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

✍ Scribed by Kenneth F. Swaiman MD, Stephen Ashwal MD, Donna M Ferriero MD, Nina Schor MD PhD

• Publisher: Saunders
• Year: 2011
• Tongue: English
• Leaves: 2282
• Edition: 5th Edition
• Category: Library

✦ Synopsis

Swaiman's Pediatric Neurology, by Drs. Kenneth Swaiman, Stephen Ashwal, Donna Ferriero, and Nina Schor, is a trusted resource in clinical pediatric neurology with comprehensive, authoritative, and clearly-written guidance. Extensively updated to reflect advancements in the field, this fifth edition covers new imaging modalities such as pediatric neuroimaging, spinal fluid examination, neurophysiology, as well as the treatment and management of epilepsy, ADHD, infections of the nervous system, and more. The fully searchable text is now available online at www.expertconsult.com, along with downloadable images and procedural videos demonstrating intraventricular hemorrhage and white matter injury, making this an indispensable multimedia resource in pediatric neurology. Gain a clear visual understanding from the numerous illustrations, informative line drawings, and summary tables. Tap into the expertise of leaders in the field from the authoritative and respected team of editors and contributors. Get comprehensive coverage of all aspects of pediatric neurology with a clinical focus useful for both the experienced clinician and the physician-in-training. Access the fully searchable text online at www.expertconsult.com, along with downloadable images, procedural videos demonstrating intraventricular hemorrhage and white matter injury, and links to PubMed. Stay current on recent developments through extensive revisions: a new chapter on paraneoplastic syndromes in children; a new section on congenital brain malformations written by leading international authorities; and another one on cutting-edge pediatric neuroscience concepts relating to plasticity, neurodegeneration of the developing brain, and neuroinflammation. Apply the latest information on diagnostic modalities, including pediatric neuroimaging, spinal fluid examination, and neurophysiology The indispensible Swaiman's Pediatric Neurology is your multimedia guide to practicing pediatric neurology.

✦ Table of Contents

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<br>Sialidosis......Page 688<br>Anatomic and Biochemical Effects of Undernutrition......Page 689<br>Mucolipidosis Type I (Sialidosis Type II)......Page 690<br>Clinical Features......Page 1932<br>Danon´s Disease......Page 694<br>Sialic Acid Storage Disorders......Page 695<br>Cystinosis......Page 696<br>Spinocerebellar Ataxia Type 20 (SCA20 - OMIM 608687)......Page 697<br>Introduction......Page 700<br>Histopathologic Disturbances......Page 703<br>Orthostatic Hypotension......Page 1856<br>Intrathecal Baclofen Therapy......Page 708<br>Neuropsychologic involvement......Page 1828<br>Other pseudo-obstruction syndromes......Page 710<br>Fiber Type Disproportion Myopathy......Page 1437<br>Management......Page 714<br>Clinical Features......Page 1067<br>Organ Donation......Page 719<br>Adrenocorticotropic Excess......Page 1044<br>Developmental Differences......Page 720<br>Posterior Reversible Encephalopathy Syndrome......Page 1287<br>Vacuolar Myopathy......Page 1860<br>Treatment......Page 724<br>Identification of Cause......Page 898<br>Laboratory Tests......Page 727<br>Pathophysiology......Page 729<br>Contiguous Deletion of the XALD Gene......Page 730<br>Ethanol......Page 1420<br>Human Herpesviruses Types 6, 7, and 8......Page 1218<br>Subdural effusion......Page 1390<br>Barriers to Palliative Care......Page 1609<br>Theophylline......Page 1996<br>Current and Future Outlook......Page 733<br>Morphology......Page 736<br>Primary injury phase......Page 737<br>Treatment......Page 1909<br>Neurologic Examination......Page 1973<br>Clinical Features......Page 1191<br>Management......Page 1179<br>The Neurologic Outcome of Children Undergoing Extracorporeal Membrane Oxygenation......Page 1830<br>Experimental therapies......Page 1832<br>Intrauterine and Perinatal Viral Infections......Page 744<br>Laboratory data......Page 750<br>Meningococcal Conjugate Vaccine......Page 751<br>Fear of Side Effects and Effective Antiepileptic Drug Use......Page 1126<br>Clinical Characteristics......Page 757<br>Mercury......Page 758<br>Arteriopathic stroke......Page 2022<br>Limb-Girdle Muscular Dystrophy 1C: Caveolinopathy......Page 1837<br>Cognitive and behavioral abnormalities......Page 1032<br>Molecular Pathogenesis......Page 1838<br>Management......Page 760<br>Epidermal Nevus Syndrome......Page 761<br>Neuropathology......Page 762<br>Health Care Decision-Making......Page 902<br>Hepatolenticular Degeneration: Wilson's Disease......Page 763<br>Wyburn-Mason Syndrome (Retinocephalic Angiomatosis)......Page 764<br>Genetics......Page 770<br>Hypertensive Encephalopathy......Page 2017<br>Risk Factors......Page 1057<br>Kidney......Page 776<br>Dialysis-Associated Seizures......Page 1127<br>Clinical Description......Page 778<br>Genetics......Page 779<br>Laboratory Findings......Page 780<br>Epilepsy......Page 1499<br>Beta-Sarcoglycanopathy (Limb-Girdle Muscular Dystrophy 2E)......Page 1841<br>Assessment and Treatment of Symptoms......Page 1354<br>Management and treatment......Page 784<br>Krabbe's Disease (Globoid Cell Leukodystrophy)......Page 1772<br>Myopathy......Page 787<br>Pathophysiology......Page 931<br>Juvenile Neuronal Ceroid-Lipofuscinosis or Juvenile Batten's Disease......Page 788<br>Molecular genetics......Page 1563<br>Laboratory data......Page 1296<br>Pathology......Page 789<br>CLN9-Deficient Juvenile-like Variant......Page 790<br>Clinical Features......Page 792<br>The Central Autonomic Nervous System......Page 793<br>Pathophysiology......Page 1970<br>Intraoperative Techniques......Page 1598<br>Supraspinal Changes......Page 798<br>Postsynaptic Defects......Page 1815<br>Biochemical Markers......Page 1421<br>Stimulants......Page 799<br>Laboratory data......Page 1355<br>Nephropathic Cystinosis......Page 1558<br>Thrombolysis......Page 1672<br>Biochemistry......Page 800<br>Treatment of Neurologic Manifestations......Page 1561<br>Biotinidase deficiency......Page 801<br>Definitions......Page 802<br>Acute Rheumatic Fever......Page 803<br>Antihistamines......Page 812<br>Mechanisms of Thromboembolism......Page 1668<br>Discussions with Family Members and Staff......Page 1487<br>Physical Examination......Page 814<br>Feeding and Nutritional Disorders......Page 820<br>Outcome and Prognosis......Page 821<br>Acknowledgments......Page 822<br>Pathogenesis: Mechanisms of Spinal Cord Injury......Page 1335<br>Developmental Language Disorders......Page 852<br>Juvenile Myoclonic Epilepsy......Page 854<br>Growth Hormone Deficiency......Page 856<br>Prematurity......Page 857<br>Lexical Syntactic Syndrome......Page 858<br>Zygomycosis......Page 859<br>Definition......Page 861<br>Mechanism of Action......Page 1090<br>Diagnosis of Attention-Deficit Hyperactivity Disorder......Page 870<br>Authoritative Narrative Content......Page 872<br>Mechanisms of Infarction and Developmental Brain Injury......Page 1433<br>Autistic Regression and Childhood Disintegrative Disorder......Page 889<br>Multiple sclerosis......Page 875<br>Cerebral Blood Flow and Metabolism After Resuscitation......Page 881<br>Historical Background......Page 1097<br>Neurologic Examination......Page 1977<br>Calcifying Leukoencephalopathies......Page 882<br>Anterior Spinal Artery Occlusion......Page 883<br>Complementary and Alternative Medications......Page 884<br>Epidemiology......Page 890<br>Hirschsprung's disease......Page 957<br>Cardiac Risk Factors......Page 1592<br>Hypothalamic/Pituitary Disorders of Thyroid Function......Page 1314<br>Other Ancillary Testing......Page 1056<br>Genetics......Page 897<br>Diabetes Insipidus......Page 1948<br>Types of Pain Medications......Page 1320<br>Malnutrition......Page 1345<br>Clinical Manifestations......Page 1664<br>Epigenetics......Page 903<br>Benzodiazepines......Page 905<br>Clinical Assessment......Page 1391<br>Complementary and Alternative Medicine......Page 907<br>Disclaimer......Page 911<br>Introduction......Page 912<br>History......Page 1149<br>Hereditary Sensory and Autonomic Neuropathy Type IV (Congenital Insensitivity to Pain with Anhidrosis)......Page 920<br>Clinical Management......Page 921<br>Treatment......Page 1156<br>History......Page 922<br>Cardiac management......Page 1834<br>Mononeuropathies......Page 1161<br>Electroencephalographic Findings......Page 924<br>Inborn Errors of Metabolism......Page 2028<br>Systemic Lupus Erythematosus......Page 1608<br>Antidepressants and risk for suicidal behavior......Page 928<br>Seizures......Page 929<br>Ataxia-Telangiectasia......Page 1219<br>Wound Botulism......Page 1817<br>OKT3......Page 1997<br>Treatment......Page 1775<br>Riboflavin-Responsive Isolated Complex II Deficiency......Page 1147<br>Apicomplexan Infections of the Central Nervous System......Page 1562<br>Pharmacology......Page 932<br>Babesiosis......Page 933<br>Measles Virus......Page 934<br>Clinical Applications......Page 935<br>Acknowledgments......Page 1299<br>Other Studies......Page 1704<br>Tocopherol (Vitamin E)......Page 2107<br>Vitamin K......Page 2109<br>Clinical applications......Page 940<br>Common Characteristics of Typical Antipsychotics......Page 941<br>Clinical Applications......Page 943<br>Common Characteristics......Page 944<br>Clinical Applications......Page 945<br>Clinical Management......Page 946<br>Clinical Applications......Page 947<br>Clinical Management......Page 948<br>Pharmacology......Page 949<br>Conclusion......Page 950<br>Pediatric Epilepsy......Page 951<br>Neuropathology......Page 1258<br>Second-line therapies......Page 1307<br>Depolarizing Conductances......Page 960<br>Development of Ionic Channels and Membrane Properties......Page 968<br>Transverse Myelitis......Page 1039<br>Mechanisms of Late Recovery......Page 1301<br>Clinical Features......Page 978<br>History......Page 981<br>Desminopathies......Page 1858<br>Epidemiology of Pediatric Stroke......Page 1059<br>Pathogenesis......Page 986<br>Hardware......Page 2171<br>Lamotrigine......Page 987<br>Levetiracetam......Page 988<br>Mechanisms of Neurologic Injury during Deep Hypothermic Cardiac Surgery......Page 1313<br>Diastematomyelia......Page 991<br>Radiation Therapy......Page 1600<br>Neurologic Abnormalities......Page 2081<br>Brain Abscess......Page 1871<br>Focal and Multifocal Seizures......Page 999<br>Benign Familial Neonatal Epilepsy......Page 1003<br>Treatment of NMO in Children......Page 1157<br>Aluminum toxicity......Page 1005<br>Glutamate......Page 1265<br>Treatment......Page 1010<br>Levels of Care......Page 1011<br>Seizures......Page 1012<br>Disorders of α-Dystroglycan Glycosylation......Page 1014<br>Epilepsy and Neurodevelopmental Disorders......Page 1015<br>Sexual Precocity......Page 1713<br>Wikis......Page 2167<br>Genetics......Page 1016<br>Tuberous Sclerosis and Other Autosomal-Dominant Conditions......Page 1048<br>Molecular Genetics and Pathogenesis of Spinal Muscular Atrophy......Page 1017<br>Evaluation of Delayed or Arrested Puberty......Page 1175<br>Risk Factors......Page 1650<br>Benign Myoclonic Epilepsy of Infancy......Page 1022<br>Clinical Features and Subclassification......Page 1915<br>Mutations in the SCN1A Gene......Page 1023<br>Sleep Physiology and Ontogeny......Page 1174<br>Perinatal Brain Injury......Page 1248<br>Corticosteroids......Page 1025<br>Fabry's Disease......Page 1030<br>Sandifer's Syndrome......Page 1035<br>Febrile Seizures......Page 1038<br>Clinical and Laboratory Tests......Page 1802<br>Overview......Page 1051<br>Valproic Acid/Valproate......Page 1055<br>Autonomic Disorders Associated with Developmental Arrest......Page 1958<br>Etiology......Page 1261<br>Acellular Pertussis Vaccine......Page 1474<br>Topiramate......Page 1072<br>Neuropsychological Impairments......Page 1075<br>Limb-Girdle Muscular Dystrophy 2B: Dysferlinopathy......Page 1839<br>Treatment......Page 1076<br>Clinical Examination......Page 1077<br>Other Medications......Page 1078<br>Laboratory Tests for Idiosyncratic Reactions......Page 1079<br>Myotonic Chondrodystrophy: Schwartz-Jampel Syndrome......Page 1693<br>Eastern Equine Encephalitis Virus......Page 1080<br>Diagnosis......Page 1527<br>Spinocerebellar Ataxia Type 23 (SCA23)......Page 1081<br>Managing Adverse Effects......Page 1082<br>Human Embryonic Stem Cells......Page 1083<br>The Ketogenic Diet......Page 1084<br>Perfection and Neuroethics......Page 1094<br>Hospitalization......Page 1096<br>Epilepsy Surgery in the Pediatric Population......Page 1102<br>Cognitive and Behavioral Disorders......Page 1103<br>Indications for Epilepsy Surgery......Page 1104<br>Magnetic Resonance Imaging......Page 1108<br>Single-Photon Emission Computed Tomography......Page 1110<br>Shunt infections......Page 1492<br>Sarcotubular System......Page 1636<br>Paraneoplastic Cerebellar Degeneration......Page 1122<br>Management of Spasticity......Page 1919<br>Benign Congenital Hypotonia......Page 1741<br>Treatment......Page 1125<br>Treatment......Page 1869<br>Laboratory data......Page 1518<br>Headaches in Infants and Children......Page 1128<br>Neurologic Examination......Page 1133<br>Vitamin and Co-factor Deficiencies......Page 1134<br>Cyclical Vomiting Syndrome......Page 1136<br>Functional Abdominal Pain......Page 1967<br>Completed Randomized Controlled Clinical Trials for Medical Treatment of Acute Spinal Cord Injury......Page 1460<br>Treatment......Page 1422<br>Nonpharmacologic Measures for CDH......Page 1143<br>Proximal Renal Tubular Acidosis......Page 2061<br>Cranial Neuralgias......Page 1144<br>Peripheral nervous system involvement......Page 1694<br>Brain Tumor Headache......Page 1146<br>Clinical Features......Page 1148<br>Other agents......Page 1153<br>Diagnostic Evaluation......Page 1155<br>Contraindications......Page 1493<br>Paroxysmal Kinesigenic Dyskinesia......Page 1159<br>Anterior spinal cord syndrome......Page 1162<br>Dialysis Disequilibrium Syndrome......Page 2047<br>Epidemiology......Page 1288<br>Diagnosis......Page 1170<br>Spinocerebellar Ataxia Type 2 (SCA2 - OMIM 183090)......Page 1203<br>Treatment......Page 2056<br>Multiple Sleep Latency Test......Page 1177<br>Idiopathic Hypersomnia......Page 1182<br>Heterotopic Ossification......Page 1184<br>Arthritis and other systemic manifestations......Page 1189<br>Neural Control of Muscle Contraction......Page 1195<br>Epidemiology......Page 1196<br>Spinocerebellar Ataxia, Autosomal-Recessive 6 (SCAR6 - OMIM 608029); Norwegian Nonprogressive Infantile Cerebellar Ataxia (CLA3......Page 1199<br>Management......Page 1266<br>Introduction......Page 2146<br>Clinical Features......Page 1200<br>Spinocerebellar Ataxia Type 1 (SCA1 - OMIM 164400)......Page 1202<br>Thrombotic Thrombocytopenic Purpura......Page 1769<br>Spinocerebellar Ataxia Type 7 (SCA7 - OMIM 164500)......Page 1205<br>Antidepressants......Page 1207<br>Drug-Induced Encephalopathy in Renal Failure......Page 1208<br>Neurodiagnostic Testing......Page 1696<br>Clinical Features......Page 1936<br>Leptospirosis......Page 1210<br>Fatty Acid Oxidation Disorders......Page 1849<br>Proposed Diagnostic Approach to the Hereditary Ataxias......Page 1212<br>Disorders of Energy Production......Page 1213<br>Risk Factors......Page 1669<br>General Examination......Page 1221<br>Flaviviruses......Page 1428<br>Classification......Page 1223<br>Clinical Features......Page 1935<br>Dopa-Responsive Dystonia......Page 1225<br>St. Louis Encephalitis Virus......Page 1226<br>Clinical Features......Page 1845<br>Neurologic Manifestations......Page 1227<br>Pantothenate Kinase-Associated Neurodegeneration......Page 1228<br>Fahr's Disease......Page 1229<br>Other Disorders Causing Secondary Dystonia......Page 1230<br>Treatments for Dystonia......Page 1231<br>Tremor......Page 1232<br>Primary Tremor......Page 1233<br>Parkinsonism......Page 1234<br>Secondary Parkinsonism......Page 1235<br>Physiologic and Developmental Myoclonus......Page 1236<br>Symptomatic Myoclonus......Page 1237<br>Ataxia......Page 1238<br>Paroxysmal Kinesigenic Dyskinesia......Page 1240<br>Necrotizing Sarcoid Granulomatosis......Page 1706<br>Tic Disorders......Page 1241<br>Transient Developmental Movement Disorders......Page 1242<br>Folate dependency......Page 1243<br>Benign Idiopathic Dystonia of Infancy......Page 1244<br>Antiphospholipid Antibody Syndrome......Page 1709<br>Restless Leg Syndrome and Periodic Leg Movements of Sleep......Page 1245<br>Spasticity......Page 1246<br>Epidemiology......Page 1247<br>Postnatal Brain Injury......Page 1250<br>Further Diagnostic Evaluation......Page 1251<br>Ataxic Cerebral Palsy......Page 1254<br>Mixed Cerebral Palsy......Page 1255<br>Pathophysiology......Page 1257<br>Casuistry......Page 2157<br>Monomelic Amyotrophy (Hirayama's Disease)......Page 1260<br>Communication and Cognitive Deficits......Page 2135<br>Clinical features......Page 1546<br>Hypomyelinating White-Matter Disorders......Page 1269<br>Hypomyelination with Atrophy of the Basal Ganglia and Cerebellum......Page 1276<br>Pain Management......Page 1284<br>Megalencephalic Leukoencephalopathy with Subcortical Cysts......Page 1289<br>Polyglucosan Body Disease and Adult Polyglucosan Body Disease......Page 1291<br>Laboratory Evaluation......Page 1793<br>Limb-Girdle Muscular Dystrophies 2C, 2D, 2E, and 2F: Sarcoglycanopathies......Page 1292<br>Defects in Glucose/Carbohydrate Metabolism......Page 1293<br>Friedreich's Ataxia......Page 1770<br>Neuropathology......Page 1615<br>Clinical characteristics, clinical laboratory tests, and diagnosis......Page 1569<br>Cystathioninuria......Page 2102<br>Pyridoxine intoxication......Page 1851<br>Magnetic Resonance Spectroscopy......Page 1367<br>B12 Deficiency During Infancy and Childhood......Page 2104<br>Cobalamin dependency......Page 1878<br>Internet Sites......Page 2005<br>Cogan's Syndrome......Page 1703<br>Mitochondrial neurogastrointestinal encephalopathy......Page 1298<br>Nesprin-Associated Congenital Muscular Dystrophy......Page 1854<br>Acute CNS Demyelination......Page 1300<br>Magnetic Resonance Imaging......Page 1302<br>Relapsing Demyelinating Disorders......Page 1305<br>Impairment of Consciousness and Coma......Page 1310<br>Definitions......Page 1312<br>General Physical Examination......Page 1321<br>Historical Perspective......Page 1323<br>Developmental, Emotional and Spiritual Concerns......Page 1931<br>Outcome Measurement......Page 1329<br>Conclusions......Page 1333<br>Management......Page 1341<br>Emergent Treatment......Page 1661<br>Herniation Syndromes......Page 1344<br>Spinal Cord Vascular Anomalies......Page 1749<br>Pathology......Page 1348<br>Uremic Peripheral Polyneuropathy (Neuropathy of Chronic Renal Failure)......Page 1349<br>Inheritance......Page 1350<br>Idiopathic Autonomic Neuropathy and Paraneoplastic Disorders......Page 1351<br>Diagnostic Evaluation......Page 1352<br>Clinical features......Page 1524<br>Magnetic Resonance Imaging......Page 1671<br>Hyperosmolar Therapy......Page 1356<br>Management......Page 1559<br>Clinical Characteristics......Page 1357<br>Temperature Control and Hypothermia......Page 1358<br>Cortical Gliomas......Page 1359<br>Mild Traumatic Brain Injury without Loss of Consciousness in Child Over 2 Years Old......Page 1361<br>Mild Traumatic Brain Injury with Brief Loss of Consciousness in Child Over 2 Years Old......Page 1363<br>Primary Vasculitic Diseases......Page 1366<br>Diagnosis......Page 1532<br>Stage 1: Hemolymphatic Stage......Page 1369<br>Post-Traumatic Epilepsy......Page 1370<br>Subacute and Chronic Subdural Hematoma......Page 1371<br>Post-Traumatic Headache......Page 1372<br>Clinical Characteristics, Clinical Laboratory Tests, and Diagnosis......Page 1575<br>Conclusions......Page 1373<br>Non-accidental Head Trauma......Page 1374<br>Scalp......Page 1379<br>Mixed-density orhyperacute´´ 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