of Pain......Page 1927
Turcot's Syndrome......Page 2079
Introduction......Page 1808
Subarachnoid Hemorrhage......Page 1073
Management/Treatment......Page 314
Minimal hepatic encephalopathy......Page 315
Laboratory data......Page 677
Spinal Epidermoid Tumor......Page 681
Congenital Indifference to Pain......Page 1389
Management of Specific Tumors......Page 317
Laboratory Evaluation......Page 1814
Epidural hematomas......Page 318
Hepatorenal Syndrome......Page 927
Progressive Hepatocerebral Disease......Page 2029
Connective Tissue Disorders......Page 322
Selective Tubular Dysfunction......Page 1842
Calcium-Mediated Injury......Page 325
Benign Epilepsy with Centrotemporal Spikes......Page 505
Infectious/inflammatory arteriopathy......Page 326
Brachial Plexus......Page 328
Laboratory Features......Page 620
Mutations in proinflammatory cytokines......Page 330
Acetazolamide-Responsive Sodium Channel Myotonia and Myotonia Fluctuans......Page 1253
Neuroimaging......Page 331
Neurologic Manifestations......Page 1685
Ataxia-Telangiectasia (AT; Louis-Bar Syndrome - OMIM 208900)*......Page 1185
Norepinephrine Transporter Deficiency......Page 1963
Chromosome Structure......Page 334
Congenital Uremic Encephalopathy......Page 1197
Zonisamide......Page 406
Whipple's Disease......Page 1457
Clinical Characteristics, Clinical Laboratory Tests, and Diagnosis......Page 336
Clinical Features......Page 563
Migraine with aura......Page 385
Pathophysiology......Page 337
Sydenham's chorea......Page 338
Epidemiology......Page 339
Hyperkinetic Movement Disorders......Page 1666
Fragile X Syndrome......Page 811
Hypoxia-Ischemia......Page 340
Acyl-CoA Oxidase Deficiency......Page 341
Amyloidosis......Page 731
Barbiturates......Page 342
Prevention......Page 343
Magnetic Resonance Imaging......Page 344
Glucose Homeostasis......Page 345
Hereditary Familial Episodic Ataxia Type 1......Page 346
Valproate......Page 1844
Acknowledgments......Page 347
Carbamazepine......Page 2066
Perinatal Metabolic Encephalopathies......Page 348
Duties as a Physician......Page 2158
Dissection and other physical injury......Page 352
Childhood Absence Epilepsy (Pyknolepsy)......Page 354
Pharmacology......Page 357
Summary......Page 974
Prognosis of postoperative seizures......Page 361
Rehabilitation Strategies......Page 1380
Sulfite Oxidase and Molybdenum Co-factor Deficiency......Page 363
Dopamine--Hydroxylase Deficiency......Page 364
Insecticides......Page 1988
Microbiologic Evaluation......Page 1495
Lindane......Page 365
Measurements of Cerebral Perfusion......Page 366
Genetic Testing......Page 465
Limb Girdle Dystrophy 1B: Lamin A/C......Page 1074
Limb-Girdle Muscular Dystrophy 2A: Calpainopathy......Page 1665
Single-Nucleotide Polymorphisms......Page 367
Complications of Transplantation......Page 2025
Definitions of Palliative Care......Page 2147
Meningismus......Page 368
Relationships to Other Neurologic Disorders......Page 370
Familial Dysautonomia......Page 1959
Anatomy and Embryology......Page 373
Clinical Discussions and Groups......Page 2165
Benign Familial Neonatal-Infantile Seizures......Page 976
Epidemiology and Pathogenesis......Page 376
Blood tests......Page 1823
Gene-Environment Interactions Influencing Neural Tube Defects......Page 377
Clinical Features......Page 768
Management......Page 641
Inflammatory Bowel Disease......Page 382
Diagnostic Criteria......Page 1456
Autonomic Dysreflexia......Page 1745
Laboratory Features......Page 1868
Biologic Toxins......Page 1987
Complications of Chronic Renal Failure......Page 2043
Trauma-Related Anterior Horn Cell Disease......Page 657
Etiology and Prognosis......Page 995
Polyneuropathy and myopathy......Page 1913
Conus medullaris syndrome......Page 386
Cyclophosphamide......Page 387
Diagnostic Testing......Page 1325
Milder Forms of Encephalopathy......Page 2049
Digital Subtraction Angiography......Page 1483
Leukoencephalopathies with Brainstem, Cerebellum and Spinal Cord Involvement......Page 468
Clinical Presentation......Page 390
Clinical Features......Page 1813
Reye's Syndrome......Page 783
The Anterior Horn Cells of Spinal Cord......Page 959
Implications of the Phonologic Model of Dyslexia......Page 394
Spirituality......Page 1824
Excitatory Synaptic Transmission......Page 398
Acute Presentation......Page 1376
Ethical Problems......Page 1052
Amyotrophic lateral sclerosis......Page 1738
Prothrombotic and Hematological Disorders......Page 400
Malabsorption Syndromes......Page 2074
Pathogenesis......Page 869
Other Tests......Page 749
Management......Page 405
Autoimmune Disorder......Page 1262
Clinical Assessment......Page 1859
Fabry´s Disease......Page 407
Epidemiology, Microbiology, and Pathology......Page 408
Vasovagal Syncope......Page 888
Defects of the Respiratory Chain......Page 410
Clinical Characteristics......Page 411
Acquired brain lesions in the fetus with congenital heart disease......Page 1093
Motor Unit System......Page 1725
Muscle biopsy......Page 795
Dystonic cerebral palsy......Page 510
Haemophilus influenzae Type b......Page 1759
Common Toxidromes......Page 1983
Pauciarticular Juvenile Idiopathic Arthritis (Oligoarthritis)......Page 1687
Complications of Dialysis......Page 587
Peripheral Neuromuscular Injury......Page 2020
Guillain-Barré Syndrome with Central Nervous System Manifestations......Page 415
Treatment......Page 1929
Cannabis......Page 1992
Cerebellar Atrophy......Page 417
Type IV......Page 1418
Differential Diagnosis......Page 418
Persistent Vegetative State......Page 419
Environment for Death and Dying......Page 2150
Etiology......Page 886
Glycogenoses......Page 1751
Aromatic l-Amino Acid Decarboxylase or Dopa-Decarboxylase Deficiency......Page 424
Epilepsy......Page 425
Ophthalmological Disorders......Page 426
Adrenocorticotropic Hormone Deficiency......Page 1922
Primary Microcephaly......Page 430
Growth Hormone Excess......Page 433
Electrophysiologic Testing......Page 1408
Genetic Counseling......Page 434
Opioids......Page 2143
Scoliosis......Page 439
Antiplatelet therapy......Page 1663
Clinical Features......Page 1441
Neuronal Damage......Page 445
Scedosporium spp. Infection......Page 446
Neonatal-Onset Multisystem Inflammatory Disease or Chronic Infantile Neurologic Cutaneous and Articular Syndrome......Page 1688
Spinal Epidural Abscess......Page 1458
Other Megalencephaly/Macrocephaly Syndromes......Page 448
Embryology......Page 450
Recent Progress......Page 451
Definition......Page 887
Treatment and Prognosis......Page 718
Imaging Studies......Page 458
Midline Tumors......Page 460
Hypothalamic/Pituitary Disorders of Water Balance......Page 645
Neurotransmitter deficiencies......Page 461
Porphyria......Page 1198
Apnea Testing......Page 1007
Thimerosal-Containing Vaccines and Developmental Disorders of Childhood......Page 1496
Thyroid Disorders......Page 1164
Strangulation Injury......Page 1414
Scrub Typhus......Page 1551
Brain Imaging......Page 467
Nitrous Oxide......Page 1137
Clinical presentation and diagnosis......Page 1605
Delta-Sarcoglycanopathy (Limb-Girdle Dystrophy 2F)......Page 473
Opiate Antagonists......Page 571
Pathology......Page 475
Brain Imaging......Page 476
Lupus aseptic meningitis......Page 786
Hydrocephalus......Page 480
Movement Disorders After Cardiac Surgery......Page 2018
Sedation......Page 492
Biochemical and molecular basis......Page 588
Changing Techniques/Delivery......Page 493
Solvents......Page 1135
Arsenic......Page 629
Infection......Page 926
Introduction......Page 495
Spinal Cord......Page 498
Associated manifestations......Page 607
Lambert-Eaton Myasthenic Syndrome......Page 502
Episodic Gastrointestinal Disease......Page 504
Management......Page 506
Defects of the Krebs Cycle......Page 507
Introduction......Page 511
Introduction......Page 1268
Prenatal Assessment of Normal Development of the Cortex......Page 513
Dissemination of Original Research......Page 873
Coccidioidomycosis......Page 517
Myotonic Dystrophy Type 2 (Formerly Proximal Myotonic Myopathy)......Page 863
Muscle biopsy......Page 1908
Loss of Autoregulation Leading to Brain Tamponade......Page 528
Cyclic Vomiting Syndrome and Recurrent Abdominal Pain......Page 2070
Brain Anomalies of Fetal Onset Associated with CHD......Page 521
Epidemiology, Microbiology, and Pathology......Page 1542
Preoperative Neurologic Complications......Page 877
Prenatal Diagnosis of Molar Tooth-Related Syndromes......Page 524
Introduction to Genetics......Page 525
Clinical Management......Page 916
Education......Page 1918
Duties as a Pediatrician......Page 2159
Pathology......Page 529
Clinical Characteristics, Clinical Laboratory Tests, and Diagnosis......Page 1543
Similar Thresholds Affect Outcome in Other Forms of Severe Brain Injury......Page 1438
Alpha B-Crystallinopathy......Page 532
Epidemiology, Microbiology, and Pathology......Page 625
Other Gastrointestinal Diseases......Page 2078
Cervicomedullary syndrome......Page 1131
Thallium......Page 535
Hereditary Sensory and Autonomic Neuropathies......Page 536
Gamma-Hydroxybutyrate......Page 630
Restriction Enzymes......Page 538
Treatment......Page 1553
Sanger sequencing......Page 539
General Management Principles for Severe Pediatric TBI......Page 540
Antipsychotic Agents (Neuroleptics)......Page 1525
Single-Base Extension......Page 541
Lithium......Page 732
Vitamin A deficiency......Page 593
Post-Cardiac Arrest Brain Injury - Potential Therapies......Page 1427
Thiamine deficiency......Page 2097
Pathology......Page 542
Diagnosis......Page 1528
Biochemistry......Page 543
Deletions and Duplications......Page 544
Kidney Stones......Page 2067
Clinical features......Page 545
Clinical Features......Page 1211
Arteriovenous malformations......Page 546
Treatment......Page 1700
Aneurysms......Page 1683
Clinical description......Page 547
Frameshift Mutations......Page 548
Patterns of Inheritance......Page 549
X-Linked Inheritance......Page 550
Genomic Imprinting......Page 551
Mitochondrial Inheritance......Page 552
Prenatal Diagnosis......Page 553
Genetics and Medicine......Page 554
Chromosomes and Chromosomal Abnormalities......Page 555
Pharmacology......Page 862
Blood Supply......Page 556
Numerical Abnormalities......Page 557
Spastic Quadriplegia......Page 561
Bandlike Intracranial Calcification with Simplified Gyration and Polymicrogyria......Page 1158
Physical Examination Findings......Page 646
Hereditary Motor and Sensory Neuropathy Type II......Page 1765
Plasmapheresis......Page 1810
Plasmapheresis......Page 565
Treatment......Page 566
Magnetic Resonance Spectroscopy......Page 809
Other treatments......Page 1036
Prader-Willi and Angelman's Syndromes......Page 569
Management of the Childhood Polyneuropathies......Page 632
Neuropsychiatric lupus......Page 572
Treatment and outcome......Page 785
Pathophysiology......Page 573
Genetics......Page 1222
Clinical features......Page 1873
Niacin (Vitamin B3)......Page 2099
Treatment and Outcome......Page 1505
Historical Background......Page 575
Aminoacidemias and Organic Acidemias......Page 576
Laboratory Approaches to Diagnosis......Page 577
Inheritance and Genetic Counseling......Page 578
Historical Overview......Page 716
Retinopathies......Page 1638
Polymyositis......Page 1910
Clinical Features......Page 583
Rotavirus......Page 584
Anti-AChR antibodies......Page 1783
Classic maple syrup urine disease......Page 585
Neuropathic Pain......Page 2145
Etiology......Page 1031
Hypokalemic Periodic Paralysis......Page 590
Prognosis......Page 1791
Biochemistry......Page 901
Correct Acid-Base and Electrolyte Imbalance......Page 1326
Neurologic Abnormalities Associated with Liver Transplantation......Page 2085
Gene Therapy......Page 1522
Medulloblastoma......Page 649
Amebic Infections of the Central Nervous System......Page 1485
Molecular pathogenesis......Page 592
Monitoring of the Comatose Patient......Page 1327
Maple syrup urine disease......Page 1294
Diphenhydramine......Page 930
Anticoagulation......Page 1673
Tacrolimus (FK-506)......Page 594
Diagnosis, Treatment, And Outcome......Page 1529
Treatment......Page 1699
Treatment......Page 596
Botulism......Page 1365
Steroids......Page 597
Pyridoxine-Dependent Epilepsy......Page 1617
Aromatic L-Amino Acid Decarboxylase Deficiency......Page 1297
Cobalamin deficiency......Page 598
Management and treatment......Page 791
Involvement of fatty acid oxidation-dependent tissues......Page 1852
3-Methylglutaconic Aciduria......Page 599
Adverse Effects......Page 937
Diagnosis......Page 600
Neurocytoma......Page 938
Adverse effects......Page 939
Diagnosis......Page 601
Treatment......Page 602
Takayasu's Arteritis......Page 1707
3-Hydroxyisobutyric Aciduria......Page 603
Mevalonate Kinase Deficiency......Page 604
Classification of Seizures......Page 605
Clinical Description of Urea Cycle Disorders......Page 606
Common Morality and Natural Law......Page 2155
Argininemia......Page 608
Facial Nerve Paralysis (Bell's Palsy)......Page 609
Personhood......Page 1123
Other Structural Congenital Myopathies......Page 613
Neurologic Examination......Page 614
Hypokalemia......Page 1045
Childhood......Page 615
Cognitive Function in Benign Childhood Epilepsy Syndromes......Page 1119
Clinical Characteristics, Clinical Laboratory Tests, and Diagnosis......Page 984
Arterial Ischemic Stroke......Page 1046
Anatomy......Page 917
Mechanical Factors......Page 1042
Conflict of Interest......Page 1825
Pathology......Page 622
Biochemistry......Page 623
Clinical Laboratory Tests......Page 1804
Acid α-Glucosidase (GAA, Acid Maltase) Deficiency, Infantile Type (Pompe's Disease, Idiopathic Generalized Glycogenosis, Glycog......Page 626
Clinical Manifestations of Raised Intracranial Pressure......Page 923
Clinical Characteristics......Page 627
Clinical Laboratory Evaluation......Page 664
Genetics......Page 1767
Late Postoperative Stroke......Page 1965
Transition in Goals of Care......Page 2148
The Role of the Basal Ganglia in Movement Disorders......Page 631
Management and outcome......Page 1216
Support During Dying......Page 904
Phosphoglycerate Mutase Deficiency......Page 813
Disorders Associated with Vitamin Deficiencies or Excesses......Page 2095
Cluster Headache......Page 633
Atropine and Related Alkaloids......Page 634
Clinical presentation and diagnosis......Page 635
Supportive Care......Page 1360
Phosphoglucomutase Deficiency (Thomson's Disease)......Page 636
Conclusions......Page 637
Breath-Holding Spells......Page 638
Management......Page 639
Autism and Autistic Spectrum Disorders......Page 640
Abnormal Permeability to Calcium and Chronic Increase of Intracellular Calcium......Page 1111
Motor Neuron Diseases of Unknown Etiology......Page 1740
Hypothalamic/Pituitary Disorders of Statural Growth......Page 1945
Clinical Features......Page 1924
Diagnosis: Neuroimaging......Page 1124
Clinical Characteristics, Clinical Laboratory Tests, and Diagnosis......Page 1544
Nerve Agents......Page 1029
Pathogenesis......Page 996
Fungal Myositides......Page 1914
Inhibitors of Receptor Tyrosine Kinases and Cellular Signal Transduction......Page 1603
Advances in Imaging......Page 810
Summary......Page 650
Pathology......Page 651
Polyneuritis Cranialis......Page 1785
Treatment......Page 1412
Metachromatic Leukodystrophy (Sulfatide Lipidosis)......Page 673
Long-Term Neurodevelopmental Dysfunction......Page 676
Chronic Inflammatory Demyelinating Polyneuropathy......Page 1552
Molecular Pathogenesis......Page 682
Initiation and titration of dose......Page 683
Communication......Page 684
Phosphoglycerate Kinase Deficiency......Page 685
Disorders of oxidative phosphorylation......Page 1872
Monitoring of Drug Concentrations......Page 686
Interpretation of ``Optimal Therapeutic Ranges´´......Page 687
Sialidosis......Page 688
Anatomic and Biochemical Effects of Undernutrition......Page 689
Mucolipidosis Type I (Sialidosis Type II)......Page 690
Clinical Features......Page 1932
Danon´s Disease......Page 694
Sialic Acid Storage Disorders......Page 695
Cystinosis......Page 696
Spinocerebellar Ataxia Type 20 (SCA20 - OMIM 608687)......Page 697
Introduction......Page 700
Histopathologic Disturbances......Page 703
Orthostatic Hypotension......Page 1856
Intrathecal Baclofen Therapy......Page 708
Neuropsychologic involvement......Page 1828
Other pseudo-obstruction syndromes......Page 710
Fiber Type Disproportion Myopathy......Page 1437
Management......Page 714
Clinical Features......Page 1067
Organ Donation......Page 719
Adrenocorticotropic Excess......Page 1044
Developmental Differences......Page 720
Posterior Reversible Encephalopathy Syndrome......Page 1287
Vacuolar Myopathy......Page 1860
Treatment......Page 724
Identification of Cause......Page 898
Laboratory Tests......Page 727
Pathophysiology......Page 729
Contiguous Deletion of the XALD Gene......Page 730
Ethanol......Page 1420
Human Herpesviruses Types 6, 7, and 8......Page 1218
Subdural effusion......Page 1390
Barriers to Palliative Care......Page 1609
Theophylline......Page 1996
Current and Future Outlook......Page 733
Morphology......Page 736
Primary injury phase......Page 737
Treatment......Page 1909
Neurologic Examination......Page 1973
Clinical Features......Page 1191
Management......Page 1179
The Neurologic Outcome of Children Undergoing Extracorporeal Membrane Oxygenation......Page 1830
Experimental therapies......Page 1832
Intrauterine and Perinatal Viral Infections......Page 744
Laboratory data......Page 750
Meningococcal Conjugate Vaccine......Page 751
Fear of Side Effects and Effective Antiepileptic Drug Use......Page 1126
Clinical Characteristics......Page 757
Mercury......Page 758
Arteriopathic stroke......Page 2022
Limb-Girdle Muscular Dystrophy 1C: Caveolinopathy......Page 1837
Cognitive and behavioral abnormalities......Page 1032
Molecular Pathogenesis......Page 1838
Management......Page 760
Epidermal Nevus Syndrome......Page 761
Neuropathology......Page 762
Health Care Decision-Making......Page 902
Hepatolenticular Degeneration: Wilson's Disease......Page 763
Wyburn-Mason Syndrome (Retinocephalic Angiomatosis)......Page 764
Genetics......Page 770
Hypertensive Encephalopathy......Page 2017
Risk Factors......Page 1057
Kidney......Page 776
Dialysis-Associated Seizures......Page 1127
Clinical Description......Page 778
Genetics......Page 779
Laboratory Findings......Page 780
Epilepsy......Page 1499
Beta-Sarcoglycanopathy (Limb-Girdle Muscular Dystrophy 2E)......Page 1841
Assessment and Treatment of Symptoms......Page 1354
Management and treatment......Page 784
Krabbe's Disease (Globoid Cell Leukodystrophy)......Page 1772
Myopathy......Page 787
Pathophysiology......Page 931
Juvenile Neuronal Ceroid-Lipofuscinosis or Juvenile Batten's Disease......Page 788
Molecular genetics......Page 1563
Laboratory data......Page 1296
Pathology......Page 789
CLN9-Deficient Juvenile-like Variant......Page 790
Clinical Features......Page 792
The Central Autonomic Nervous System......Page 793
Pathophysiology......Page 1970
Intraoperative Techniques......Page 1598
Supraspinal Changes......Page 798
Postsynaptic Defects......Page 1815
Biochemical Markers......Page 1421
Stimulants......Page 799
Laboratory data......Page 1355
Nephropathic Cystinosis......Page 1558
Thrombolysis......Page 1672
Biochemistry......Page 800
Treatment of Neurologic Manifestations......Page 1561
Biotinidase deficiency......Page 801
Definitions......Page 802
Acute Rheumatic Fever......Page 803
Antihistamines......Page 812
Mechanisms of Thromboembolism......Page 1668
Discussions with Family Members and Staff......Page 1487
Physical Examination......Page 814
Feeding and Nutritional Disorders......Page 820
Outcome and Prognosis......Page 821
Acknowledgments......Page 822
Pathogenesis: Mechanisms of Spinal Cord Injury......Page 1335
Developmental Language Disorders......Page 852
Juvenile Myoclonic Epilepsy......Page 854
Growth Hormone Deficiency......Page 856
Prematurity......Page 857
Lexical Syntactic Syndrome......Page 858
Zygomycosis......Page 859
Definition......Page 861
Mechanism of Action......Page 1090
Diagnosis of Attention-Deficit Hyperactivity Disorder......Page 870
Authoritative Narrative Content......Page 872
Mechanisms of Infarction and Developmental Brain Injury......Page 1433
Autistic Regression and Childhood Disintegrative Disorder......Page 889
Multiple sclerosis......Page 875
Cerebral Blood Flow and Metabolism After Resuscitation......Page 881
Historical Background......Page 1097
Neurologic Examination......Page 1977
Calcifying Leukoencephalopathies......Page 882
Anterior Spinal Artery Occlusion......Page 883
Complementary and Alternative Medications......Page 884
Epidemiology......Page 890
Hirschsprung's disease......Page 957
Cardiac Risk Factors......Page 1592
Hypothalamic/Pituitary Disorders of Thyroid Function......Page 1314
Other Ancillary Testing......Page 1056
Genetics......Page 897
Diabetes Insipidus......Page 1948
Types of Pain Medications......Page 1320
Malnutrition......Page 1345
Clinical Manifestations......Page 1664
Epigenetics......Page 903
Benzodiazepines......Page 905
Clinical Assessment......Page 1391
Complementary and Alternative Medicine......Page 907
Disclaimer......Page 911
Introduction......Page 912
History......Page 1149
Hereditary Sensory and Autonomic Neuropathy Type IV (Congenital Insensitivity to Pain with Anhidrosis)......Page 920
Clinical Management......Page 921
Treatment......Page 1156
History......Page 922
Cardiac management......Page 1834
Mononeuropathies......Page 1161
Electroencephalographic Findings......Page 924
Inborn Errors of Metabolism......Page 2028
Systemic Lupus Erythematosus......Page 1608
Antidepressants and risk for suicidal behavior......Page 928
Seizures......Page 929
Ataxia-Telangiectasia......Page 1219
Wound Botulism......Page 1817
OKT3......Page 1997
Treatment......Page 1775
Riboflavin-Responsive Isolated Complex II Deficiency......Page 1147
Apicomplexan Infections of the Central Nervous System......Page 1562
Pharmacology......Page 932
Babesiosis......Page 933
Measles Virus......Page 934
Clinical Applications......Page 935
Acknowledgments......Page 1299
Other Studies......Page 1704
Tocopherol (Vitamin E)......Page 2107
Vitamin K......Page 2109
Clinical applications......Page 940
Common Characteristics of Typical Antipsychotics......Page 941
Clinical Applications......Page 943
Common Characteristics......Page 944
Clinical Applications......Page 945
Clinical Management......Page 946
Clinical Applications......Page 947
Clinical Management......Page 948
Pharmacology......Page 949
Conclusion......Page 950
Pediatric Epilepsy......Page 951
Neuropathology......Page 1258
Second-line therapies......Page 1307
Depolarizing Conductances......Page 960
Development of Ionic Channels and Membrane Properties......Page 968
Transverse Myelitis......Page 1039
Mechanisms of Late Recovery......Page 1301
Clinical Features......Page 978
History......Page 981
Desminopathies......Page 1858
Epidemiology of Pediatric Stroke......Page 1059
Pathogenesis......Page 986
Hardware......Page 2171
Lamotrigine......Page 987
Levetiracetam......Page 988
Mechanisms of Neurologic Injury during Deep Hypothermic Cardiac Surgery......Page 1313
Diastematomyelia......Page 991
Radiation Therapy......Page 1600
Neurologic Abnormalities......Page 2081
Brain Abscess......Page 1871
Focal and Multifocal Seizures......Page 999
Benign Familial Neonatal Epilepsy......Page 1003
Treatment of NMO in Children......Page 1157
Aluminum toxicity......Page 1005
Glutamate......Page 1265
Treatment......Page 1010
Levels of Care......Page 1011
Seizures......Page 1012
Disorders of α-Dystroglycan Glycosylation......Page 1014
Epilepsy and Neurodevelopmental Disorders......Page 1015
Sexual Precocity......Page 1713
Wikis......Page 2167
Genetics......Page 1016
Tuberous Sclerosis and Other Autosomal-Dominant Conditions......Page 1048
Molecular Genetics and Pathogenesis of Spinal Muscular Atrophy......Page 1017
Evaluation of Delayed or Arrested Puberty......Page 1175
Risk Factors......Page 1650
Benign Myoclonic Epilepsy of Infancy......Page 1022
Clinical Features and Subclassification......Page 1915
Mutations in the SCN1A Gene......Page 1023
Sleep Physiology and Ontogeny......Page 1174
Perinatal Brain Injury......Page 1248
Corticosteroids......Page 1025
Fabry's Disease......Page 1030
Sandifer's Syndrome......Page 1035
Febrile Seizures......Page 1038
Clinical and Laboratory Tests......Page 1802
Overview......Page 1051
Valproic Acid/Valproate......Page 1055
Autonomic Disorders Associated with Developmental Arrest......Page 1958
Etiology......Page 1261
Acellular Pertussis Vaccine......Page 1474
Topiramate......Page 1072
Neuropsychological Impairments......Page 1075
Limb-Girdle Muscular Dystrophy 2B: Dysferlinopathy......Page 1839
Treatment......Page 1076
Clinical Examination......Page 1077
Other Medications......Page 1078
Laboratory Tests for Idiosyncratic Reactions......Page 1079
Myotonic Chondrodystrophy: Schwartz-Jampel Syndrome......Page 1693
Eastern Equine Encephalitis Virus......Page 1080
Diagnosis......Page 1527
Spinocerebellar Ataxia Type 23 (SCA23)......Page 1081
Managing Adverse Effects......Page 1082
Human Embryonic Stem Cells......Page 1083
The Ketogenic Diet......Page 1084
Perfection and Neuroethics......Page 1094
Hospitalization......Page 1096
Epilepsy Surgery in the Pediatric Population......Page 1102
Cognitive and Behavioral Disorders......Page 1103
Indications for Epilepsy Surgery......Page 1104
Magnetic Resonance Imaging......Page 1108
Single-Photon Emission Computed Tomography......Page 1110
Shunt infections......Page 1492
Sarcotubular System......Page 1636
Paraneoplastic Cerebellar Degeneration......Page 1122
Management of Spasticity......Page 1919
Benign Congenital Hypotonia......Page 1741
Treatment......Page 1125
Treatment......Page 1869
Laboratory data......Page 1518
Headaches in Infants and Children......Page 1128
Neurologic Examination......Page 1133
Vitamin and Co-factor Deficiencies......Page 1134
Cyclical Vomiting Syndrome......Page 1136
Functional Abdominal Pain......Page 1967
Completed Randomized Controlled Clinical Trials for Medical Treatment of Acute Spinal Cord Injury......Page 1460
Treatment......Page 1422
Nonpharmacologic Measures for CDH......Page 1143
Proximal Renal Tubular Acidosis......Page 2061
Cranial Neuralgias......Page 1144
Peripheral nervous system involvement......Page 1694
Brain Tumor Headache......Page 1146
Clinical Features......Page 1148
Other agents......Page 1153
Diagnostic Evaluation......Page 1155
Contraindications......Page 1493
Paroxysmal Kinesigenic Dyskinesia......Page 1159
Anterior spinal cord syndrome......Page 1162
Dialysis Disequilibrium Syndrome......Page 2047
Epidemiology......Page 1288
Diagnosis......Page 1170
Spinocerebellar Ataxia Type 2 (SCA2 - OMIM 183090)......Page 1203
Treatment......Page 2056
Multiple Sleep Latency Test......Page 1177
Idiopathic Hypersomnia......Page 1182
Heterotopic Ossification......Page 1184
Arthritis and other systemic manifestations......Page 1189
Neural Control of Muscle Contraction......Page 1195
Epidemiology......Page 1196
Spinocerebellar Ataxia, Autosomal-Recessive 6 (SCAR6 - OMIM 608029); Norwegian Nonprogressive Infantile Cerebellar Ataxia (CLA3......Page 1199
Management......Page 1266
Introduction......Page 2146
Clinical Features......Page 1200
Spinocerebellar Ataxia Type 1 (SCA1 - OMIM 164400)......Page 1202
Thrombotic Thrombocytopenic Purpura......Page 1769
Spinocerebellar Ataxia Type 7 (SCA7 - OMIM 164500)......Page 1205
Antidepressants......Page 1207
Drug-Induced Encephalopathy in Renal Failure......Page 1208
Neurodiagnostic Testing......Page 1696
Clinical Features......Page 1936
Leptospirosis......Page 1210
Fatty Acid Oxidation Disorders......Page 1849
Proposed Diagnostic Approach to the Hereditary Ataxias......Page 1212
Disorders of Energy Production......Page 1213
Risk Factors......Page 1669
General Examination......Page 1221
Flaviviruses......Page 1428
Classification......Page 1223
Clinical Features......Page 1935
Dopa-Responsive Dystonia......Page 1225
St. Louis Encephalitis Virus......Page 1226
Clinical Features......Page 1845
Neurologic Manifestations......Page 1227
Pantothenate Kinase-Associated Neurodegeneration......Page 1228
Fahr's Disease......Page 1229
Other Disorders Causing Secondary Dystonia......Page 1230
Treatments for Dystonia......Page 1231
Tremor......Page 1232
Primary Tremor......Page 1233
Parkinsonism......Page 1234
Secondary Parkinsonism......Page 1235
Physiologic and Developmental Myoclonus......Page 1236
Symptomatic Myoclonus......Page 1237
Ataxia......Page 1238
Paroxysmal Kinesigenic Dyskinesia......Page 1240
Necrotizing Sarcoid Granulomatosis......Page 1706
Tic Disorders......Page 1241
Transient Developmental Movement Disorders......Page 1242
Folate dependency......Page 1243
Benign Idiopathic Dystonia of Infancy......Page 1244
Antiphospholipid Antibody Syndrome......Page 1709
Restless Leg Syndrome and Periodic Leg Movements of Sleep......Page 1245
Spasticity......Page 1246
Epidemiology......Page 1247
Postnatal Brain Injury......Page 1250
Further Diagnostic Evaluation......Page 1251
Ataxic Cerebral Palsy......Page 1254
Mixed Cerebral Palsy......Page 1255
Pathophysiology......Page 1257
Casuistry......Page 2157
Monomelic Amyotrophy (Hirayama's Disease)......Page 1260
Communication and Cognitive Deficits......Page 2135
Clinical features......Page 1546
Hypomyelinating White-Matter Disorders......Page 1269
Hypomyelination with Atrophy of the Basal Ganglia and Cerebellum......Page 1276
Pain Management......Page 1284
Megalencephalic Leukoencephalopathy with Subcortical Cysts......Page 1289
Polyglucosan Body Disease and Adult Polyglucosan Body Disease......Page 1291
Laboratory Evaluation......Page 1793
Limb-Girdle Muscular Dystrophies 2C, 2D, 2E, and 2F: Sarcoglycanopathies......Page 1292
Defects in Glucose/Carbohydrate Metabolism......Page 1293
Friedreich's Ataxia......Page 1770
Neuropathology......Page 1615
Clinical characteristics, clinical laboratory tests, and diagnosis......Page 1569
Cystathioninuria......Page 2102
Pyridoxine intoxication......Page 1851
Magnetic Resonance Spectroscopy......Page 1367
B12 Deficiency During Infancy and Childhood......Page 2104
Cobalamin dependency......Page 1878
Internet Sites......Page 2005
Cogan's Syndrome......Page 1703
Mitochondrial neurogastrointestinal encephalopathy......Page 1298
Nesprin-Associated Congenital Muscular Dystrophy......Page 1854
Acute CNS Demyelination......Page 1300
Magnetic Resonance Imaging......Page 1302
Relapsing Demyelinating Disorders......Page 1305
Impairment of Consciousness and Coma......Page 1310
Definitions......Page 1312
General Physical Examination......Page 1321
Historical Perspective......Page 1323
Developmental, Emotional and Spiritual Concerns......Page 1931
Outcome Measurement......Page 1329
Conclusions......Page 1333
Management......Page 1341
Emergent Treatment......Page 1661
Herniation Syndromes......Page 1344
Spinal Cord Vascular Anomalies......Page 1749
Pathology......Page 1348
Uremic Peripheral Polyneuropathy (Neuropathy of Chronic Renal Failure)......Page 1349
Inheritance......Page 1350
Idiopathic Autonomic Neuropathy and Paraneoplastic Disorders......Page 1351
Diagnostic Evaluation......Page 1352
Clinical features......Page 1524
Magnetic Resonance Imaging......Page 1671
Hyperosmolar Therapy......Page 1356
Management......Page 1559
Clinical Characteristics......Page 1357
Temperature Control and Hypothermia......Page 1358
Cortical Gliomas......Page 1359
Mild Traumatic Brain Injury without Loss of Consciousness in Child Over 2 Years Old......Page 1361
Mild Traumatic Brain Injury with Brief Loss of Consciousness in Child Over 2 Years Old......Page 1363
Primary Vasculitic Diseases......Page 1366
Diagnosis......Page 1532
Stage 1: Hemolymphatic Stage......Page 1369
Post-Traumatic Epilepsy......Page 1370
Subacute and Chronic Subdural Hematoma......Page 1371
Post-Traumatic Headache......Page 1372
Clinical Characteristics, Clinical Laboratory Tests, and Diagnosis......Page 1575
Conclusions......Page 1373
Non-accidental Head Trauma......Page 1374
Scalp......Page 1379
Mixed-density or ``hyperacute´´ subdural hematoma......Page 1381
Future Directions......Page 1662
Cerebral infarction......Page 1383
Genetics......Page 1386
Spinal Injuries......Page 1388
Biochemical Markers......Page 1392
General Considerations......Page 1393
Riboflavin-Dependent Glutaric Acidemia Type I......Page 1395
Conclusion......Page 1396
Formation of Oxygen Radicals......Page 1404
Meningitis after cochlear implants......Page 1405
Short Bowel Syndrome......Page 1407
Immunotherapy......Page 1417
Resuscitation......Page 1424
Treatment......Page 1425
Riluzole......Page 1426
Chloramphenicol......Page 1429
Reduction of Lipid Peroxidation......Page 1430
Molecular Biology......Page 1431
Epidemiology, microbiology, and pathology......Page 1432
Medical Aspects of Acute Pediatric Rehabilitation Management......Page 2127
Effects of Intracranial Hypertension on Cerebral Perfusion......Page 1434
Intracranial Pressure Monitoring......Page 1436
Effects of Gender on Increased Intracranial Pressure......Page 1440
Diagnosis and Management......Page 1443
Bony Spine and Ligaments......Page 1446
Euthanasia......Page 2162
Laboratory Studies......Page 1453
Trichinosis......Page 1806
Lambert-Eaton Myasthenic Syndrome......Page 1459
Abnormalities of α-Dystroglycan Glycosylation......Page 1850
Bereavement......Page 2091
Thyrotropin releasing hormone......Page 1462
Metabolic Diseases Producing Generalized Renal and Neurologic Dysfunction......Page 2060
Clinical features......Page 1463
Treatment......Page 1934
Epidemiology and Pathogenesis......Page 1504
Ethosuximide......Page 1464
Antiviral Agents......Page 1465
Long-Term Management......Page 1466
Cavernous malformations......Page 1682
Prognosis......Page 1467
Historical Perspective......Page 1469
Number of Examinations and Examiners......Page 1475
Analgesia......Page 1477
Comparison of EEG and CBF studies......Page 1484
Ancillary Studies......Page 1486
Bacterial Infections of the Nervous System......Page 1489
Oral Medications......Page 2130
Hyponatremia, dehydration, and inappropriate secretion of antidiuretic hormone......Page 1494
Insect repellents......Page 1990
Extra-Axial Fluid Collections......Page 1497
Intracranial hypertension......Page 1500
Prognosis......Page 1501
Recurrent Acute Bacterial Meningitis......Page 1502
Vitamin A (Retinol)......Page 1503
Treatment......Page 1506
Molecular Genetics......Page 1847
Clinical presentation and diagnosis......Page 1507
Leprosy......Page 1508
General Considerations......Page 1510
Slow Viral Infections......Page 1514
Clinical features......Page 1521
Treatment and outcome......Page 1523
Togaviruses......Page 1526
Diagnosis, treatment, and outcome......Page 1530
Paramyxoviruses......Page 1531
Fasting Adaptation......Page 1568
Ganglioglioma......Page 1533
Arenavirisues......Page 1534
Clinical features......Page 1535
Diagnosis......Page 1536
Human T-Cell Lymphotropic Virus Type I......Page 1537
Parechoviruses......Page 1538
Cryptococcosis......Page 1539
Candidiasis......Page 1545
Cyclic Vomiting Syndrome......Page 1966
Lyme Disease......Page 1691
Cerebral Amebiasis: Entamoeba histolytica......Page 1556
Clinical characteristics, clinical laboratory tests, and diagnosis......Page 1557
Epidemiology, microbiology, and pathology......Page 1560
Vinca Alkaloids......Page 1998
Malaria......Page 1564
Management......Page 1567
Technique of Drug Administration......Page 2000
Clinical characteristics, clinical laboratory tests, and diagnosis......Page 1570
African Sleeping Sickness......Page 1571
Specific Features of Individual Genetic Defects......Page 1572
History and Physical Examination......Page 1883
Toxocariasis......Page 1574
Carnitine uptake studies......Page 1884
Epidemiology, Microbiology, and Pathology......Page 1576
Epidemiology, Microbiology, and Pathology......Page 1577
Clinical Characteristics, Clinical Laboratory Tests, and Diagnosis......Page 1578
Triheptanoin......Page 1887
Cognitive Impairments......Page 2111
Epidemiology, microbiology, and pathology......Page 1579
Management......Page 1580
Epidemiology, microbiology, and pathology......Page 1581
Erythromelalgia and Erythermalgia......Page 1582
Epidemiology, microbiology, and pathology......Page 1583
Clinical characteristics, clinical laboratory tests, and diagnosis......Page 1584
Clinical characteristics, clinical laboratory tests, and diagnosis......Page 1585
Management......Page 1586
Tumors of the Brain and Spine......Page 1587
Li-Fraumeni Syndrome......Page 1589
Magnetic Resonance Spectroscopy......Page 1593
Emergency Evaluation and Management......Page 1980
History......Page 1595
Chemotherapy......Page 1602
Embryonal Tumors......Page 1604
Atypical Teratoid/Rhabdoid Tumors......Page 1610
Other Embryonal Tumors/Medulloepitheliomas and Ependymoblastomas......Page 1611
Management and outcome......Page 1612
Management and outcome......Page 1613
Refsum's Disease (Heredopathia Atactica Polyneuritiformis) and Peroxisome Biogenesis Disorders......Page 1616
Pyridox(am)ine-5-Phosphate Oxidase......Page 2101
Laboratory findings......Page 1618
Chemotherapeutic Agent-Induced Neuropathy......Page 1778
Clinical presentation and diagnosis......Page 1619
Management and outcome......Page 1620
Vitamin D dependency......Page 1621
Sarcoidosis......Page 1622
Germ Cell Tumors......Page 1623
Clinical presentation and diagnosis......Page 1624
Historical Considerations......Page 1625
Management and outcome......Page 1626
Clinical presentation and diagnosis......Page 1627
Pituitary Tumors......Page 1628
Clinical presentation and diagnosis......Page 1629
Spinal Cord Tumors......Page 1630
Management and outcome......Page 1631
Plexiform Neurofibromas......Page 1632
Central Nervous System Leukemia......Page 1633
Lymphoma......Page 1634
Therapeutic Approaches to Mitochondrial Diseases......Page 1904
Neuroblastoma......Page 1635
Incidence......Page 1639
Other Medical Issues......Page 2138
Summary and Future Perspectives......Page 1642
Cerebrovascular Disease in Children......Page 1643
Iron deficiency anemia......Page 1659
Endocrinopathy......Page 2046
Pathophysiology......Page 1667
Protein-Energy Malnutrition......Page 2092
Computed Tomography......Page 1670
Clinical Features......Page 1675
Risk Factors......Page 1677
Niacin deficiency......Page 1679
Treatment......Page 1680
Vein of galen malformations......Page 1684
Neuropathies......Page 1686
Diseases Affecting Both Kidney and Nervous System......Page 2055
Neurologic Manifestations......Page 1692
Scleroderma......Page 1698
Classic Botulism......Page 1777
Polyarteritis Nodosa......Page 1701
Biotin deficiency......Page 2108
Laboratory findings......Page 1705
Zinc Deficiency......Page 1708
Acknowledgment......Page 1710
Embryology and Development......Page 1711
Nuclear Membrane-Related Proteins: Emerin and Lamin A/C......Page 1720
Spinal Cord Anomalies Affecting Motor Neurons......Page 1742
Muscle Metabolism......Page 1726
Muscle Imaging......Page 1728
Genetics......Page 1855
Other Hereditary Diseases Affecting Spinal Motor Neurons......Page 1734
Transverse Myelitis......Page 1746
Acute Inflammatory Demyelinating Polyradiculoneuropathy (Guillain-Barré Syndrome)......Page 1756
Investigations......Page 1758
Congenital and Early Infantile Axonal Types......Page 1766
Becker Muscular Dystrophy......Page 1835
Cerebrospinal Fluid......Page 1786
Diabetes Mellitus......Page 1773
Abetalipoproteinemia......Page 1774
Heavy Metal Neuropathy......Page 1779
Clinical Features......Page 1780
Clinical Features......Page 1792
Pathologic Findings......Page 1794
Other causes of Immune-Mediated Neuropathies in Children......Page 1796
Synthesis......Page 1799
Neurologic Examination......Page 1800
Cyclosporine......Page 1809
Clinical Features......Page 1811
Rheumatic Heart Disease......Page 1812
Botulism......Page 1816
Dystrophinopathies (Duchenne and Becker Muscular Dystrophies and Clinical Variants)......Page 1818
Plasticity of the Nervous System......Page 1822
Female Duchenne Muscular Dystrophy, Carriers, and Manifesting Carriers......Page 1836
Ullrich's Congenital Muscular Dystrophy and Bethlem's Myopathy......Page 1853
Utilization of Bioenergetic Substrates in Exercise......Page 1861
Pathophysiology......Page 1863
Acid Maltase Deficiency......Page 1865
Biochemistry......Page 1874
Increased Susceptibility of the Child......Page 1875
Normal Pathway of Fatty Acid Oxidation......Page 1876
Alterations in plasma and tissue concentrations of carnitine......Page 1877
Differentiating Laboratory Features......Page 1881
Carnitine......Page 1882
Vitamin D......Page 2106
Molecular studies......Page 1885
Biotin-Dependent Holocarboxylase Synthetase Deficiency (Neonatal Multiple Carboxylase Deficiency)......Page 1886
Mitochondrial Encephalomyopathies......Page 1888
Biochemical Classification......Page 1889
Genetic Classification......Page 1890
Sporadic Progressive External Ophthalmoplegia with Ragged Red Fibers......Page 1894
MELAS Syndrome......Page 1895
ATPase 6 mutation......Page 1896
Diseases due to Mutations in nDNA......Page 1897
Mutations in genes encoding subunits or ancillary proteins of the respiratory chain......Page 1898
Complex III deficiency......Page 1899
Combined defects of the respiratory chain......Page 1900
Defects of Mitochondrial Protein Importation......Page 1901
Multiple mitochondrial DNA deletions......Page 1902
Depletion of mitochondrial DNA......Page 1903
Acknowledgments......Page 1905
Dermatomyositis......Page 1906
Treatment......Page 1928
Periodic Paralysis with Cardiac Arrhythmia: Andersen-Tawil Syndrome......Page 1930
Treatment......Page 1937
Anatomic and Physiologic Aspects......Page 1938
Theoretical Approaches to Ethics......Page 2154
Normal Biochemistry and Physiology of Prolactin......Page 1943
Pneumococcal Conjugated Vaccine......Page 1949
The Peripheral Autonomic Nervous System......Page 1952
Disorders of Micturition and Defecation......Page 1968
Rehabilitation Therapy......Page 1969
History......Page 1972
Clinical Laboratory Tests......Page 1974
Management......Page 1978
Introduction......Page 1979
Other Nonpharmacologic Compounds......Page 1991
``Ecstasy´´......Page 1993
Other Sedatives......Page 1994
Other Agents Used in Cancer Chemotherapy......Page 1999
Developmental Immaturity......Page 2001
Underlying Disease......Page 2002
Neuroteratology......Page 2003
Neurologic Complications Following Anatomic Intervention......Page 2010
Interrelationships between Renal and Neurologic Diseases and Therapies......Page 2030
Stroke......Page 2045
Uremic Myopathy (Myopathy of Chronic Renal Failure)......Page 2052
Vasculitic Diseases with Neurologic-Renal Presentations......Page 2057
Neurologic Drugs that May Affect Renal Function in Individuals with Normal Kidneys......Page 2062
Drug Therapy in Renal Disease......Page 2065
Other Neurologic Drugs......Page 2068
Nipah Virus......Page 2077
Fulminant liver failure......Page 2082
Neuropathology and Pathophysiology......Page 2083
Pathogenesis......Page 2087
Therapy......Page 2089
Bilirubin Encephalopathy: Kernicterus......Page 2090
Riboflavin dependency......Page 2098
Pyridoxine deficiency......Page 2100
Cobalamin (Vitamin B12)......Page 2103
Vitamin C deficiency......Page 2105
Folate (Vitamin M)......Page 2110
Methylenetetrahydrofolate Reductase Deficiency......Page 2112
Iodine Deficiency......Page 2113
Neurologic Complications of Immunization......Page 2115
Inactivated Polio Vaccine......Page 2116
Human Papillomavirus Vaccine......Page 2121
Pediatric Neurorehabilitation Medicine......Page 2124
Principles of Pediatric Neurorehabilitation......Page 2126
Bowel and Bladder Dysfunction......Page 2137
Follow-Up Conference......Page 2151
Summary......Page 2153
Principlism......Page 2156
Diagnostic Decision Support......Page 2168
A......Page 2176
B......Page 2185
C......Page 2189
D......Page 2200
E......Page 2205
F......Page 2211
G......Page 2214
H......Page 2219
I......Page 2225
K......Page 2229
L......Page 2230
M......Page 2234
N......Page 2243
O......Page 2248
P......Page 2250
R......Page 2259
S......Page 2262
T......Page 2271
U......Page 2277
V......Page 2278
W......Page 2280
Z......Page 2281