IAP Textbook of Pediatric Neurology Anoop Verma, PAM Kunju, Sujata Kanhere, TM Anandakeshavan
INDEX
Page numbers followed by b refer to box, f refer to figure, fc refer to flow chart, and t refer to table.
A
Abdominal pain 217
episodic 229
Abducens 15
Abetalipoproteinemia 354, 372
Abortive therapy 231
Abortive treatment, drugs for 418t
Abscess 132
pyogenic 131
tubercular 123, 132
Acanthamoeba 111
Aceruloplasminemia 358
Acetazolamide 517
Achondroplasia 36
Acid-base imbalance 57
Acidemia, propionic 87
Acid-fast bacillus 104, 115, 125, 168
Acidosis 217
metabolic 83, 86
respiratory 261
Acral pigmentation 431f
Acyclovir 518
artesunate 118
Addison disease 53
Adductor spasm 5f
Adenoma sebaceum 423f
Adenosine
deaminase 125
triphosphate 243
Adenovirus 111, 186
Adrenal hyperplasia, congenital 90
Adrenocorticotropic hormone 248, 274, 366, 371, 519
Adrenoleukodystrophy 42, 45, 349f, 515f
Aerophobia 113
Afebrile seizures, history of 187
Agraphesthesia 3
Agraphia 3
Agyria 94, 95
pachygyria spectrum 24
Aicardi syndrome 23
Airway 295
breathing and circulation 260, 267
care of 138
of resuscitation 256
Albendazole 146148
regime of 147
therapy 146
Alcohol
maternal use of 53
use of 215
Alexander's disease 45, 515f
Alexia 3
Alkalosis, metabolic 86
Allogeneic hematopoietic stem cell transplantation 363
Almotriptan 418
Alobar holoprosencephaly 22f
Alopecia 45
Alpers’ disease 45
Alpha rhythm
abnormal 483
normal 478f
Alpha-aminoadipic semialdehyde 88
deficiency of 246
Alpha-fetoprotein 21, 396
American Academy of Pediatrics 65, 144
clinical practice guidelines 183
American Association on Intellectual and Developmental Disability 64
American Psychiatric Association 63
Amiel-Tison
assessment 313f
method 46, 313
Amino acid 80, 249
disorders 87
Amino acidopathies 53, 82
Aminoglycosides 381
Amiodarone 356
Ammonia 80, 91, 249, 323
serum 323
Amphetamines 356
Amphotericin B 118, 158
Analyzing cerebrospinal fluid 471
Anarthria 206
Andermann syndrome 23
Androgen receptor triplet repeat 55
Anemia, severe 138
Anesthesia, general 259, 271
Anesthetic dugs like ketamine 106
Angioid streaks 465
Angioinvasion 151
Angioma, venous 30
Animal bite 112
classification of 442t
wound, management of 443
Ankle
arthrodesis of 61
dorsiflexion 6f, 313f
jerk 56
absent 19
Anoxia 57, 255
Antalgic gait 8
Anti-acetylcholine receptor 299
Antibiotic 55, 255
therapy 149
Anticholinergics 61
Anticonvulsant 251, 256
drugs 251
therapy of 251
maternal use of 53
rapid acting 257
Anticonvulsive medications 225
Anticysticercal therapy 146, 148
Antidepressants 255
Antiepileptic drug 140, 184, 215, 216t, 217, 221, 244, 258, 261, 268, 270, 277, 279, 284
indications of newer 216t
resistance, mechanisms of 214
schedules 213
therapy 205
Antiepileptic toxicity 169
Antiepileptic treatment 146
Antihistamines 255, 356
Anti-inflammatory drugs 147
Antimalarial treatment
initiation of 136
specific 137
Antimigraine medications 230
Anti-myelin oligodendrocyte glycoprotein 339
Antineutrophil cytoplasmic antibodies 178
Antinuclear antibodies 178
Antiparasitic therapy 146
Antipsychotics 255, 356
Antipyretics 189
Antiquitin gene 246
Antirabies vaccines 446, 449
Antithrombin 177
Antitubercular drugs 127t
first-line 128t
side effects of 128
Antitubercular treatment 127
Anti-tuberculosis treatment 127
Anxiety disorders 69
Apert's syndrome 39
Aplastic anemia 150
Apnea 249
epileptic 249
exhibit signs of 228
nonepileptic 249
Appetite, loss of 132
Apraxia 3
constructional 3
Aqueductal stenosis 29, 29f, 36
Arachnoid cyst 29, 30
Arboviruses 111
Archicerebellum 15, 16
Arginase deficiency 84, 307
Argininosuccinic acid 84
Argininosuccinic aciduria 45
Arginosuccinate lyase 325
Arginosuccinate synthetase 325
Aripiprazole 520
Arnold-Chiari malformation 26
Aromatic L-amino acid decarboxylase 87
Arrhinencephaly 23
Arrhythmias 261
Artemether 138
Artemisinin combination therapy 138
Artemisinin derivatives 137
Arterial anomalies 177
Arterial blood gas 88, 166, 299, 323, 373
Arterial dissection 175
Arterial ischemic strokes 172
Arterial oxygen, partial pressure of 295
Arteriovenous malformations 180, 281, 296, 387, 399
Artersunate 138
Artery, vertebrobasilar 297
Arthroconidia 152
Arthroconidia of coccidioidosis 152
Ascorbic acid 89
Asperger syndrome 56, 71, 78
Aspergillosis 150, 151, 154, 159
Aspergillus 150, 152, 153, 155
flavus 150
fumigatus 150, 156f
isolation of 155
spread 152
terreus 150
Asphyxia, perinatal 53, 251
Aspiration pneumonia 118, 261
Astereognosis 3
Ataxia 35, 113, 226, 238, 305, 352, 364, 368
acute 86, 369
chronic 371
progressive 371
onset of 369
predominant 306
recurrent 371
subacute 370
telangiectasia 60, 307, 354, 369, 372, 426, 427f
X-linked 373
Ataxic cerebral palsy 59, 306
Athetoid 57
Athetosis 61, 305, 352, 353
severe 305
Atkins diet, modified 280
Atonia, periodic episodes of 491f
Atrophy
cortical 431f
muscular 10
Attacks, shuddering 226
Attention deficit hyperactivity disorder 64, 69, 77
Auditory brainstem evoked response 48
Auditory meatus
external 505f
internal 510f
Auditory pathway, anatomy of 494
Auditory stimulation 314
Aura 229, 415, 415b, 415b
Autism 71, 74, 354
cause of 79f
diagnosis of 76fc
diagnostic interview revised 75
diagnostic observation schedule 75
differential diagnoses 77
features of 71, 79f
spectrum disorder 54, 56, 71, 72f
diagnosis of 75
early diagnosis of 76b
Autistic spectrum disorder 64
Autoimmune 339
limbic encephalitis 105
Autonomic involvement 391
Autopsy, metabolic 91
Autosomal recessive 56, 368
Axonal injury, diffuse 54
Azithromycin 118
B
Babinski's reflex 7
Babinski's sign 2, 7
Babinski's tonus test 50
Baby's body language 317f
Bacillus Calmette-Guérin 133
Baclofen 60, 61, 299, 310, 521
Bacteria, pyogenic 101
Bacterial meningitis, acute 187
Bacterial population 127t
Ballismus 353
Band heterotopia 28f
Barbiturates 255
coma 296
Baroda scale 313
Barth syndrome 96
Basal ganglia
calcification 104, 203f
degeneration 354
hyperintense signals 203f
Basidiospores of cryptococci 152
Basilar artery 510f
Bassen-Kornzweig syndrome 45, 372
Bat wing appearance 325f
Becker muscular dystrophy 379
Behavioral arrest 197
Behavioral disturbances 139
Behavioral paroxysms 242
Benzhexol 299, 536
Benzodiazepines 226, 228, 255, 257, 259, 284, 299
Benztropine 299, 522
Bergmeister's papilla 462
Betaine 89
Bicarbonate 137
Bickerstaff brainstem encephalitis 114
Bile acids disorders 82
Biochemical tests 54
Biotin 89, 90
Biotinidase deficiency 45, 83
Birth
asphyxia, severe 35f
trauma 57
weight, low 57
Biting animal, observation of 442
Bladder dysfunction 18
Blastomyces 150, 153, 158
dermatitidis 149
Blastomycosis 150152, 160
dermatitidis 151
Bleeding manifestation 113
Blindness, cortical 139
Blood
brain barrier 469, 469f
cell specific enzyme analysis 54
culture 103, 249
gas 91, 137
analyses 260
venous 249
glucose 91, 137
grouping 137
lactate, high 86
peripheral smear of 136
pH 137
pressure 166, 170, 260, 284
samples 118
sugar 88
level 249
levels, monitoring 252
transfusion, history of 112
urine 167
Bobble head doll syndrome 359
Bony deformity 390
Bony tenderness 390
Botox 523
Botulinum toxin 61, 523
Botulism 395
Brachioradialis, bilateral absence of 7
Brachycephalic head shape 38
Brachycephalic skull 39
Brachycephaly 39
Bradycardia 104
Brain 11
abscess 126, 156f
atrophy, diffuse 104
congenital malformation of 20, 57
cross-section of 469f
damage, prevention of 256
death 490
injury 243
mechanism of 243fc
perinatal 34
traumatic 293
iron accumulation 82, 355
magnetic resonance imaging of 156f, 157f, 347f, 348f
malformation, congenital 20, 20b, 30
noninfectious inflammations of 110
simplified organization of 12
stimulation, deep 204
tumor 126
Brainstem 7, 13, 13f, 18, 353
affection of 235
anatomy, anteroposterior 13
auditory evoked potential 494, 495, 495t
waves 495f, 496f
compression, extrinsic 18
encephalitis 378
evoked response audiometry 237
glioma 370f
involvement of 122
lesions 4, 18
middle of 14
migraine 229
release phenomena 245
rule of four of 13
tracks, posterior 326f
tumors 239
Breath-holding spells 222
Breathing dysfunction 80
British Medical Research Council 123
Bronchiectasis 369
Brucellosis 111
Bruxism 80
Bulbar weakness 381
Bulbospinal muscular atrophy 55
Bulging fontanelle 40
Bulk 4
Burst suppression pattern 245f, 488, 488f
C
Café-au-lait spots 195f
Calcium 249, 258, 299
gluconate 251
levels, monitoring 252
Calf muscles 6f
Calorie deficiency 53
Campylobacter 340, 343
Canavan disease 45, 333f
Candida 150, 152, 157
albicans 149
meningitis 150
score 158
tropicalis 149
Capsule, internal 17
Carbamazepine 146, 147, 190, 200, 207, 207f, 273, 523
Carbamoyl phosphate synthase 84, 325
Carbidopa 299, 527
Carbon dioxide, partial pressure of 167
Carboxylase deficiency, multiple 83, 87, 90
Cardiac anomalies 177
Cardiac arrest 261
Cardiac arrhythmias 256
Cardiac conduction defects 369
Cardiac disorders 173
Cardiac examination, abnormal 232
Cardiac failure 261
Cardiac hypercontractility 232
Cardiac syncope 232
Cardioinhibitory syncope 232
Cardiomyopathy 84
Cardiovascular examination, abnormal 232
Carnitine 89, 524
acylcarnitine translocase 87
metabolic disorders 84
palmitoyl transferase 87
Carotid artery, internal 174, 177, 510f
Caspofungin 159
Cataracts 44, 45
Catastrophic childhood epilepsy 273
Catastrophic epilepsies 273
Caudate nucleus 509f
Cavernous hemangioma 30
Cavernous sinus 152
lesion 4
thrombosis 378
Cavum septum pellucidum, persistent 95
Cell
count 125
differential 125
culture vaccines, adverse effects of 446
developmental abnormalities of 28
migration, abnormalities of 28
multiple types of 81
sparse layer 93f
Centers for Disease Control and Prevention 71
Central cerebellar white matter 326f
Central coordination disorder 46
Central disorder 44
family history of progressive 43
progressive 53, 329
Central hypotonia 58f, 377t, 402t, 403b, 405b
Central nervous system 10, 20, 50, 101, 121, 128, 153, 164, 178, 183, 193, 244, 247, 268, 284, 293, 330, 332, 339, 375
affection of 327
aspergillosis 157f
classification of 123t
demyelinating disorders of 339
disease 151
infection 33
involvement 440
malformations, congenital 255
mucormycosis 151
primary 174
treatment of 132
tuberculoma, presumptive 130
Central nonprogressive disorders 55
Central sulcus 509f, 512f
Central venous
catheterizations 170
pressure, high 255
Centrotemporal spikes 194, 205, 278, 484, 487f
Cephalocele 21
Cephalometry 37
Cerebellar agenesis 25
Cerebellar aplasia 26
Cerebellar ataxia
autosomal dominant 373
postinfectious 370
Cerebellar atrophy 427f
Cerebellar cistern, superior 511f
Cerebellar deficits, presence of 2
Cerebellar degeneration, severe 373f
Cerebellar development, disorder of 20, 25
Cerebellar dysfunction 43
Cerebellar hemisphere 510f, 511f
Cerebellar hypoplasia 30, 96, 99
Cerebellar palsy 59
Cerebellar pathology 240
Cerebellar peduncle, inferior 15
Cerebellar signs 18, 51
Cerebellar systems 8
Cerebellitis, acute 370
Cerebellopontine 239
angle 510f
lesion 4
Cerebellum 15, 16f, 29f, 353, 508f, 511f
affection of 235
downward displacement of 277f
Cerebral angiography 175
Cerebral aqueduct 13
Cerebral arteriopathy, transient 174
Cerebral artery
anterior 174, 177, 297
middle 174, 177, 179, 297, 507
posterior 174, 177, 297
Cerebral autosomal dominant arteriopathy 339
Cerebral blood
flow 293
imaging 504
Cerebral edema, vasogenic 112
Cerebral function monitoring 250
Cerebral hemispheres 25
Cerebral malaria 101, 106, 113, 135
acute 187
diagnosis of 136
Cerebral palsy 34, 43, 52, 57, 58f, 59, 302, 303, 306, 307b, 309, 312, 318f, 329, 354
diagnosis of 304b
early signs of 51f
features of 59
management of 60
natural history of 61
prevention of 60
types of 57, 304
Cerebral peduncle 508f, 511f
Cerebral perfusion pressure 284
Cerebral vessels
thrombosis of 151
vasculitis of 151
Cerebrospinal fluid 11, 21, 54, 88, 91, 104, 104t, 106, 126, 127, 136, 143, 167, 169, 187, 200, 246, 260, 261, 267, 268, 284, 294, 365, 373, 382, 391, 392, 396, 399, 400, 468, 468t, 469, 471t
antigen 155
examination 137, 155
interpretation 467
normal 126t
pathway 467f
rhinorrhea 101
sampling 125
testing 125
Cerebrovascular accidents 136, 369
Cerebrovascular disease 130
Cerebrum 18, 28f
Cervical spine, upper 132
Charcot-Marie-tooth disease 56
Chédiak Higashi syndrome 45
Chelation therapy 90
Chemotherapy 133
success of 127
Cherry-red spot 45, 462
Chest
radiography 155
X-ray 124
Chiari malformation 25, 26, 27f, 240
Chikungunya 105, 111, 112
Child abuse and neglect 78
Child Development Center 49
Childhood arterial ischemic stroke 172, 173t
Childhood autism rating scale 75
Childhood disintegrative disorder 71, 79
Childhood epilepsy 487
management of 265
with centrotemporal spikes 205
Childhood headache patterns 414
Childhood neurotuberculosis 121
Cholestatic jaundice 85
Chorea 352, 353
classical signs of 353
Choreoathetoid
cerebral palsy 59
movement 112
Choreoathetosis 226, 322
Choreoathetotic movements 75
Chorioretinitis 45
Choroid plexus 511f
Chromatography, thin layer 88
Chromosomal abnormalities 53
Chromosomal disorders 34
Chromosomal disturbances 56
Chromosomal microarray 65, 91
analysis 55, 67
Cinchona alkaloids 138
Cingulate gyrus 12
dysplasia of 28
Cisternal puncture 470
Clonazepam 299, 525
Clonic activity, unilateral 206
Clonidine 526
Clozapine 526
Coadministration of steroids 147
Coagulation system 81
Coagulative necrosis 176
Cobalamin 89, 429, 430, 432
Cobblestone lissencephaly 95
Cocaine 255
Coccidioides 150, 153, 158
immitis 149, 151
posadasii 151
Coccidioidomycosis 151, 154, 160
Coccidioidosis 152
Cochlear implants 101
Cockayne syndrome 45
Coenzyme-Q 89
Cogwheel rigidity 5
Collagen vascular disorders 109
Colliculus 511f
Colpocephaly 23
Coma 138, 164, 259
causes of 137, 165b
etiology of 168t
pentobarbital 259
Communication disorders 75
Complete blood count 54, 103, 166, 177, 249, 260
Complex febrile seizure 184
Complex lipid metabolic disorders 82
Complex molecules, disorders of 82
Confusional migraine 229
Congenital syndromes 36
Conjugate eye movements 315f
Conjunctival telangiectasia 427f
Connective tissue diseases 177
Consciousness
abnormal states of 489
loss of 103f
Constipation 75, 217
Contraceptives 200
Convulsions 138, 269
management after stopping of 259
Convulsive activity 232
Copper
deficiency 437
histidine 89
Cord myelitis 345f
Corneal opacity 45
Cornelia de Lange syndrome 45, 78
Corona radiate 512f
bilateral 116f
Coronal craniosynostosis
bilateral 39
unilateral 39f
Corpus callosal agenesis 53
Corpus callosotomy 218
Corpus callosum 23, 35f, 347f, 509f
agenesis of 22, 23f, 95
lipoma of 23, 27
malformations 36
splenium of 511f, 512f
Cortical function tests, higher 2, 3
Cortical sensory signs 9
Cortical thumb, unilateral 58
Corticobulbar disruption, bilateral 29
Corticocerebellar fibers pass 59
Corticopontocerebellar pathway 15
Corticospinal fibers, stretching of 29
Corticospinal tract 13
of spinal cord 10
Corticosteroids 262
Corynebacterium diphtheriae 385
Cover test 51, 52f
Coxsackie virus 381
Cranial fossa
anterior 507
middle 505, 507
posterior 507
Cranial index 37
Cranial nerve 24, 11, 13, 14, 26
abnormalities plus 4
distribution of 13f
examination 50
lesions 4
signs 113
Craniofacial syndrome 39
Craniosynostosis 39
management of 40
symptoms of 40
types of 39, 39f
C-reactive protein 103, 258
Creatine 90
kinase 299, 392
metabolic disorders 84
phosphokinase 284, 378
transport, disorders of 75
Creatinine metabolites 54
Cri-du-chat syndrome 78
Crouzon's syndrome 39
Cry high-pitched 40
Cryptococcal antigen testing 126
Cryptococcal meningitis 126
Cryptococcomas 151
Cryptococcosis 151, 154, 159
Cryptococcus neoformans 149151
Cutaneous lesions 2
Cyanosis, attacks of 227
Cyclic vomiting syndrome 230
Cyclical vomiting, episode of 230
Cyproheptadine 230
Cyst
center of 143f
porencephalic 28, 37f, 61
pseudoporencephalic 28, 29
Cystathionine 90
Cysteamine 90
Cystic encephalomalacia 35f
Cystic fluid, high intensity of 143
Cysticercosis 111
ophthalmic 141
Cysticercus cyst, stages of 141
Cysticidal drugs, use of 140, 145
Cysticidal therapy, administration of 270
Cystinuria 90
Cytomegalovirus 43, 45, 111, 192, 202, 343
congenital 97
infection, periventricular calcification of 515f
Cytotoxic cerebral edema 112
D
Daily learning, activities of 318
Daily living, activities of 317
Dancing eyes-dancing feet syndrome 363
Dandy-Walker
malformation 27, 27f, 36, 37f
syndrome 25
variant 30
Dantrolene 528
sodium 60, 61
Day care attendance 186
De novo copy-number variation, carriers of 80
Decompressive craniectomy 296
Deficiency diseases 430t
Dehydration 255
Dehydrogenase 246
Delta rhythms, abnormal 483
Demyelinating encephalomyelitis, acute 113
Demyelination 111, 239
differential diagnosis of 348
Dengue 112
encephalopathy 106
Dengvaxia 119
Dentate nucleus 353
Denver developmental screening test 46, 313
Deoxyribonucleic acid, double stranded 178
Dermatomyositis 411
Developmental disorders 56
Developmental observation card 49
Dexamethasone, use of 128
Dextromethorphan 89
Diabetes mellitus 151, 369
Diabetic ketoacidosis 169
Diabetic mothers, infants of 246
Diarrhea 75, 217
Diazepam 60, 61, 258, 299, 310, 529
Diencephalon 12
Diffusion tensor imaging 326
Diffusion weighted imaging 504
Dimorphic fungi 150
Dinitrophenylhydrazine 88
Dinitrophenylhydrazine test 54
Diphtheritic polyneuropathy 299, 385, 394
Diplegic cerebral palsy 57
Disconjugate eye gaze 136
Disease-specific therapy 90
Disopyramide 232
Disseminated intravascular coagulation 256
Disuse atrophy 4
Disuse hemiatrophy 4
Dix-Hallpike maneuver 238f
Dizziness 234
Dolichocephaly 38, 40
Doose syndrome 275, 278
Dopa responsive dystonia 307, 357, 360, 361
Dopa sensitive dystonia 53
Double cortex 93, 96
syndrome 25f
Down syndrome 53, 54, 78, 193f
Downbeating nystagmus 240
Doxycycline 118
Dravet syndrome 184, 213, 217, 273, 275, 278
mutation 186
variant of 185
Drug levels 169
Drug resistance, mechanism of 214
Drug resistant epilepsy 279, 280
Drug therapy 256
Drug transporter 214
Duchenne muscular dystrophy 55, 307, 379, 409
Durck's microgranulomata 136
Dyke-Davidoff-Masson syndrome 59
Dysarthria 206, 226
Dyscalculia 68
Dyselectrolytemia 102, 381
Dysgenesis 251
Dysgraphia 68
Dyskinesia 352
paroxysmal kinesigenic 354
Dyskinetic cerebral palsy 306, 308f
Dyslexia 66, 68
Dysmorphic facies 34
Dysmorphism 80
Dysostosis multiplex 44, 329f
Dyspeptic dystonia 358
Dysplasia, cortical 24, 25
Dystonia 5, 113, 309, 310, 352, 353
signs of 52
Dystonic reaction, acute 356, 356f
Dystonic spells 225
Dystrophia myotonica 498f
Dystrophy, muscular 10, 54, 375f, 396, 409
E
Ear, anatomy of inner 494f
Eating disorder 78
Echinococcosis 111
Echocardiography 378
Echolalia 72
Ectopicneurons, collection of 25
Edema
appears hypointense 143
perilesional 143, 269
Edward syndrome 56
Electrical status 271
Electrocardiogram 177, 232
Electrocardiography 166, 229, 235, 236, 260
Electroclinical seizures 243, 245
Electroclinical syndromes 196
Electroconvulsive therapy 262
Electrode placements 476
Electroencephalogram 67, 93, 103, 105, 171, 205, 206, 206f, 209f, 215, 218, 222, 245, 245f, 365, 477, 478f
continuous 250
features of 477
role of 250
Electroencephalography 23, 88, 115, 168, 184, 188, 193, 208, 210, 230, 239, 244, 250, 251, 257, 261, 266, 267, 271, 284, 296, 352, 475, 487f
activity, abnormal 483
clinical uses of 483
patterns, abnormal 483
physiology of 475
Electrographic seizures 243, 245
Electrolyte 249
imbalance 101, 381
serum 88
Electromyography 55, 226, 299, 378, 396, 495, 497, 498f
Eletriptan 418
Embryonic prosencephalic vein 30
Emergency computed tomography 104
Emergency management issues 381
Emery-Dreifuss muscular dystrophy 55
Emotional stimulation 314
Emotions, sharing of 73
Empiric antibiotic therapy 169
Empyema, subdural 104
Encephalitis 54, 109, 114t, 126, 117fc, 187
acute 109, 110
disseminated 104
cause of 110
diagnosis of 113
etiology of 109
incidence of 110
major symptoms of 103f
syndrome, acute 102, 109
Encephalocele 277f
Encephalo-duro-arterio-myo-synangiosis 179
Encephalo-duro-arterio-synangiosis 179
Encephalomyelitis
acquired demyelinating 339341
acute disseminated 109, 170, 514
multiphasic demyelinating 342
Encephalopathy 35, 83, 83f, 114t, 178, 323t, 339, 364
acute 87, 102b, 322, 324fc, 325fc
chronic 255, 327, 330fc, 332fc, 331fc
metabolic 105, 136, 354
mitochondrial 229, 325
sepsis-associated 105
Endocrine 53
disorders 230
system 81
Endoscopic third ventriculostomy 129
Endotracheal intubation 295
Energy deficiency 83
Energy metabolism, disorders of 82
Enflurane 255
Enteric encephalopathy 106, 111, 126
Enterovirus 111, 118
Enzyme replacement therapy 90, 338
Enzyme-linked
immunosorbent assay 124
immunospot 125
immuno-transfer blot 144
Epidermal nevus syndrome 99
Epigastric hernia 227
Epilepsia partialis continua 276
Epilepsy 45, 54, 75, 192, 195, 219t, 271, 273, 276, 281t, 309
abdominal 229
absence juvenile myoclonic 194
atypical benign partial 208
awareness 284
benign 194, 205, 278
childhood 484
frontal 205
occipital 205
partial 205, 210
rolandic 270
childhood absence 201f, 270, 271
classification of 197fc
diagnosis of childhood 265
early-onset childhood 270
family history of 190, 192
generalized 185, 195, 197, 198
higher prevalence of 36
juvenile
absence 271
myoclonic 271
mainstay of management of 213
poorly controlled 272
risk factors for 190
risk of 251
surgery 204, 218, 218b, 263fc
symptomatic 213
syndrome 184, 192
severe 273
uncontrolled 271
Epileptic encephalopathy 272, 273
early infantile 248
Epileptic seizures 239, 245
Epileptic syndromes 248
identification of 205
Epileptiform discharges, abnormal 484f
Epileptogenesis, multifocal 218
Episodic ataxia 371
Episodic hyperpnea 369
Epley maneuver 238
Epstein-Barr virus 111, 340
Erythrocyte 135
sedimentation rate 169, 177
Erythromycin 200, 231
Esotropia 44
Estimated tuberculosis incidence rates 121f
Ethambutol 128
Ethosuximide 200, 530
Ethylenediaminetetraacetic acid 91, 167
Exotropia 44
Extensor plantar 8, 59
Extradural hematoma 381
Extramedullary lesions 389, 391t
Extrapulmonary aspergillosis 151
Extrapulmonary tuberculosis 121, 125
Extrapyramidal movements 113
Extreme distress 72
Eye
and developmental disorders 45
anomalies 177
convergence 14
Eyebrows 2
Eyelid myoclonia 197
F
Fabry's disease 90, 340
reddish maculopapular rash of 177
Facial dysmorphism 35f
Facial palsy 4f
Faciopharyngeomasticatory diplegia 98
Factor V Leiden mutation 177
Fahr disease 354
Failure to thrive syndrome 44
Faints, history of 235
Familial ataxias 307
Familial chorea, benign 354
Familial dysautonomia 53
Familial neonatal seizures, benign 248
Farber disease 45
Farber lipogranulomatosis 45
Fasciculation 4, 19
Fatty acid
oxidation disorders 83, 84, 87, 90
polyunsaturated 262
seven polyunsaturated 42
very long chain 88, 377
Febrile convulsions 255
Febrile encephalopathy, acute 101, 106, 106t, 107f, 169
Febrile infection-related epilepsy syndrome 105, 184, 282
Febrile seizures 183, 185, 187, 188, 190, 192, 255, 268
classification of 183
family history of 192
late onset 268
long-term management of 189
plus 185, 268
related epilepsies 186
Febrile status epilepticus 184, 269
Felbamate 203, 531
Fentanyl 255
Ferric chloride 54
Fetal malnutrition 53
Fever
cause of 187
low grade 122
related epilepsies 183, 184, 186, 187
symptom of 101
treatment of cause of 188
Figure of eight appearance 24f
Fine motor 3
development 47, 48t
skills 15
Fine-needle aspiration cytology 124
Finger agnosia 3
Fires, management of 106
First unprovoked seizure, management of 272
Fixation off sensitivity 210, 210f
Flaccid myelitis, acute 384
Flaccid paralysis
acute 109, 380, 381t, 389
causes of acute 383
Flaccid paraplegia 386
causes of 387
Flash technique 493f
Flexor plantar response 7
Flexor spasm 390
Floppy infant 376, 401
Fluconazole 159
Fluid attenuated inversion recovery 511, 512
Fluid replacing cerebral tissue 29f
Fluid-attenuated inversion recovery 341
Flunarizine 226
Fluorodeoxyglucose-positron emission tomography 218
Fluorquinolone 118
Focal cerebral pathology 488
Focal clonic seizures 244
Focal cortical
dysgenesis 92
dysplasia 98
Focal neurologic signs 101
Focal neurological deficits 154
Focal seizures 198, 206
migrating 273
Focal traumatic injury 244
Folate 432t
deficiency 432
Folic acid 89
supplementation 277
Folinic acid 89
responsive seizures 83, 246
Foot, dorsiflexion angle of 46
Foramen
jugular lesions 4
magnum level 506f
of Monro 506, 508f, 511f
Forebrain, midline malformation of 20, 22
Fossa anomalies, posterior 177
Fossa mass, posterior 509
Foveal hypoplasia 465
Fragile X syndrome 36, 78, 79f, 80
Fragmentary myoclonic jerks 249
Friedreich ataxia 45, 369
Frontonasal meningoencephalocele 21f
Frontoparietal polymicrogyria 97f
Fukuyama congenital muscular dystrophy 53, 95
Full blood count 167
Fundus 460
Fungal culture 126
Fungal infection 118, 150
of central nervous system 149
Fusiform gyrus 11
G
Gabapentin 203
Gait 8
circumduction 8
Galactomannan index, serum 156
Galactose, disorders of 82
Galactosemia 45, 84
Galactosialidosis 82
Gamma-aminobutyric acid 186
ergic systems 242
Ganglia hyperintensities, bilateral basal 308f
Ganglioneuroma 363
Gangliosidosis 45, 82, 84
Gas chromatography mass spectrometry 81, 88, 267, 323
Gastroenteritis 269
Gastroesophageal reflux 228, 249, 309
disease 75
Gastrointestinal disorders 75
Gaucher disease 78, 90
Gaze palsy 18
Genetic 93, 356
disorders 307
epilepsy 268
generalized epilepsies 270, 271
syndromes 355
testing 55, 80, 227
Genomic deoxyribonucleic acid 124
Genu of corpus callosum 509f, 511f
Giant cell arteritis 67
Girdle-like sensation 390
Glasgow coma scale 103, 113, 166, 167
modified 136, 295
Glaucoma 45
Glioma 131
Globus pallidus 12
Glucose 251, 258
infusion rate 169
transporter type 1 syndrome 246
Glutamic pyruvic transaminase, serum 129
Glutaric aciduria 36, 82, 83, 307, 325f, 354
Gluteus maximus 6f
Glycine 90
encephalopathy 246
transporter 227
Glycogen storage
diseases 53
disorder 83, 84
Glycopyrrolate 61
Glycosaminoglycans 54
Glycosylation
congenital disorders of 82, 87
disorders of 34, 54
Granular nodular stage 141
Granuloma 132, 154, 269
Granulomatous disease, chronic 150
Gray matter disorders 329f, 329t
Guanidinoacetate methyltransferase 90
Guillain-Barré syndrome 299, 300, 375, 380, 383, 387, 391, 392, 393, 400
Gyrus rectus 511f
H
H1N1 encephalopathy 106
Haemophilus influenzae 104, 269
Hair
abnormal 44
and developmental disorders 45
Hallervorden-Spartz disease 354
Hallervorden-Spatz syndrome 45
Haloperidol 299, 532
Halothane 255
Hand flapping 75
Hartnup disease 371
Hashimoto's encephalopathy 169
Head
abnormal shape of 37
circumference 2, 34
abnormal 33
measurement 33, 33f
growth, abnormal 34
injury, history of 33
trauma 136
Headache 229, 414, 416
classification of 414
courses of 415f
primary 415
secondary 417
symptom of 101
tension-type 416, 416b, 419
Hearing impairment 78
undiagnosed 64
Heart
disease, cyanotic congenital 36
rate 166
Heel gait 6f
Helical computed tomography 500
Heller dementia 79
Hemangioma 177
Hematocrit 137
Hematogenous spread 152
Hematological disorders 296
nonmalignant 151
Hematological malignancies 149, 150
Hematoma
nontraumatic subdural 325f
subdural 36
Hematopoietic stem cell transplantation 151, 156
Hemiatrophy 2
Hemiballismus 352
Hemimegalencephaly 28f, 93, 98, 99f
Hemiparesis 2
Hemiplegia
episodes of bilateral 225
recurrent attacks of 225
repeated attacks of 225
Hemiplegic cerebral palsy 307f
Hemiplegic migraine 229
Hemispheric dominance 12
Hemispheric dysplasias 280
Hemisyndrome 52f
Hemodialysis 87
Hemoglobin 137
Hemophagocytic lymphohistiocytosis 151
Hemorrhage
parenchymal 180
periventricular 246
subarachnoid 11, 106, 144, 180, 244
subdural 114
Hemorrhagic conjunctivitis 113
Hemorrhagic strokes 172, 180
meta-analysis of 180
Hemorrhagic transformations 176
Heparin, unfractionated 180
Hepatic encephalopathy 113
Hepatic failure 135
Hepatitis, management of drug-induced 129fc
Hepatosplenomegaly 44, 85, 328, 331, 369
Hepatotoxicity, development of 128
Hereditary ataxias 372
Hereditary motor
and sensory neuropathy 43f
sensory neuropathy 43, 43f, 375, 377379
Hereditary spastic paraplegia 62, 307
Heredopathia atactica polyneuritiformis 372
Hernia, umbilical 227
Herpes encephalitis, early stages of 115f
Herpes simplex 45
encephalitis 104, 110, 168
favor of 105
virus 106, 111
encephalitis 491f
Herpes virus group 111
Herpes zoster 340
Herpetic lesion 112
Heterotopia 24, 25, 92, 96
periventricular nodular 96
subcortical band 93, 94, 94f
Hexosaminidase A 55
Hip, congenital dislocation of 227
Hirsutism 45
Histoplasma 153, 150, 155, 158
capsulatum 149
spores of 152
Histoplasmosis 151, 154, 160
Hoffman's reflex 8
Holoprosencephaly 22, 27, 45
Homocysteine 430, 432
Homocystinuria 45, 90
Horn cell
anterior 299, 375f, 381
disorders, anterior 62
involvement, anterior 113
lesion, anterior 19, 387
myelitis, anterior 381, 383
HSV encephalitis 106
Human immunodeficiency virus 43, 65, 102, 124, 149, 174, 247, 339
associated tuberculosis 124
encephalopathy 106
infection 78, 121
advanced 151
positive 121, 128
status 130
Human leukocyte antigen 179
Human-to-human transmission 443
Hunter syndrome 322
Huntington's disease 354
Hydranencephaly 29
Hydrocephalus 26, 27, 36, 37f, 45, 95, 104, 130, 141, 386
Hydrogen, power of 167
Hydrophobia 113
Hygroma, subdural 28f, 37f
Hyperammonemia 81, 83, 84fc, 86, 90
transient 84
Hyperbilirubinemia 59
indirect 57
Hyperbrachycephalic head shape 38
Hypercholesterolemia 217
Hyperekplexia 227, 248
Hyperesthesia, zone of 390
Hypergammaglobulinemia 135
Hyperglycinemia, nonketotic 83, 88, 273
Hyperkalemic periodic paralysis 299
Hyperlactatemia, congenital 83
Hypermagnesemia 381
Hypernatremia 130
Hyperoxaluria, primary 90
Hyperpyrexia 101, 138, 255
Hypersalivation 208
Hypertension 103, 104, 255
Hypertensive encephalopathy 102, 113, 169, 170t
Hyperthyroidism 354
Hypertonia 83
Hypertonic saline 295, 296
Hypertrophic dysplasia of brain 20, 28
Hyperuricemia 217
Hyperventilation 485f
effect 482f
Hypocalcemia
early 246
late 246
Hypoglycemia 84, 86, 101, 130, 135, 136, 138, 217, 246, 249, 255, 256, 354
neonatal 274
Hypoglycemic seizures 85
Hypogonadism 45
Hypokalemia 381
Hypokalemic paralysis 385
periodic 396
Hypomagnesemia 354, 381
Hypomelanosis of Ito 45, 78, 79f
Hypomelanotic skin lesions 423f
Hypomyelination 95
Hyponatremia 130, 256
treatment of 118
Hypoparathyroidism 203f
Hypoperfusion, placental 97
Hypopigmentation 45
Hypoplasia 95
of cerebellum 26f
of optic nerve 23
Hypoplastic corpus callosum 95
Hypotension 101, 103, 256, 261
orthostatic 234
presence of 116
Hypothermia 262
Hypothyroidism 45, 53, 65
Hypotonia 80, 226, 306
generalized 75
of trunk 50
peripheral 58f, 377t, 402t, 403b, 404b, 405b
Hypoxia 59, 101, 255, 354
ischemia, perinatal 97
perinatal 57, 354
Hypoxic ischemic
brain injury 214
encephalopathy 45, 53, 245, 246, 267, 274, 368, 396
history of 192
Hypsarrhythmia 201f
I
I-cell disease 82
Ichthyosis 45, 369
Icterus 113
Idiopathic neonatal seizures, benign 248
Idiopathic torsion dystonia 307, 354, 359, 361
Immune neuromuscular disorders 55
Immunodeficiency syndrome, acquired 45, 121, 149
Immunoglobulin 532
A 372
administration 445
G 399
Immunomodulatory therapy 262
Immunosuppressant therapy 175
Immunosuppressive drugs 150, 151
Implantable prosthetic devices, use of 150
Inappropriate antidiuretic hormone, syndrome of 116
Incontinentia pigmenti 45, 54
Infantile
hemiplegia 226
mimics 222
neuroaxonal degeneration 53
Infantogram 91
Infection 111, 133, 304
acute 370
control of 133
droplet 112
prevention of 133
risk of 151
Infectious Diseases Society of America 159
Infectious myelitis 381
Inflammatory demyelinating polyneuropathy
acute 383
chronic 378
Inflammatory demyelinating polyradiculoneuropathy, acute 378, 381
Inflammatory myopathies 299, 375f, 410
Inflammatory myositis 381
Influenza 118
myositis 411
virus 111
Inguinal hernia 227
Intellectual developmental disorder 72, 74
Intellectual disability 63, 65t, 72, 75
Intellectual quotient, standardized tools of 63
Intensive care unit 149
Intensive teaching techniques 69
Intensive treatment unit 284
Interferon gamma 151
release assays 125
Interhemispheric fissure 509f
International Categories of Head Shapes 38
International Classification of Headache Disorders 237, 415, 416
International Collaborative Infantile Spasms Study 274
International League Against Epilepsy 183, 205, 243, 254, 265
Interpeduncular cistern 511f
Intoxication 136
disorders 82
Intracerebral hemorrhage 54, 180
Intracranial aneurysms 173
Intracranial content, herniation of 21
Intracranial infection 104t, 169t, 249
symptoms of 101
Intracranial mass 307
Intracranial pressure 60, 129, 140, 153, 167, 170, 259, 284, 293, 294, 322
monitoring 294
Intracranial space occupying lesions 60, 255, 369, 370
Intracranial tumors 36
Intracranial vasculitis 174
Intracranial vessels 177f
Intracranila pressure 170
Intractable epilepsy 213, 221fc
Intractable seizures 213
Intradermal inoculation 447f
Intramedullary lesions 389, 391
Intraparenchymal bleed, presentation of 181
Intrathecal baclofen 61
Intrauterine disorders 34
Intrauterine growth
restrictions 57
retardation 82, 246
Intrauterine infection 36, 53, 54, 246
intracranial calcification of 35f
Intrauterine period 82
Intrauterine stroke 53
Intravenous dexamethasone 128
Intravenous immunoglobulin 262, 284, 300, 366, 392
therapy 366
Intravenous macrolide 118
Intraventricular cyst 144f
Intraventricular hemorrhage 36, 244, 252
Intraventricular tumor 510
Invasive fungal infections, incidence of 150
Ipsilateral cerebral atrophy 426f
Iron supplementation 225
Ischemia, vertebrobasilar 239
Ischemic attack, transient 179
Ischemic stroke 54, 172
Isoniazid 127129, 255
hydralazine 435
Isovaleric academia 83, 87
Itraconazole 159
J
Jack in box tongue 354
Jactatio capitis 228
nocturna 228
Japanese encephalitis 101, 104, 106, 110, 113, 381
virus 110
Jerky, rapid flowing movements 353
Jitteriness 222, 248, 249
Joubert's syndrome 25, 26, 26f, 45, 369, 372f
K
Kanner's autism 56
Kearns-Sayre syndrome 325
Kernicterus 53, 59, 354
Ketamine 255
Ketogenic diet 106, 217, 217b,262, 279
classic 280
Ketosis 86
Kidney function test 166
Kinky hair 45
Kinsbourne syndrome 363
Klippel-Trenaunay syndrome 99
Krabbe disease 45
Kufor-Rakeb disease 358
Kyasanur forest disease 111
L
Lacosamide 203
Lactate 137, 249
dehydrogenase 379
serum 54, 80
Lactic acidosis 178, 229, 325, 325fc, 339
Lamotrigine 203, 356
Landau reflex 8
Landau-Kleffner syndrome 207, 273
surgical management of 219
Language development 74b
L-arginine 89
Latent tuberculosis infection 145
Laurence-Moon-Biedl syndrome 45
Learning disabilities 22
Learning disorder 68, 78
Leber optic atrophy 45
Legs, bilateral spasticity of 57
Leigh's disease 84
Leigh's syndrome 45
Lennox-Gastaut syndrome 213, 217, 219, 272, 275, 280, 486f
Lens
defects 95
dislocation 45
Leopard syndrome 45
Leptomeningeal glioneuronal heterotopias 97
Leptospirosis 105, 111, 113
Lesch-nyhan syndrome 75, 78, 307, 354
Lesion, cortical 17
Leukemia, acute 150
Leukocyte
count, differential 144
polymorphonuclear 104, 400
Leukodystrophy 36
metabolic 307
metachromatic 45, 307, 333f, 514
Leukoencephalopathy 339
progressive multifocal 104, 339
Leukomalacia
bilateral periventricular 307f
periventricular 53
Levetiracetam 203, 538
Levocarnitine 524
Levodopa 299, 529
Limb
girdle muscular dystrophies 56, 379
hypertonia 85
synchronously 222
Limbic system 12
Lipid storage disease 53
Lipoidosis, progressive 53
Liquid chromatography, high performance 88, 323
Lissencephaly 24, 24f, 53, 92, 93, 93f, 96, 99
Listeria 111
Liver
cells 135
enzymes 299
failure 85
function 103
routine assessment of 128
test 166, 167, 177, 258
transplantation 90
Lobar holoprosencephaly 22f
Lorazepam 258
Lorenzo's oil 42
Louis-bar syndrome 372
Low glycemic index therapy 280
Low molecular weight 178
heparin 298
use of 178
Lowe syndrome 45
Lower motor neuron 4, 17, 18, 50, 327, 375, 391
disorders 62, 299
localization 19
type of lesion 387
weakness 6
L-serine 90
Lumbar plexus injury 387
Lumbar puncture 103, 124, 131, 169, 171, 188, 249, 469471
Lyme disease 111
Lymphocytic leukemia, acute 157f
Lysosomal storage
diseases 53, 54, 354
disorders 81
M
Macrocephaly 36, 44, 45, 194f, 325f
causes of 34, 36
management of 36, 38fc
Macrophage dysfunction 150
Macular coloboma 465
Macular degeneration 45
Magnesium 249
Magnetic resonance
angiography 175, 235, 236, 503
findings 513
imaging 24, 36, 54, 80, 94, 104, 124, 140, 146, 168, 171, 177, 177f, 180, 184, 199, 203f, 207, 218, 235, 236, 248f, 261, 266, 294, 307f, 308f, 323, 326, 365, 370, 377, 382, 424f, 427f, 501
functional 326, 503
spectroscopy 326, 504
scan 143
Maintenance therapy 251
Malaria 118
Malarial parasite 107
Malformation, congenital 396
Mammillary bodies 12
Mammillothalamic tract 12
Mannitol 296, 539
Mantoux test 145
Maple syrup urine disease 65, 83, 85, 87, 90, 326
Marginal glioneural heterotopia 96
Marginal zone heterotopias 97
Mass lesions-abscess 154
Masturbation, cycle of 228
Maternal disorders 53
Maternal drug 97
Maternal infection
history of 43
prevention of 60
Mean arterial pressure 167, 284
Measles
encephalopathy 106
mumps, and rubella 30
Mechanical ventilation 457
Medial longitudinal fasciculus 13
Medial orbital gyrus 511f
Medical marijuana 216
Medical Research Council Scale 6
Medical Research Council Staging of Tuberculous Meningitis 123
Medically intractable epilepsy 214b
Medulla 4, 13
section of 15f
Megalencephaly 36
Melatonin 77
Meniere disease 236
Meningeal layers, organization of 11f
Meningeal signs 113
Meninges 11
Meningitis 36, 54, 104, 246
major symptoms of 103f
purulent 126
pyogenic 106
tubercular 106, 123, 128
tuberculous 104, 112, 123, 123f, 124t, 128, 129
Meningocele, occipital 21f
Meningococcemia 101, 112, 113
Meningoencephalitis 101, 136, 151, 214, 255
tuberculous 101, 174
Meningoencephalocele 21
Meningomyelocele 36
Menkes disease 45
Mental deficiency 45
Mental disorders 71
statistical manual of 56, 71
Mental function testing, complete higher 2
Mental retardation 3, 22, 354
Mesencephalic-Pontine junction 95
Mesial temporal
lobe epilepsy 276
sclerosis 280
Mesocephalic head shape 38
Metabolic
crisis 81, 83, 87
recurrent 83fc
disorder 75, 296, 373f
dysfunction 57
Metabolism 127
inborn errors of 53, 81, 82, 87, 88, 90, 115, 168, 244, 261
treatable inborn errors of 65, 66t
Metabolites
abnormal storage of 53
exogenously, deficient 90
Metaiodobenzylguanidine 371
scintigraphy 365
Methemoglobinemia, congenital 373f
Methyl malonic aciduria 326f
Methylcrotonyl-CoA carboxylase 87
Methylene tetrahydrofolate reductase 174
Methylmalonic
academia 83, 87
acid 430
acidemia 90
aciduria 90
Methylprednisolone 549
pulses 366
Metoclopramide 356, 356f
Metronidazole 255
Microbiological cure 127
Microcephalic vera 34
Microcephaly 34, 45, 193f
degree of 34
primary 34
Microlissencephaly 96
Midazolam 257, 285, 540
Midbrain 4
tectal plate of 508f
Migraine 229, 239, 415, 415b
abdominal 229
diagnostic criteria of 415b
headaches, family history of 230
Migration disorders 24, 36
Miller-Dieker syndrome 24, 94, 94f
Miller-Fisher syndrome 378
Minimal brain tissue 21f
Mitochondrial disease 45, 53, 55
Mitochondrial disorders 34, 53, 75, 307
Molar tooth
appearance, classical 26f
sign 372f
Molds 150
Molecular testing 55
Monoamine oxidase 361
Monosodium glutamate 230
Mood disorders 77
Moro reflex 8
Motor
action potential, compound 379
area, primary 11
automatisms 244
axonal neuropathy, acute 381, 383
development 47
fasciculotomy, selective 61
function, quantity of 44
milestones, loss of 43
pathway 13
profiles, abnormal 75
regression, late 80
skills, asymmetry of 46
speech 11
stereotypes 75
system, examination of 4
tics 75
unit
disease 53
disorder 44, 55
recruitment 499
weakness, causes of acute onset 299t
Mouse lymphoma assay 67
Movement disorder 351, 353t, 354t, 356, 359361
acute 293, 298
drug-induced 355
drugs in 361t
paroxysmal 354
phenomenology of 352t
Moyamoya pattern 174, 175
Mucolipidosis 45, 84
Mucopolysaccharidosis 36, 45, 322, 329f, 331
Mucormycosis 111, 149, 153
Multidetector row computed tomography 500
Multidrug resistance transporter protein 1 214
Multiple acyl-CoA dehydrogenase deficiency 87
Multiple lentigines syndrome 45
Multiple sulfatase deficiency 45
Multisystem disorder 55
Muscle 299
artifacts 493f
atrophy 19, 306
biopsy 55
contraction of 499f
disease 19
disorders 56, 60
enzyme studies 55
eye-brain disease 95
fiber 375f
lengthening 61
movements, voluntary 15
power, functional assessment of 6
specific kinase 299
stiffness 304
testing 6f
tone 5, 19, 46
defective regulation of 59
wasting 4
weakness 378
asymmetric 19
Muscular dystrophy, congenital 82, 377f
Myasthenia
congenital 53
gravis 53, 375f, 380, 381, 387, 395
gravis, history of 51
Myasthenic crisis 300
Mycobacterial culture 126
Mycobacterium tuberculosis 101, 121, 125
Mycoplasma 118, 340, 381
pneumoniae 111
Myelination 92
Myelinoclastic diffuse sclerosis 345
Myelitis 132
Myelopathy
compressive 381, 382, 389, 390t
noncompressive 381, 389, 390t
traumatic 381, 382
Myesthenic syndromes, congenital 62
Myocarditis, concomitant 113
Myoclonic astatic epilepsy 213
Myoclonic encephalopathy 354
early 248, 273
Myoclonic epilepsy 336
severe 184, 275
Myoclonic seizures 244
generalized 245
Myoclonus 352, 353, 364
ataxia dementia syndrome 336
of infancy, benign 226
Myoglobinuria 261
Myopathic facies 377f
Myopathy 56, 62, 375f, 406, 408b
classification of 407b
congenital 406
critical care 118, 381
metabolic 299, 411
mitochondrial 178, 339
Myophosphorylase deficiency 55
Myositis 380, 396, 406
features of 113
Myotonia 5
Myotonic dystrophy 54, 375f, 377f, 378
neonatal 53
Myotonic muscular dystrophy 410
N
N-acetyl aspartate 97
N-acetyl glutamate synthetase 84, 325
Nails 2
Narcotics 255
Nasal meningoencephalocele 21f
National Institute of Health and Care Excellenece 170
National Vector Borne Disease Control Program 109, 135
Neck
retraction 356f
turning 3
Negri bodies 441f
Neisseria meningitidis 126
Neocerebellum 15
Neonatal brain 242
injury 354
Neonatal reflex 7
disappearance of 8
Neonatal seizures 242, 243, 249, 266
benign 196
classification of 243
management of 250, 250t, 267fc
mimics 222
prevention of 251
Neonatal sleep myoclonus, benign 222, 248, 249
Neoplasia 54
Neostigmine test 51
Nerve
biopsy 55
conduction
studies 383, 495497
velocity 378, 379
stimulation 496f
Nervous system
autonomic 11
parasitic disease of 140
routine examination of 50
Neural tissues, compression of 132
Neuritis
retrobulbar 462t
traumatic 385
Neuroacanthosis 354
Neuroaxonal dystrophy 45
Neurobehavioral disorders 277
Neurocognitive deficits 141
Neurocutaneous syndrome 36, 54, 420
Neurocysticercosis 118, 131, 131t, 140, 141, 145147, 202f, 269
types of 141
unusual presentation of 141
Neurocysticercus cyst 141f
stages of 140
Neurodegenerative disorders 2, 60, 321, 327, 334t
clinical classification of 322b
Neurodevelopmental disorder, types of 66
Neuroferritinopathy 358
Neurofibroma 195f
Neurofibromatosis 45, 54, 99, 175, 420, 421f, 422, 428
stigmata of 177
Neuroichthyosis 45
Neurologic dysfunction, evidence of 109
Neurological disorder 255, 306
group of 20
localization of 10
progressive 306b
Neurological lesion, localization of 16
Neurometabolic disorders 321
Neuromuscular diseases 375, 376t
Neuromuscular disorders 54, 375f
common 378t
Neuromuscular emergencies 293, 299
Neuromuscular junction 4, 299, 375f, 378, 381, 453f
disorder 19
Neuromyelitis optica 339, 343
spectrum disorders 381
Neuronal cells 111
primary 96
Neuronal ceroid lipofuscinosis 45, 331
Neuronal migration 92
disorder 20, 23, 34, 92
means nerve cells 92
Neuropathy 56
Neurosurgery 150
Neurotoxic envenomation, specific treatment for 456
Neurotoxic snake
bite 454b
envenomation 451
Neurotoxicity
mechanism of 452
reversal of 457
Neurotransmitter metabolites 54
Neurotropic ribonucleic acid virus 383
Neurotuberculosis 122, 133, 145
Neurulation
disorder of 20, 21
primary 92
Neutropenia 86, 150
Neutrophil, polymorphonuclear 126
Newer antiepileptic drugs, role of 250
Newer computed tomography technologies 501
Niacin 434, 434t
Niemann-Pick disease 45
Nipah virus 105
Nitrazepam 60, 61
Nitrous oxide 430
N-methyl-D-aspartate 66
Nonepileptic attack disorders 231
Nonepileptic seizure 231
Nonepileptic syndromes 248
Nonpolio enterovirus 380, 381
Nonprogressive central motor disorder 52, 329
Non-rapid eye movement 277
Nonseptate hyphae 154
Nonstereotype flexion 7
Nonverbal communication 73
Normal visual evoked potential
findings 493
pattern 493f
Nosocomial infection 118
Nucleic acid amplification test, cartridge-based 114, 124, 125
Nutrition, abnormal postnatal 53
Nutritional disorders of nervous system 429
Nystagmus 18, 27, 45, 238, 239, 369
accompanying 225
peripheral 236
O
Obsessive-compulsive disorder 77
Occipital horn, enlarged 23
Occipitofrontal diameter 37
Occipitotemporal gyrus 11
Occupational therapy 60
Ocular cysticercosis 146
Ocular movements, intermittent abnormal 226
Oculo-cerebello-myoclonic syndrome 363
Oculomotor 15
abnormalities 225
apraxia 372
Ohtahara syndrome 213, 248, 273
Olanzapine 543
Olfactory nerve 14
Oligosaccharides 54
Open lip schizencephaly 24
Ophthalmologic disorders 35
Ophthalmoplegia 45, 113, 130, 369
Opisthotonus 136
Opportunistic fungi 149
Opportunistic infection, evidence of 104
Opsoclonic cerebellopathy 363
Opsoclonus 364
myoclonus syndrome 240, 363, 363b, 365fc, 366t, 367, 370
Optic atrophy 45, 322
Optic disk swelling 460
Optic nerve 15, 511f
drusen 461
Oral antispasticity drugs 310t
Oral benzodiazepine 189
Oral contraceptives 356
Oral genital mucosa 112
Oral steroids 366
Orbit 511f
Organ transplants 149
Organic acid 249
analysis 54
disorders 323
Organic acidemias 323
Organic acidurias 82
Organophosphate poisoning 299
Organophosphorus poisoning 381, 395
Organs, multiple types of 81
Ornithine transcarbamylase 84, 325
deficiency 322
Orphenadrine 61
Oseltamivir 118
Osteomyelitis, tuberculous 398
Osteopetrosis 37f
Otitis media, chronic 101
Oxcarbazepine 203, 207, 544
Oxygenation 297
P
Pachygyria 94
degree of 94
Packed cell volume 249
Pain, recurrent abdominal 86
Paleocerebellum 15
Palliative epilepsy surgery 218
Pallidal hyperintensity, bilateral 326f
Palmar grasp reflexes 8
Panayiotopoulos syndrome 208, 209f, 270
Panencephalitis, subacute sclerosing 115, 199f, 339, 491f
Paper, repeated rolling of 79f
Papilledema 113, 462t
basis of 104
Papillitis 462t
Parachute response 8, 51
Paracoccidioides 150, 153
brasiliensis 149
Parahippocampal gyrus 4, 12
Parainfluenza virus 186
Parallel nonconverging lateral ventricles 23
Paralysis, periodic 381
Paralytic polio virus, vaccine derived 381
Paraneoplastic syndromes 109
Paraparesis 123
acute 392fc
Paraplegia 386, 387, 389, 390t
acute 389, 391fc, 392fc
chronic 392, 396t, 397t
Parasitemia 137
assessment of 136
Parasites 142t
Parasympathetic fibers 4
Parenchymal cyst, degenerating 141
Parenteral therapy, initial 137
Parietal lobule, superior 11
Parieto-occipital sulcus 11
Parotid swelling 113
Paroxysmal torticollis, benign 225
Paroxysmal vertigo, benign 225, 235, 237
Parvovirus 111
Pediatric
advanced life support 169
autoimmune neuropsychiatric disorder 352
higher cortical function testing 2
intensive care unit 170, 259, 284
Pelizaeus merzbacher disease 45
Penicillamine 545
Penicillins 255
Perilymph fistula, post-traumatic 236
Periodic lateralized epileptiform discharges 105, 168, 491f, 200f
Peripheral disorders 55
Peripheral nerve 299, 375f, 378, 381
disease 19
disorders 62
section 61
Peripheral nervous system 10, 11, 42, 327, 330, 332
Peripheral oxygen, saturation of 284
Peripheral progressive disorder 53, 332
Perisylvian syndrome, bilateral 25
Peritoneal dialysis 87
Peroxisomal disorders 82, 83
Persistent mongolian blue spots 328f
Pes cavus 43, 369
Pfeiffer's syndrome 39 39
Phencyclidine 255
Phenobarbitone 190, 273, 546
Phenylalanine, dietary restriction of 90
Phenylketonuria 45, 75, 78, 90
Phenytoin 251, 257, 273, 547
Photic stimulation 482f
Photodermatitis, severe 328f
Photosensitivity 205
Physiotherapy, management of 30
Picorna viridae 383
Pimozide 548
Piracetam 549
Placental disorders 53
Plagiocephaly 39
Plantar reflexes 7, 8
Plasma
acylcarnitine 323
amino acids 54
ammonia 88
bicarbonate 137
cells 112
lactate 88
Plasmapheresis 262
Plasmodium 111
falciparum 135
Platelet count 103
Platybasia 26
Pleomorphism 145
Pleurodynia 113
Plus lobe function 3
Poisoning 136, 255
Polio virus 381
Poliomyelitis 375, 375f, 383, 387, 393
Polygranulomatous disease 151
Polymerase chain reaction 67, 105, 104, 106, 124, 155, 157, 168, 169, 396
advent of 114
Polymicrogyria 28, 92, 97, 98
Polymyoclonus syndrome 363
Polymyositis 381, 499f
Polyneuropathy
acute 388
critical illness 381
peripheral 19
porphyritic 299
postdiphtheritic 381
Pompe's disease 53, 84, 90, 378
Pons, simplified plan of 14f
Popliteal angle 46, 313f
Porencephaly 27, 28
Porphyria 381, 396
Port wine stain 194f
Positron emission tomography 54, 200, 218, 279, 365fc, 371
Postcentral gyrus 11
Postexposure prophylaxis 442t, 443, 448
Potassium 258
Pott's disease 132
Pott's spine 132, 381
Praziquantel 146, 147
Preacademic skills, acquire 64
Precentral gyrus 11
Precentral sulcus 512f
Pregabalin 203
Prelinguistic milestones 48
Primaquine
dosages 138, 138t
single dose of 138
Primidone 535
Primitive reflexes 7, 46
Progressive disorder 43, 44
Proinflammatory cytokines, production of 135
Pronator sign 143f, 354
Prophylactic pharmacotherapy 230
Prophylaxis 189
drugs for 418t
types of 189
Propranolol 550
Prosencephalon, development of 92
Protein
C 174
calorie malnutrition 429
content 125
S 174
Proteus syndrome 99
Prothrombotic disorders 174
Pseudobulbar palsy 4, 57, 98
Pseudoseizures 231
Pseudostationary stage 80
Psoas abscess 387
Psychiatric disorders 130
Psychological stressors trigger episodes 231
Ptosis 369
Pupillary reflex 3
Pyramidal cell 476f
Pyramids 13
Pyrazinamide 127, 128
Pyridoxal phosphate 89
responsive seizures 83
Pyridoxine 88, 435, 435t
Pyruvate dehydrogenase 83
Q
Quadrigeminal cistern 508f
Quadriplegia, episodes of bilateral 225
Quantiferon gold 125
Quinine per rectal 138
R
Rabbit syndrome 358
Rabies 104, 439
immunoglobulin 449
treatment of 448
virus 440f
Radial spoke-like orientation of Gyri 23
Raised intracranial pressure 293
management of 295
signs of 116, 140, 166
Raised intracranial tension 293
Randomized controlled trials 274
Raphe nuclei 14
Rapid diagnostic test 136, 166
Rapid eye movement 248
sleep electroencephalogram 481, 481f
Rasmussen's encephalitis 280
Reactive attachment disorder 78
Reading disorder 66
Recurrent febrile seizures, prevention of 190
Red blood cell 81, 432, 433
Reflexes 7, 391
anoxic seizure 222, 224
asymmetry of 7
asystolic syncope 224
behavior 51
stimulation of antagonistic muscles 61
superficial 7
Refractory epilepsy 213, 276
management of 215
Refractory seizures 83, 85
Refractory status epilepticus 281
management of 261
Refsum disease 45, 369, 372
Regurgitation 1
Relapses, management of 347
Renal failure, acute 261
Renal function test 103, 177, 258
Respiratory distress, exhibit signs of 228
Respiratory muscle weakness 381
Respiratory rate 166, 284
Retinal disorders 463t
Retinal dysplasia 95
syndrome 95
Retinitis pigmentosa 45, 369
Retino-meningoencephalitis 111
Rett syndrome 45, 54, 71, 77, 78f, 79, 80, 307, 354, 355, 356f
Revised Tuberculosis Control Program 123, 125
Reye syndrome 86, 111, 114
Rhinocerebral disease 151, 160
Rhinocerebral mucormycosis 152, 156f
Rhinocerebral syndrome 154
Rhizomucor 151
Rhizopus 150
Rhizotomy, selective posterior 61
Rhombencephalitis, midline 104
Riboflavin 88
Rickettsia 118
Rickettsial diseases 111
Rickettsial scrub typhus 111
Rifampicin 124, 125, 127, 128
Risperidone 552
Rituximab 366
Rocky mountain spotted fever 111
Root pain 390, 391
Rubella 45, 111, 192, 202
Rud syndrome 45
Rufinamide 203
Russell's viper 454
S
Sacral sensation 391
Saliva, drooling of 309, 310
Salivary ducts 61
Salt-and-pepper fundus 465
Sandifer's syndrome 358
Scalp hair 2
Scaphocephaly 39, 40
Scarf sign 46, 313
anterior 50
Schilder's disease 345
Schizencephaly 24, 28, 92, 98, 99f
Sclerosis, multiple 239, 339, 345, 381, 387, 399
Scoliosis 80, 369
Scrub typhus 105, 112, 118, 363
Seborrheic dermatitis 369
Segawa syndrome 53
Seizure 35, 40, 83, 98, 130, 140, 249t, 251, 322
activity 232
continuous 254
acute attack of 188
cause of 187, 251
clinical 243, 244
control of 188, 189
correct diagnosis of 187
diagnosis of neonatal 250
disorder 192
domiciliary management of acute 284
dominant 56
etiology of neonatal 246, 247b
generalized 195
imitator 222
management of 30, 261
metabolic 187
mimic 222, 223t, 249
neonatal electrographic 245
new onset of 103
partial 195
prophylaxis 296
psychogenic 231
recurrent febrile 189
semiology 187
subtle 244
symptomatic 255
types of 214, 244
unidentified 195
Selective metabolic testing 54
Selective serotonin reuptake inhibitors 77
Sella turcica 506
level of 507f, 511f
Sensations, complete loss of 133
Sensorium, alteration of 102
Sensory
area
primary 11
receive sensory 11
deficit 391
examination 8, 52
integration 314
loss
prominent 306
upper level of 390
nucleus 13
signs, absence of 19
stimulation 314
symptoms 73
Septo-optic dysplasia 22, 23
Serine biosynthesis 34
Serological tests 144, 155
Serum creatine kinase level 54
Sexual contact 112
Shagreen patches 423f
Shigella encephalopathy 101
Shigellosis 111
Shock 170t, 261
evidence of 101
Short-chain acyl-CoA dehydrogenase 83
Sialidosis type 45
Sickle cell disease 175, 179
Simple febrile seizure 103, 109, 183
Single cranial nerve palsies 123
Single gene tests 65
Single photon emission computed tomography 200, 279, 218, 280
Single seizure per febrile illness 183
Sino-orbital mucormycosis 152
Sinovenous thrombosis 54
Sinusitis, chronic 369
Situs inversus 462
Sjögren-Larsson syndrome 45
Skeletal dysplasia 36
Skin 44
biopsy 91
disorders 45
rash 113
test 134
Skull
contains brain 33
osteomyelitis of 152
thickness 36
Sleep 207f, 479
architecture, normal 479
disorders 75
dysfunction 77
issues 309
myoclonus 249
record 478
Slow wave sleep 271, 479, 480f
Small molecule disorders 82, 322, 324fc, 325fc
Smith-Lemli-Opitz syndrome 54
Snake
big four Indian 452f
envenomation 381
Social communication deficits 73
Social development 49
and language 3
Social emotional reciprocity 73
Sodium 258
activated channel 279
benzoate 90
dichloroacetate 89
valproate 554
Soft signs 52
Solid organ transplantation 151
Somatization disorder 231, 234, 239
Somatosensory stimuli 207
Somnolence 18
Soto syndrome 36
Space occupying lesion 36, 123, 181
Spasms 245
structural etiology of 274
Spasmus nutans 228
Spastic cerebral palsy 305
management of 62fc
Spastic diplegia 307f
severe 57
Spastic hemiplegic cerebral palsy 58
Spastic paraplegia 386
causes of 386
Spastic quadriplegic cerebral palsy 57
Spasticity 5, 27, 61, 386
dominant 56
drug treatment 61
Specific learning
disability 64, 69
disorder 63, 66
Speech
comprehension of 11
disorders 139
therapy, hearing assessment and 316f
to-text translation 69
Spider envenomation 299
Spinal arteriovascular malformation 381
Spinal cord 16, 353, 389
cross-sectional anatomy of 16
injury 396
lesions 18, 307, 390t
localization of 389
simplified organization of 12
syndrome 26
Spinal curvature 26
Spinal epidural abscess 381
Spinal lemniscus 14
Spinal muscular atrophy 4, 50, 54, 55, 58f, 334, 375f, 377, 499f
Spinal nerves 11
Spinal syndrome 154
Spinal tenderness 18
Spinal tuberculosis 132
complications of 132
treatment of 133
Spinal tuberculous arachnoiditis 130, 132
Spinal tumor 354
Spine, magnetic resonance imaging of 344f
Spinocerebellar ataxias 368
Spinocerebellar degeneration 354
Spinocerebellar pathways 13
Spinocerebellar tracts
anterior 15
posterior 15
Spinothalamic pathway 13
Sporadic lesion 112
Sporothrix species 149
Sporotrichum 153
Squeezing gastrocnemius 8
Stabilize pelvis 318f
Stagnation, stage of 79
Starry sky appearance 142, 143f
Static ataxia, chronic 371
Static encephalopathy 17
Status dystonicus 298
Status epilepticus 207f, 254, 260fc, 281, 281t, 282t, 284, 293
classification of 255
convulsive 254t
management of 254, 271
new-onset refractory 281
nonconvulsive 169, 271, 284, 371, 488
nonintravenous 259
outcome of 254
refractory 259, 262
treatment of 259
Stem cell
therapy 311
transplantation, posthematopoietic 156f
Stereotypic movements 73
Sternomastoid wasting 3
Steroids 145
role of 118, 128
Stiffness, attacks of 227
Storage disorders 369
Streptococcus pneumoniae 126, 188
immunizations 269
Striatum 353
Stroke 293, 354
episodes 178, 229, 325, 339
management, acute 296
manifestations of 297t
pediatric 172
syndrome 154
acute 172
Strongyloides stercoralis hyperinfection 145
Sturge-Weber syndrome 30, 45, 425, 475f
Subarachnoid hygroma 45
Subcallosal gyrus 12
Subependymal nodular heterotopia, bilateral 96f
Substrate reduction therapy 90
Sub-thalamic nuclei 353
Succinic semialdehyde dehydrogenase deficiency 75
Sudden infant death syndrome 39, 227
Sulfite oxidase deficiency 45, 83
Superficial temporal artery 179
Superior frontal sulcus 512f
joining precentral sulcus 512f
Supportive care 137, 2, 256
Supportive therapy 392
Supramarginal gyrus 11
Supranuclear lesion 4
Suprasegmental lesions 7
Suprasellar cistern 507f
Surgery, role of 148
Survival motor neuron 334
gene deletion 55
Sydenham's chorea 354
Sylvian fissure 11, 24f, 28, 508f, 511f
dilated 325f
shallow 94
underdeveloped 25
Sympathetic nerve cell 12
Symptomatic seizures, acute 129, 268
Syncope 231, 234
benign 231
causes of 234, 235t
recurrent 232
vasovagal 231
Synophrys 45
Syntaxin binding protein 274
Syphilis 111, 192, 202
Syringomyelia 26
Syskinesia, paroxysmal nonkinesigenic 355
Systemic inflammatory disorders 381
Systemic lupus erythematosus 114, 340, 361
T
Tachycardia 255
Taenia solium 145
Tandem mass spectrometry 81, 88, 169, 267
Tardive syndromes 356
Tegmentum 14
Temporal lobe 510f, 511f
epilepsy 185
risk of 190
seizures 198
Temporo-parieto-occipital auras 97
Tendon
jerks 57
reflexes 59
deep 7
exaggerated 386
surgical transfixation of 61
Tensilon test 51
Testing eye movement 3f
Tetrabenazine 299
Thalamic nuclei 96
Thalamus 509f
Thalassemia 36
Thermometer 189
Theta pointu alternant 248
Thiamine 88, 433, 433t, 556
Thrombocytopenia 86
perinatal alloimmune 28
Thyroid
function test 67
stimulating hormone 299
Tiagabine 203
Tics 232, 352, 353
Tilt table testing 232
Tissue
abnormal 512t
anoxia 135
infarction of 28
plasminogen activator 298
Titubation 27
Tizanidine 310
Toe flexors 6f
Tongue thrusting 369
Tonic activity, unilateral 206
Tonic clonic
convulsions, generalized 192
seizures, generalized 243, 271
tonic activity, generalized 183
Tonic neck
reflex 316
asymmetric 8, 51, 51f, 316
response 8
Tonic seizures 244
Tonic-clonic
convulsions, bilateral 209
seizures, bilateral 192, 197
Topiramate 203, 226, 556
Torticollis, paroxysmal 225
Touchdown sign 354
Tourette syndrome 232, 352, 354
Toxic encephalopathies 354
Toxic metabolites 87
reduction of 90
removal of 87
Toxic myopathies 381
Toxic neuropathies 299
Toxic screen 258
Toxic succinyl acetone, reduced production of 90
Toxicity 78
Toxidrome 169
Toxin 321, 382
drugs and 369
exposure 304
Toxoplasma 43, 45, 111
Toxoplasmosis 118, 202
rubella, cytomegalovirus, and herpes simplex virus 65
Tracheostomy, elective 118
Transilluminant sac 21f
Transverse myelitis 18, 343, 344f
acute 343, 380, 381, 384
idiopathic acute 399
Trauma 255
history of 368
Tremor 352, 353
Trichinosis 381
Trichosporon 149
Trichothiodystrophy 45
Triclofos sodium 557
Tricyclic antidepressants 255
Trientine dihydrochloride 535
Trigeminal lemniscus 14
Trigonocephaly 40
Trihexyphenidyl 61, 299, 536
Triphasic waves 105
Trivandrum developmental scale chart 313
Trypanosoma 111
Tubercular encephalopathy, tubercular 123
Tubercular meningitis, pathogenesis of 122fc
Tuberculoma 123, 130, 131f, 131t, 145, 145f
diagnosis of 130
increased risk of 130
Tuberculosis 121, 123t, 125, 128, 129, 168, 513
disease, previous history of 130
drug-resistant 124
transmission, prevention of 133
Tuberculous meningitis
complications of 129
differential diagnosis of 126
presumptive 123
sequelae of 130
Tuberous sclerosis 78, 98, 99, 177, 203
adenoma sebaceum 194f
complex 54, 423, 423f, 428
hypopigmented spots 194f
subependymal calcification of 515f
Tubulinopathy 93, 99
Tumor necrosis factor 135
Typhoid 101
Typical spin-echo sequences 503t
Typical vasovagal syncope 232
Tyrosine, dietary restriction of 90
Tyrosinemia 84, 90
U
Ultrasonography 21, 155, 249, 365
United Kingdom Encephalitis Registry 103
Upper cervical spinal cord 18
Upper motor neuron 4, 16, 51, 57, 327, 375, 386, 387, 391
weakness 6
Upper respiratory tract infection 384
Urea cycle defects 82, 323, 325, 371
Urea cycle disorder 323
exceptions of 322
Uric acid
level 54
serum 88
Urine
culture 103
ketones 91
organic acids 80, 91, 169, 323
unusual smell of 44
V
Vagus nerve stimulation 204, 215, 218, 219
placement of 220f
Valproic acid 185, 216, 257, 260
Varicella 112, 369
encephalitis 106
zoster virus 106, 174
Vascular conditions 54
Vascular lesion 354
Vascular malformation 30, 36
Vascular occlusion 58
Vascular tortuosity 465
Vasculitis 54, 55
Vasculopathy, tubercular 123
Vein of Galen
aneurysm 30
malformations 30, 31f
Vein of Rosenthal 30
Venoms 382
neutralization of 456
Venous sinus thrombosis 179, 181f
Ventilation 166
Ventricle, partial separation of 22f
Ventricular system 28
disorder of 20, 28
Verbal painful unresponsiveness 166, 167
Vermis
complete agenesis of 27
partial agenesis of 27, 27f
Vertebral column, tuberculosis of 132
Vertebral fractures 381
Vertigo 234, 235
central causes of 239
peripheral causes of 236, 237t
pharmacological treatment of 239
symptoms of 175
Vesicular rash 369
Vestibulocerebellar dysfunction 240
Vibration, contralateral loss of 13
Vigabatrin 203, 537
Vigevano maneuver 227
Vincristine 381
Viral
encephalitis 111b, 116f, 354
meningoencephalitis 106
myositis 381
Virtual endoscopy 501
Virus
actively multiplying 111
inactivation of 444f
mechanical removal of 443f
neutralization of 444f
Visceral function 14
regulation of 11
Vision testing 3
Visual evoked potentials 493
Visual hallucinations
elementary 209
nonstereotype 232
Visual impairment, severe 64
Visual loss, early 113
Visual stimulation 314
Visual techniques 69
Visual tracking, improve 315f
Vital functions
stabilization of 87
supporting 256
Vitamin 53
A 436
D 436
E 89, 436
Vojta's method 46
Vomiting 35, 40, 217
von Hippel-Lindau syndrome 427
von Willebrand's disease 28
Voriconazole 159
W
Walker-Warburg syndrome 95
Weakness
bilateral 18
grading of 6
Wernicke and Korsakoff syndromes 13
Wernicke encephalopathy 433
West syndrome 273, 274
White breath-holding attacks 224
White matter disorders 329t, 333f
Wilson's disease 45, 84, 307, 354
Worsening
clinical
signs of 91
symptoms of 91
seizures 272
Wound management 449
Wrist, arthrodesis of 61
X
Xeroderma pigmentosa 45
Y
Yeast 150, 152
Z
Zellweger syndrome 45
Zika virus 34
Zonisamide 203, 537
Zoster varicella 118
Zygomycetes 149152
Zygomycosis 153, 154
×
Chapter Notes

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Approach to Neurological Assessment in ChildrenCHAPTER 1

PAM Kunju
 
INTRODUCTION
Neurological assessment by history and examination in a child is itself an art and it can be mastered only by constant practice. As in any pediatric case, steps can be followed as per the usual teaching. However, improvization may be needed at every step. During the conversation with the guardian, observation of the child must also be done. History from the child should also not be forgotten. The aim in the neurologic assessment is to obtain an accurate localization thereby helping in the etiological diagnosis and management. By taking a neurological examination, one should try to:
  • Assimilate the pertinent findings and make a list of differential diagnoses regarding localization and etiologies. Remember—common things are common. So, exclude the less likely ones and narrow down to the possible differential diagnoses.
  • Plan the investigations and order tests systematically to confirm or support the most probable diagnosis or to exclude others.
  • Discuss the management options with the parents and establish a follow-up plan.
 
HISTORY
History is the most fundamental part of the neurologic evaluation. Most neurological disorders can be diagnosed to a considerable extend by a good history. Do not forget to ask the child about the disease. A child older than 3 years will be able to provide very useful information that is more accurate than the parent.
  • Review of the presenting illness in a chronological manner and evolution of specific symptoms like headaches, seizures, weakness, etc. must be listed and evaluated systematically.
  • For the youngster with a learning disability or an attention deficit disorder, social and environmental aspects are also to be covered.
  • Review of the developmental history necessitates a survey of antenatal, perinatal, and postnatal development.
  • Information about the feeding history other than dietary intake and calculation of calorie must be obtained. This includes failure to establish breastfeeding, regurgitation, excessive colic, or frequent formula changes. These symptoms along with abnormal sleeping habits will be an important clue for a perinatal brain injury or a developing progressive disorder.
  • The developmental milestones must always be recorded. Most mothers recall these and can compare one youngster with the siblings.
  • A system review focuses on the major childhood illnesses, immunizations, and injuries.2
  • Recurrent injuries suggest hyperactivity, impaired coordination, or poor impulse control.
  • The family history is relevant in most neurodegenerative disorders, which are transmitted as a recessive or dominant trait and that questions about the health of siblings and the presence of consanguinity are in order.
 
NEUROLOGICAL EXAMINATION
Neurological examination of child is not only different, complex, and takes longer than any other organ, but is also unfamiliar to the majority. Childhood neurological examination starts as soon as you see the child whether he is crying or smiling. Do assess the sensorium, cranial nerve (CN) and axial musculature at that observation. Similarly, observe while he walks or resists being carried. Each activity must be considered as an examination opportunity. It is important to get a good rapport with the child and to explain to the parent what you are going to do. Play and communication with the child during the examination is crucial. To gain trust and cooperation from your young patient and the parents, pay more attention to the problem; you may want to examine this area first, and if you decided to check it later, inform the parent. The comfort of the patient should be considered during the examination. For example, if the patient is lying on the bed, start with the areas that can be tested in that position, such as leg movement, leg reflexes, abdominal reflexes, sensory testing, Babinski sign, and so on. Then, examine the child in the sitting position.
 
GENERAL EXAMINATION
This includes the head-to-toe examination including vital signs and the anthropometry.
In addition to the knee hammer and the usual neurological gadgets for a pediatric neurological examination, handy instruments include a ball; a few small toys like a car that can be used to assess fine motor coordination; a bell; and some object that attracts the child's attention (e.g. a pinwheel and a flashlight with a rubber adapter for transillumination).
In the child above 5 years of age, general physical and neurological examinations can be performed in the same manner as for adults, except that the more uncomfortable aspects, such as fundus examination, corneal and gag reflexes, and sensory testing should be postponed until the end.
Child of 1–3 years of age is best approached by seating the child in the mother's or father's lap and talking to the child and infant either in the cradle or in the mother's lap as he wishes.
  • Height, weight, blood pressure, and head circumference must always be measured
  • As far as possible, examine the child undressed—but remember the modesty.
  • Look specifically:
    • Dysmorphic features
    • Cutaneous lesions
    • Scalp hair, eyebrows, and nails
    • Comparison of the size of the limbs and thumbnails (for hemiatrophy with hemiparesis)
    • Presence of an unusual body/urine odor
    • Child's dominant handedness and the presence of cerebellar deficits, hemiparesis, and perhaps even a visual field defect
    • General appearance of the skull as it gives a lot of idea about the brain inside.
 
HIGHER CORTICAL FUNCTION TESTS
Pediatric higher cortical function testing is equivalent to cognitive developmental examination. So a developmental assessment, which includes fine motor/adaptive, language and social development, means complete higher mental function testing. Before pursuing formal developmental assessment, you must establish that the patient is awake, alert, can3 hear, and comprehend. Cognitive function in older children is tested while you are taking the history. So take the history from the child than from the parent.
Check consciousness, alertness, orientation, attention, language, and memory. A detailed higher cortical function testing is done if child has mental retardation, cognitive decline, etc.
 
Steps
In youngsters, do a full developmental assessment giving more attention to cognitive (fine motor, adaptive behavior, social development and language) aspect. Thus, a developmental quotient (DQ) can be obtained.
In older child, look for all the fund of knowledge, abstract thinking, calculation, thought (FACT) process (plus lobe function, if you are conversant with it).
This will vary in an acute disease and a child who has more chronic problems including a pervasive developmental disorder. A more social behavior and play activities evaluation will be required in such situations. Peculiar repertoire/repetitive behavior will be suggestive of autism and hyperactive inattentive behavior will be in favor of attention deficit hyperactivity disorder (ADHD).
In an older child, make certain that agnosia and apraxia are also tested. Agnosia and apraxia are generally indicative of a parietal or occipital lobe lesion, provided they have learned earlier and lost it. Otherwise such cases will be seen as a case of mental retardation or learning disorder.
  • Inability to draw a clock or cross (constructional apraxia)
  • Inability to comb hair or drink through a straw (ideational apraxia)
  • Inability to name fingers (finger agnosia)
  • Inability to recognize the objects by touch (astereognosis)
  • Inability to recognize drawing number in hands (agraphesthesia)
  • Inability to understand written language (alexia)
  • Inability to write (agraphia).
 
Cranial Nerves
Detailed evaluation techniques can be obtained from any clinical methods.
Cranial nerves can be examined in all including a newborn. A proper observation of the child is the only thing you have to do.
For example:
  • Response to light and facial regard for vision testing (CN II)
  • Spontaneous eye turning to different direction and dolls eye maneuver and pupillary reflex (III, IV, VI CN) (Fig. 1)
  • Mouth opening during cry/chewing (V CN)
  • Nasolabial fold during cry/smile (VII CN) (Fig. 2)
  • Responds to sound by stop crying or startle (VIII CN)
  • Watching the throat and tongue while crying (IX, X, XII CN)
  • Neck turning and sternomastoid wasting (XI CN).
Of course, one has to do a detailed and accurate testing for an exact localization of a specific lesion if some abnormality is found in above observation.
zoom view
Fig. 1: Simple method of testing eye movement. Observe spontaneous movement. Normal extraocular movement—child looking to left and down = left lateral and right medial rectus + right superior oblique (in the adducting eye) and left inferior rectus (in the abducting eye). Opposite can be inferred from the lady's eyes.
4
zoom view
Fig. 2: Left upper motor neuron (UMN) facial palsy observed while the child was crying. Reduced nasolabial fold left and deviation of angle of mouth to right.
All CNs originate from the brainstem, except CNs I and II.
  • Midbrain—CN III and IV
  • Pons—CNs V, VI, VII, and VIII
  • Medulla—CNs IX, X, XI, and XII
  • Cranial nerves I, II, and VIII are purely sensory
  • Cranial nerves III, IV, VI, XI, and XII are purely motor in function and the remainders are mixed
  • Cranial nerves III, VII, IX, and X have parasympathetic fibers.
Certain points to be remembered—cranial nerve lesions occur due to:
  • Supranuclear lesion [upper motor neuron (UMN), facial, supranuclear eye movement abnormality, pseudobulbar palsy]
  • Brainstem lesions [lower motor neuron (LMN) type with pyramidal signs]
  • Cranial nerve lesions (isolated)
  • Neuromuscular junction (in myasthenia gravis III, IV, and VII CNS are common)
  • Surrounding structures, such as the cavernous sinus.
    • A spinal cord lesion does not give rise to symptoms and signs of CN abnormalities.
    • Cranial nerve abnormalities plus ipsilateral limb motor or sensory deficits are consistent with a lesion above that CN (only seen along with VII CN).
    • Lower motor neuron CN abnormalities plus contralateral, limb, motor, or sensory deficits means a brainstem lesion at the level of that CN.
  • A unilateral ptosis in a child with altered sensorium and motor weakness can be due to lateral transtentorial herniation (downward and medial descent of uncus and parahippocampal gyrus). It is a dire emergency.
    • Unilateral CNs V–VIII dysfunction plus contralateral motor or sensory deficit is indicative of a cerebellopontine angle lesion
    • Unilateral CNs IX–XI dysfunction without appendicular deficit is seen in foramen jugular lesions.
    • Unilateral abnormality of CNs III, IV, V, and VI is suggestive of cavernous sinus lesion.
    • Unilateral abnormality of CNs IX, X, XI, and XII is indicative of bulbar palsy.
 
EXAMINING THE MOTOR SYSTEM
Examination of the motor system:
  • Bulk
  • Tone
  • Power
  • Reflexes (superficial, deep, primitive/neonatal)
  • Coordination and abnormal movements.
 
Muscle Wasting and Fasciculation
Commonly seen in a LMN lesion, such as in spinal muscular atrophy (SMA), radiculopathy, chronic polyneuropathies, and rarely in polymyositis. Consider muscular dystrophy when there is specific muscle group wasting without fasciculations. Disuse atrophy/hemiatrophy is seen in UMN lesion.5
 
Muscle Tone
Tone is increased resistance to passive movements of the limb at the joint level. Tone examination is often neglected and this is partly because of the fact that the patient does not complain of a tone problem.
Young infants must be picked up to assess the tone—pull to sitting, lift by the axilla and then ventrally suspend. In older children, palpate the muscle and then move joints actively and passively and then grade the tone.
  • Normal tone
  • Decreased tone: Mild = hypotonia; severe = flaccid
  • Increased tone (Figs. 3A and B).
 
Tone Abnormalities
  • Spasticity: Increased tone throughout range of motion, and then there is a sudden release (catch). It is seen in UMN lesion. Spasticity is velocity dependent (sudden release).
  • Rigidity: Increased tone throughout the range of motion. If intermittent and ratchet-like, is called cogwheel rigidity which is seen in extrapyramidal diseases, such as choreoathetotic cerebral palsy. Rigidity is not velocity dependent (continuous).
  • Paratonia or gegenhalten: Increased tone appears when the patient opposes the movement of a limb. It is seen in a bifrontal lobe lesion and diffuse encephalopathy.
  • Myotonia: Delay in muscle relaxation after the muscle is activated, either spontaneously (e.g. handgrip myotonia) or induced by percussion (percussion myotonia). It is seen typically in myotonia congenita.
  • Dystonia: Contraction of agonist and antagonist muscle producing sustained abnormal limb posture. It is seen in extrapyramidal disorders.
 
Power Testing
  • Weakness assessment by functional testing and by active resistance testing depending on the age
  • The onset, course, and distribution of weakness
  • Whether the weakness is because of a UMN or LMN lesion and/or nonorganic
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Figs. 3A and B: (A) Examining adductor spasm; (B) Axillar hanging reaction to look for scissoring.
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  • Keeping both hands outstretched will give lot of information about muscle weakness (Fig. 4)
Examples of functional assessment of muscle power are given in Figures 5A to C.
 
Interpretation
  • Lower motor neuron weakness: Normal or decreased muscle tone, hyporeflexia, atrophy, fasciculation, with/without sensory deficit.
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Fig. 4: Hand drift (when arm extended) and the pronator sign (when arms are raised over the head). Note the weakness of the right upper extremity.
  • Upper motor neuron weakness: Weakness of extensors in the upper extremities and flexors in the lower extremities, hypo-or hypertonia, hyper-reflexia, clonus, and the presence of pathological reflexes.
  • Nonorganic weakness: Nonanatomical, erratic (giveaway) weakness, discrepancy between voluntary use of muscles and when tested directly, normal reflexes, tone, and sensory examination.
 
Grading of Weakness
The Medical Research Council Scale is commonly used to demonstrate the degree of weakness by manual muscle testing.
 
Medical Research Council Scale
5 Normal
4 Moderate movement against resistance
3 Movement against gravity
2 Movement when gravity is eliminated
1 Trace of movement
0 No movement.
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Figs. 5A to C: Muscle testing in children can be done by observation of activities and functional assessment. Three examples are as follows. (A) Wheel barrowing will help in identifying upper limb proximal power of muscles like triceps latissimus dorsi and neck extensors; (B) Bridging: Tests gluteus maximus, calf muscles, hamstrings and toe flexors; and (C) Heel gait for ankle dorsiflexors.
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REFLEXES
In practice, there are four groups of reflexes:
(1) Superficial reflexes; (2) Deep tendon (DT) reflexes; (3) Neonatal reflexes, and (4) Primitive (pathological) reflexes.
 
Remember
  • The seated position is best for obtaining DT reflexes
  • Abdominal reflexes, cremasteric, and Babinski's reflex are better done in the supine position
  • Feel the tendon for localization or tenderness before tapping
  • Relax the joint before tapping
  • Swing your hammerhead to tap, not just touch the tendon
  • The response to tapping may be jerking of the limb, twitching of corresponding muscles, or feeling the tendon twitch under your finger
  • Listen for the sound of the taps, because true absence of reflexes has a dull sound
  • Always check reflexes simultaneously between two sides to establish symmetry
  • Hyper- or hyporeflexia is clinically significant when either are associated with other neurological signs, if they are asymmetric
  • Bilateral absence of brachioradialis or ankle jerks in an asymptomatic patient often has no clinical significance
  • Unilateral absence of ankle jerks is consistent with an S1 root lesion
  • Do “reinforcement” before concluding that the reflex is absent
  • Asymmetry of reflexes is significant when it is reproducible
  • Reflex segmental level can be remembered by remembering the reflex man (Fig. 6).
 
Superficial Reflexes
Superficial reflexes are elicited by stroking the skin (abdominals, cremasteric, plantar) or mucous membranes (gag, corneal). The most important reflex in this group that has to be checked in all patients with a neurological presentation is the plantar response. The majority of superficial reflexes are polysynaptic and affected by a segmental (root, nerve) lesion, as well as suprasegmental (cortical, brainstem) lesions.
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Fig. 6: Reflex man.
 
Plantar Reflexes
The plantar reflex is best obtained when the patient is supine. Hold the ankle with the other hand. Explain to the patient what you are going to do. Stroke the lateral border of the foot across to the pad of the foot with a blunt object such as thumbnail and observe the following responses:
  • All toes flex = flexor plantar response. This is normal
  • Extension of the big toe and spreading of the other toes = extensor plantar response or Babinski's sign. This is abnormal
  • Extension of the big toe only = “toe sign,” in other words, upgoing toe. This is abnormal
  • Dorsiflexion of the foot, flexion at the knee, and flexion at the hip = triple flexor or response. This is abnormal
  • Nonstereotype flexion of the foot and knee = withdrawal response. This is normal
  • No movement = no response
  • Babinski's sign, extensor toe sign, and stereotyped tripleflex or response are abnormal, and indicative of a UMN lesion involving the pyramidal tract from the L2 level of the spinal cord to the cerebral cortex.
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Babies of up to 2 years of age may have upgoing plantar; so interpret the response with relation to other definite UMN signs.
 
Alternate Methods for Extensor Plantar
If you do not succeed, use an alternate stimuli, such as stroking the side of foot
  • Chaddock's (side of foot = Shaddock)
  • Over the shin = Oppenheim's sign and
  • Squeezing gastrocnemius = Gordon reflex.
 
Release Reflexes
Snout, rooting, sucking, etc. are clinically significant when they are hyperactive. In young children do a detailed neonatal reflex examination which includes:
  • Moro reflex
  • Tonic neck response
  • Righting reflex
  • Palmar and plantar grasp reflexes
  • Landau reflex
  • Parachute response
  • Placing and stepping responses.
Identifying late disappearance of neonatal reflex, [e.g. asymmetrical tonic neck reflex (ATNR)] and late appearance of infantile reflex (e.g. Landau reflex) are helpful in early detection of cerebral palsy. In older children indicative of bifrontal lesions are the following. Grasp glabellar, palmomental, finger flexion, and Hoffman's reflex. Hoffman's reflex is elicited by holding the middle phalanx of middle finger and then terminal phalanx is flexed and then flicked into extension. Positive response is sudden flexion and adduction of other fingers and thumb.
 
Gait
Gait is a function of the motor (pyramidal and extrapyramidal), sensory, vestibular, visual, and cerebellar systems.
Observe as the child walks and runs while playing. Formal testing also includes walking on toes, heels, and tandem walking with rotation around a chair. Making the child climb up and down will also yield lot of information. When possible, do Romberg's test also.
  • Circumduction gait: In children who are hemiplegic, gait is one leg swing out and then adduction
  • Lurching: Cerebellar ataxia
  • Scissoring: Cerebral palsy; bilateral UMN lesions.
  • Waddling: Muscular dystrophy, proximal weakness
  • Astasia-abasia: Inconsistent, worse when watched—psychogenic
  • Antalgic gait—pain and limping—arthritis
  • Steppage: Foot drop, peripheral neuropathy, and L5 root lesion.
 
COORDINATION AND ABNORMAL MOVEMENT
  • Child's manipulation of toys and reaching and releasing objects on request will help us in assessing coordination.
  • Specific tests: Finger-to-nose and heel-to-shin testing can be done in appropriate situation.
  • Keeping the arm fully outstretched is very important in finger-to-nose test (Figs. 7A and B).
  • Abnormal movements are also observed and defined.
  • Physiological in coordination and abnormal movements must be distinguished. Small, choreiform movements are common in the healthy infant maximally between 9 weeks and 12 weeks of age.
 
Sensory Examination
Even though sensory examination is difficult at any age, significant sensory deficits can be determined by an examiner who uses ingenuity. Deficits can be detected by moving the hand slowly up the child's body.9
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Figs. 7A and B: For testing coordination arm must be fully outstretched and then brought to the nose tip with a wide arc thus testing all the cerebellar functions like dyssynergia, dysmetria, and intention tremor.
Cortical sensory signs like object discrimination can be determined in the older one by the use of coin, or small, and familiar items, such as paper clips or mobile phone.
While doing the final assessment, do a peripheral nerve palpation and look for any signs of meningeal irritation. Description of the skull and spine concludes the neurological examination. Examination of other relevant system will definitely help in arriving at an etiological diagnosis.
 
How to Formulate the Diagnosis
The neurologic examination findings of the infant cannot be summarized as normal or abnormal by single sitting. Multiple examinations may also be required.
 
Steps in Diagnosis
  • Functional diagnosis: What are the disabilities and which are the parts of nervous system involved?
  • Anatomical diagnosis: Where is the lesion? UMN/LMN; cortical, subcortical, etc.
  • Etiological diagnosis: Considering the onset, evolution, and associations.
Thus list the differential diagnosis:
  • Plan the diagnostic tests in order to support the diagnosis
  • Make available all the therapeutic measures possible and seek help of colleagues and paramedics.
  • It is the child that matters.
SUGGESTED READING
  1. Aminoff MJ, Simon RR, Greenberg D. Clinical neurology. Europe: McGraw-Hill Education;  2015.
  1. Brazis PW, Masdeu JC, Biller J. Localization in Clinical Neurology 7th edition. Lippincott Williams & Wilkins (LWW);  2016.
  1. Duderstad KG, Neurological system (Chapter 19) in Pediatric Physical Examination—An illustrated handbok Elsevier;  2013. pp. 288–310.
  1. Dulac O, Lassonde M, Sarnat HB. Pediatric Neurology: Part I: Handbook of Clinical Neurology. US: Elsevier;  2013.
  1. Kotagal A, Nordli DR Jr, Armsby C. Detailed neurologic assessment of infants and children. UptoDate Review article.
  1. Swaiman KF, Ashwal S, Ferriero DM. Swaiman's pediatric neurology: principles and practice, 6th edition. Philadelphia: Elsevier;  2018.