International Neurology (eBook)

Robert P. Lisak, Daniel D. Truong, William M. Carroll, Roongroj Bhidayasiri (Herausgeber)

eBook Download: PDF

2016 | 2. Auflage
John Wiley & Sons (Verlag)
978-1-118-77735-0 (ISBN)

Infos

This unique textbook deals with the variations in the causes, presentations and treatment of neurological disease throughout human populations. International Neurology is an indispensable guide to the full range of neurological conditions you will see in your ever-changing patient population.

• Comprehensive coverage of neurological diseases and disorders with a clinical approach to diagnosis, treatment and management
• Truly international authorship distils expert knowledge from around the world
• Succinct, bite-sized, templated chapters allow for rapid clinical referral
• Further reading recommendations for each chapter guide readers requiring more depth of information
• Endorsed by the World Federation of Neurology

ROBERT P. LISAK, FAAN, FRCP (E), FANA
Parker Webber Chair in Neurology, Professor of Neurology, Professor of Immunology and Microbiology, Wayne State University School of Medicine; Attending Neurologist, Detroit Medical Center, Detroit, MI, USA

DANIEL D. TRUONG MD FAAN
The Truong Neuroscience Institute, Orange Coast Memorial Medical Center, Fountain Valley, CA, USA; Professor, University California Riverside, Riverside, CA, USA

WILLIAM M. CARROLL MBBS MD FRACP FRCP(E)
Clinical Professor of Neurology, Western Australian Research Institute, University of Western Australia, Australia

ROONGROJ BHIDAYASIRI MD FRCP
Director, Chulalongkorn Comprehensive Movement Disorders Center
Chulalongkorn University Hospital, Bangkok, Thailand;
University of California at Los Angeles, Los Angeles, CA, USA

Inhalt

International Neurology 3
Dedication 4
Contents 7
Editors 12
List of contributors 13
Foreword 23
Endorsement from the World Federation of Neurology 24
Preface 25
Part 1 Vascular Disorders 27
1 Overview of stroke 27
2 Ischemic stroke and transient events, TIA 29
History 29
Epidemiology 29
Pathophysiology 30
Atherosclerosis of great vessels 30
Occlusion of small vessels 30
Cardioembolism 30
Clinical features 30
Investigations 30
Treatment 31
Conclusions 32
Further reading 32
3 Atherothrombotic disease 33
Site of atheroslerosis 33
Atherosclerosis of extracranial arteries in the neck 33
Atherosclerosis of the intracranial arteries 33
Ascending aorta 33
Mechanisms of stroke and clinical manifestations 33
Arterial to arterial embolism 33
Low?flow state secondary to severe arterial stenosisor occlusion 33
Occlusion of the perforating arteries due to atherosclerotic plaque 34
Investigations 34
Management of large vessel atherosclerosis 34
Further reading 34
4 Occlusive disease of small penetrating arteries 36
Epidemiology 36
Pathophysiology 36
Clinical features 37
Investigations 37
Treatment 37
Acute stroke therapy 37
Secondary prevention 37
Further reading 38
5 Ischemic white matter disease (Binswanger’s disease) 39
Epidemiology 39
Pathophysiology 39
Clinical features 39
Investigations 39
Treatment 40
Further reading 40
6 Brain embolism 41
Epidemiology 41
Pathophysiology 41
Clinical features 41
Investigations 42
Treatment 42
Thrombolysis and mechanical clot retrieval 42
Prevention of recurrent cardiac embolism 43
Infective endocarditis 43
Atrial fibrillation 43
Valvular diseases 44
Patent foramen ovale 44
Further reading 44
7 Systemic hypotensive injury (border?zone infarction) 45
Epidemiology 45
Pathophysiology 45
Clinical features 45
Investigation 45
Treatment 46
Further reading 47
8 Dissection of the cervicocerebral arteries 48
Introduction and epidemiology 48
Etiology and pathophysiology 48
Clinical features 48
Dissection of the extracranial internal carotid artery 49
Dissection of the vertebral artery 49
Investigations 49
Treatment 49
Further reading 50
9 Coagulation and Hematological Disorders in Stroke 51
Hyperviscosity disorders 51
Sickle cell anemia 51
Polycythemia rubra vera and thrombocytosis 51
Prothrombotic disorders 51
AT III deficiency 52
Protein C and protein S deficiencies 52
Homocysteinemia 52
Antiphospholipid syndrome 52
Platelet dysfunction disorders 52
Other coagulopathies 52
Investigations 52
Treatment 53
Further reading 53
10 Hemorrhagic strokes 54
Primary intracerebral hemorrhage 54
Epidemiology 54
Risk factors 54
Pathophysiology 54
Clinical features 55
Investigations 55
Medical management 55
Management of increased intracranial pressure 56
Acute hemostatic therapy 56
Management of acute hypertensive response 56
Intracerebral hemorrhage related to use of oral anticoagulants 56
Treatment of ICH related to fibrinolysis 57
Surgical treatment 57
Subarachnoid hemorrhage 57
Epidemiology 57
Pathophysiology 57
Clinical features 58
Complications of SAH 58
Diagnostic investigations 59
Medical treatment 59
Surgical and endovascular management 60
Cerebral arteriovenous malformation 60
Epidemiology 60
Pathophysiology 60
Clinical features 61
Diagnostic studies 61
Treatment 61
Cerebral amyloid angiopathy 61
Epidemiology 61
Pathophysiology 61
Clinical features 62
Diagnostic studies 62
Management 62
Further reading 62
11 Strokes in children and young adults 63
Etiology and epidemiology 63
Clinical features 64
Investigations 64
Treatment 64
Further reading 65
12 Other cerebrovascular syndromes 66
Stroke and migraine 66
Stroke and substance abuse 66
Cardiac 66
Extracranial large arteries 66
Intracranial vessels 67
Other 67
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) and related conditions 67
Hypertensive encephalopathy posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstrictive syndrome (RCVS) 67
Periprocedural, postoperative, and in?hospital stroke 67
Rare stroke syndromes 68
Conclusion 68
Further reading 68
13 Cerebral venous disease 69
Epidemiology 69
Pathophysiology 69
Clinical features 70
Investigations 70
Treatment 70
Further reading 71
14 Spinal cord strokes 72
Spinal cord vascular supply 72
Spinal cord ischemia and infarction 72
Causes of spinal cord ischemia 73
Disease of the aorta 73
Embolism 73
Dissections 74
Infections and inflammation 74
Drug abuse 74
Global ischemia 74
Iatrogenic 74
Venous infarction 74
Spinal vascular malformations 74
Dural malformations 74
Arteriovenous malformations 74
Investigations and management 74
Spinal cord ischemia 74
Spinal hemorrhages 75
Further reading 75
Part 2 Disorders of Cerebrocascular Autonomic Control 76
15 Reversible cerebral vasoconstriction syndrome (RCVS) 76
Epidemiology 76
Pathophysiology 76
Clinical features 77
Investigations 77
Treatment 78
Conclusion 78
Further reading 78
16 Posterior reversible encephalopathy syndrome (PRES) 79
Epidemiology 79
Pathophysiology 79
Etiology 79
Clinical features 80
Investigations 80
Treatment 80
Prognosis 80
Conclusion 80
Further reading 80
Part 3 The Vasculitides 81
17 Extracranial and intracranial granulomatous arteritis (giant cell arteritis) 81
Epidemiology 81
Pathogenesis 81
Cellular immunity (vascular lesion) 81
Humoral immunity 81
Clinical manifestations 81
Systemic manifestations 81
Neuro?ophthalmic manifestations 82
Neurological manifestations 82
Large vessel arteritis 82
Polymyalgia rheumatica 82
Systemic inflammatory syndrome with arteritis 82
Diagnostic approaches to GCA 82
Laboratory findings 82
Diagnosis 82
Imaging 82
Treatment 83
Prognosis 83
Further reading 83
18 Intracranial granulomatous arteritis (primary angiitis of the CNS)/idiopathic CNS vasculitis 84
Introduction and nomenclature 84
Clinical course and diagnosis 84
Treatment 84
Future developments 85
Further Reading 85
19 Takayasu’s arteritis 86
Epidemiology 86
Pathophysiology 86
Pathology 86
Pathogenesis 86
Clinical features 86
Investigations 88
Treatment 88
Further reading 88
20 Polyarteritis nodosa, eosinophilic granulomatosis with polyangiitis, and overlap vasculitis syndrome 89
Polyarteritis nodosa (PAN) 89
Epidemiology 89
Pathophysiology 89
Clinical features 90
Central nervous system involvement 90
Peripheral nervous system involvement 90
Investigations 90
Treatment 90
Eosinophilic granulomatosis with polyangiitis (EGPA) 90
Epidemiology 91
Pathophysiology 91
Clinical features 91
Neurological involvement 91
Treatment 91
Overlap vasculitis 91
Further reading 92
21 Granulomatosis with polyangiitis (GPA) 93
Epidemiology 93
Clinical features 93
Investigations 93
Treatment 93
Further reading 93
22 Cerebrovascular disease associated withantiphospholipid antibodies 94
Epidemiology 94
Pathophysiology 94
Clinical features 95
Investigations 95
Treatment 95
Further reading 96
23 Thromboangiitis obliterans (Buerger’s disease) 97
Epidemiology 97
Pathophysiology 97
Clinical features 97
Investigations 97
Treatment 98
Further reading 98
24 Susac syndrome 99
Epidemiology 99
Pathophysiology 99
Clinical features 99
Investigations 99
Treatment 100
Further reading 100
Part 4 Inflammatory Vasculopathies 101
25 SLE, rheumatoid arthritis, and Sjögren’s syndrome 101
Systemic lupus erythematosus (SLE) 101
Epidemiololgy 101
Pathophysiology 101
Clinical features 102
Rheumatoid arthritis 107
Epidemiology 107
Clinical features 107
Sjögren’s syndrome 107
Treatment of SLE, RA, and SS 107
Further reading 107
26 Systemic sclerosis 109
Epidemiology 109
Pathophysiology 109
Clinical features 109
Investigations 110
Treatment 110
Further reading 110
27 Mixed connective tissue disease 112
Epidemiology 112
Pathophysiology 112
Clinical features 112
Investigations 113
Treatment 113
Further reading 113
28 Behcet’s syndrome and nervous system involvement 114
Epidemiology 114
Pathophysiology 114
Clinical features 114
Oral ulcers and other mucocutaneous symptoms 114
Ocular involvement 114
The pathergy phenomenon 114
Primary neurological involvement (neurological involvement directly related to BS) 115
Secondary neurological involvement (neurological involvement indirectly related to BS) 116
Investigations for NBS 116
Neuroimaging 116
Cerebrospinal fluid 117
Differential diagnosis 117
Intra?axial NBS 117
Treatment of NBS 117
Intra?axial (parenchymal) NBS 117
Extra?axial (CVST) NBS 119
Prognosis 119
Further reading 119
29 Sarcoidosis 120
Epidemiology 120
Pathophysiology 120
Clinical features 120
Investigations 121
Treatment 122
Further reading 123
30 Autoimmune encephalitis with neuronal cell surface antibodies 124
Antibodies and ancillary tests 124
Clinical spectrum 125
Differential diagnoses 125
Treatment 126
Further reading 126
Part 5 Paroxysmal Disorders 127
31 Epilepsy overview 127
Classification 127
Epidemiology 128
Age and seizure types 128
Mortality 128
Pathophysiology 128
Risks and etiology 129
Investigations 129
Differential diagnosis 129
Convulsive syncope 129
Sleep disorders 129
Movement disorders 129
Treatment/management 129
Special populations 130
Epilepsy in the elderly 130
Epilepsy in women 130
Epilepsy in individuals with psychiatric comorbidities 130
Standardizing care for persons with epilepsy 131
Conclusions 131
Further reading 131
32 Structural, genetic and unknown generalized epilepsies and syndromes 132
Ohtahara syndrome (EIEE) 132
Pathophysiology 132
Clinical features and investigations 132
Treatment 132
Prognosis 132
Severe myoclonic epilepsy in infancy (SMEI) or Dravet syndrome 132
Pathophysiology 132
Clinical features 133
Investigations 133
Treatment 133
Prognosis 133
West syndrome (WS) 133
Epidemiology 133
Pathophysiology 133
Clinical features 134
Investigations 134
Treatment 134
Prognosis 134
Lennox–Gastaut syndrome (LGS) 134
Epidemiology 134
Pathophysiology 134
Clinical features 134
Investigations 135
Treatment 135
Prognosis 135
Epilepsy with myoclonic absences (EMA) 135
Epidemiology 135
Etiology 135
Clinical features 135
Investigations 135
Treatment 135
Prognosis 135
Inflammatory epilepsies 135
Further reading 136
33 Genetic (primary) idiopathic generalized epilepsies 137
Advances in the classification of epilepsy 137
Advances in molecular genetics 137
Febrile seizure (FS) 137
Clinical 137
Genetic 137
Febrile seizures plus (FS+) 138
Severe myoclonic epilepsy of infancy(SMEI) 138
Clinical 138
Genetic 138
Childhood absence epilepsy (CAE) 138
Clinical 138
Genetic 138
Juvenile absence epilepsy (JAE) 138
Clinical 138
Genetic 138
Juvenile myoclonic epilepsy (JME) 138
Clinical 138
Genetic 139
Novel progressive myoclonic epilepsy(PME) 139
Clinical 139
Genetic 139
De novo mutations 139
Advances in technologies 139
Targeting sequencing and epilepsy gene panel 139
Massively parallel sequencing or next secondgenerationtechnology 139
Copy number variation (CNV) 139
Whole?exome sequencing (WES) 140
Further reading 140
34 Localization?related epilepsies 141
Idiopathic LRE 141
Panayiotopoulos syndrome 141
Rolandic epilepsy 141
Idiopathic childhood occipital epilepsy of Gastaut 142
Monogenic focal epilepsies 143
Benign familial neonatal seizures 143
Benign familial infantile seizures 143
Autosomal dominant nocturnal frontal lobe epilepsy 143
Familial mesial temporal lobe epilepsy 143
Familial lateral temporal lobe epilepsy 143
Familial partial epilepsy with variable foci 144
Temporal lobe epilepsy (TLE) 144
Frontal lobe epilepsy 145
Parietal lobe epilepsy 145
Occipital lobe epilepsy (OLE) 145
Further reading 145
35 Neurodiagnostic tools for the paroxysmal disorders 146
Electrophysiology 146
Electroencephalography 146
Video EEG 146
Intracranial recordings 147
Magnetoencephalography 147
Neuroimaging 147
Computerized axial tomography and magnetic resonance imaging 147
Functional imaging 147
Functional MRI 147
MRS 147
Other techniques 147
Conclusion 147
Further reading 147
36 Neuropharmacology of antiepileptic drugs 148
History of antiepileptic drugs 148
Mechanisms of action for antiepileptic drugs 148
Rationale for antiepileptic drug selection: Choice of initial drug 149
Spectrum of action 149
Pharmacokinetics and compliance 149
Route of administration 149
Associated medical illness 150
Other conditions treated with antiepileptic drugs 150
Essential tremor 150
Migraine 150
Neuropathic pain 150
Psychiatric disorders 150
Failure of initial treatment: Rationale for replacement of initial drug or combination AED therapy 151
AEDs in the treatment of status epilepticus 151
Convulsive status epilepticus 151
Refractory status epilepticus 151
Super?refractory status epilepticus 151
Non?convulsive status epilepticus 151
Epilepsia partialis continua 151
Complications of antiepileptic drug therapy 152
Allergic reactions 152
Ophthalmic reactions 152
Hepatic reactions 152
Psychiatric reactions 152
Elective discontinuation of antiepileptic drugs 152
Herbal treatments as AEDs 153
Further reading 153
37 Surgical treatment of epilepsy 154
Investigations and indications for epilepsy surgery 154
Semi?invasive and invasive video EEG 154
Operations 155
Curative procedures 155
Palliative procedures 156
Acknowledgments 157
Further reading 157
Part 6 Dementia 158
38 Dementia overview 158
Acknowledgment 158
39 Mild cognitive impairment and Alzheimer’s disease 159
Mild cognitive impairment 159
Epidemiology 159
Pathophysiology 159
Clinical features 159
Investigations 160
Alzheimer’s disease 160
Epidemiology 160
Pathophysiology 160
Clinical features 161
Investigations 161
Use of biomarkers for MCI and AD 162
Structural MRI 163
Functional imaging 163
Amyloid imaging 163
CSF biomarkers 163
Treatment 163
MCI 163
Alzheimer’s disease 164
Further reading 164
40 Parkinson’s Disease Dementia, Dementia with Lewy Bodies, and Other Synucleinopathies 166
Parkinson’s disease dementia 166
Epidemiology 166
Neuropathology of cognitive impairment and dementia in PD 166
Clinical features 167
Investigations 168
Treatment of PDD 168
Dementia with Lewy bodies 168
Epidemiology 168
Neuropathology 168
Clinical features 168
Investigations 169
Treatment 169
Multiple system atrophy 169
Epidemiology 169
Neuropathology 169
Clinical features 170
Investigations 170
Treatment 171
Further reading 171
41 Specific vascular syndromes 172
Cerebral amyloid angiopathy 172
Hereditary small vessel syndromes 172
Poststroke dementia 172
Further reading 173
42 Vascular cognitive impairment 174
Epidemiology 174
Risk factors 174
Neuropathology and pathophysiology of VCI 174
Large vessel disease 174
Small vessel disease 174
Subcortical ischemic vascular disease 175
Non?infarct ischemic changes and atrophy 175
Neuropathology 175
Clinical features 176
Clinical evaluation 177
Cognitive assessment 177
Biomarkers 177
Treatment 178
Conclusions 178
Further reading 178
43 Frontotemporal dementia 179
Epidemiology 179
Pathophysiology 179
Genetics 179
Behavioral variant frontotemporal dementia 179
Possible bvFTD 179
Probable bvFTD 180
BvFTD with definite FTLD pathology 180
Primary progressive aphasia 180
Non?fluent/agrammatic variant of PPA 181
Semantic variant of PPA 181
Lopogenic variant of PPA 181
Cross?cultural issues 182
Treatment 182
Acknowledgment 182
Further reading 182
44 Progressive supranuclear palsy, corticobasal syndrome, and other tauopathies 183
Pathophysiology 183
FTLD?tau with MAPT mutation 183
Animal models of tauopathies and transmission 183
Progressive supranuclear palsy syndrome 184
Clinical features 184
Epidemiology 184
Treatment 184
Clinicopathological correlations 184
Corticobasal syndrome 185
Clinical features 185
Epidemiology 185
Treatment 185
Clinicopathological correlations 185
Other tauopathies (FTLD?tau) 185
Pick’s disease and FTD clinical syndromes 185
Geographically isolated tauopathies with parkinsonism 186
Other tauopathies 186
Future directions 186
Further reading 186
45 Amyotrophic lateral sclerosis and other TDP?43 proteinopathies 187
Amyotrophic lateral sclerosis 187
Epidemiology 187
Clinical features 187
Treatment 187
Neuropathology 187
Frontotemporal dementias 188
Epidemiology 188
Clinical features 188
Treatment 189
Neuropathology 189
Conclusion 189
Further reading 189
46 Metabolic, toxic, infectious, inflammatory, and other dementias 190
Normal pressure hydrocephalus 190
Toxic and metabolic disorders 191
Alcohol 191
Wernicke–Korsakoff syndrome 191
Vitamin B12 deficiency 191
Hypo? and hyperthyroidism 192
Infectious causes 192
Dementia associated with human immunodeficiency virus infection 192
Neurosyphilis (syphilitic dementia) 192
Progressive multifocal leukoencephalopathy 193
Whipple’s disease 193
Viral encephalitides 193
Other infectious agents 193
Genetic causes 193
Huntington’s disease 193
Dementia associated with ataxia 194
Dementia associated with other movement disorders 194
Genetically determined storage disorders 194
Autoimmune disorders 195
Systemic autoimmune disorders 195
Autoimmune encephalitides 195
Anti?NMDAR encephalitis 195
Limbic encephalitis 195
Morvan’s syndrome 195
Other steroid?responsive autoimmune encephalopathies 195
Conclusion 196
Further reading 196
Part 7 Movement Disorders 197
47 Movement disorders: An overview 197
Diagnostic recommendations 197
Further reading 198
48 Tremor 199
Clinical features 199
Clinical approach 199
Types of pathological tremors 200
Epidemiology 201
Pathophysiology 202
Investigations 202
Treatment 202
Further reading 204
49 Parkinsonism 205
Progressive supranuclear palsy 205
Epidemiology 205
Pathophysiology 205
Clinical features 205
Management 206
Corticobasal syndrome 206
Epidemiology 207
Pathophysiology 207
Clinical features 207
Investigations 207
Management 208
Multiple system atrophy 208
Epidemiology 208
Clinical features 208
Investigations 209
Management 209
Dementia with Lewy bodies 210
Epidemiology 210
Pathophysiology 210
Clinical features 210
Investigations 210
Management 210
Frontotemporal dementia 210
Epidemiology 210
Pathophysiology 211
Clinical features 211
Investigations 211
Management 211
Vascular parkinsonism 211
Normal pressure hydrocephalus 211
Parkinsonism dementia complex of Guam 211
Drug?induced parkinsonism 211
Toxin?induced parkinsonism 212
Infectious and postinfectious parkinsonism 212
Further reading 212
50 Parkinson’s disease 214
Neuropathology 214
Clinical presentation 215
Imaging 216
Differential diagnosis 216
Treatment 218
Management of early Parkinson’s disease 218
Management of advanced (complicated) Parkinson’s disease 219
Treatment of dyskinesia 220
Treatment of “off” dystonia 220
Treatment of dose failures or no “on” response and delayed “on” 220
Treatment of Parkinson’s disease psychosis 221
Conclusion 222
Further reading 222
51 Dystonia 223
Epidemiology 223
Pathobiology 223
Pathology 223
Etiology 223
Genetics 223
Pathophysiology 224
Classification 224
Clinical features 226
Adult-onset focal dystonias 226
Oppenheim dystonia 227
Dopa-responsive dystonia (DRD) 227
Acquired and heredodegenerative dystonias 228
Treatment 228
Further reading 228
52 Chorea and related disorders 229
Classification 229
Hereditary causes 229
Huntington’s disease 229
Epidemiology 229
Genetics 230
Clinical features 230
Differential diagnosis 230
Neuropathology 230
Treatment 230
Neuroacanthocytosis 232
Senile chorea 233
Others 233
Parainfectious and autoimmune causes 233
Sydenham’s chorea 233
Chorea gravidarum 233
Systemic lupus erythematosus and antiphospholipid syndrome 233
Paraneoplastic syndrome 233
Drug-related causes 233
Vascular causes 233
Metabolic causes 233
Infectious causes 234
Diagnosis 234
Treatment 234
Conclusion 234
Further reading 235
53 Myoclonus 236
Classification 236
Exam findings 236
Clinical neurophysiology 236
Clinical etiology classification 236
Features of major myoclonic disorders 237
Posthypoxic myoclonus 237
Progressive myoclonus epilepsy 237
Essential myoclonus 237
Neurodegenerative disorders 237
Metabolic disorders 237
Drugs 237
Autoimmune disease 237
Psychogenic jerks 238
Evaluation 238
Treatment 238
Levetiracetam 238
Valproic acid 238
Clonazepam 239
Other drugs 239
Botulinum toxin 239
Deep brain stimulation 239
Further reading 239
54 Tics and Tourette syndrome 240
Pathophysiology 241
Clinical features 241
Investigations 241
Treatment 241
Further reading 242
55 Ataxia 243
Clinical features 243
Cerebellar ataxia 243
Sensory ataxia 243
Vestibular ataxia 243
Frontal ataxia 244
Pathophysiology 244
Cerebellar ataxic disorders 244
Acute ataxia 244
Chronic ataxia 244
Hereditary ataxias 245
Sporadic (idiopathic) degenerative ataxia 245
Diagnosis 245
Management 245
Further reading 246
56 Drug?induced movement disorders 247
Acute syndromes 247
Acute dystonic reactions 247
Acute akathisia 247
Acute choreoathetosis 248
Acute tics 248
Subacute syndromes 248
Drug?induced parkinsonism (DIP) 248
Chronic or tardive syndromes 248
Further reading 249
57 Paroxysmal movement disorders 250
Historical aspects 250
Epidemiology 250
Pathophysiology 250
Clinical features 251
Paroxysmal kinesigenic dyskinesia 251
Paroxysmal non?kinesigenic dyskinesia 251
Paroxysmal exercise?induced dyskinesia 251
Investigations/diagnosis 252
Treatment 252
Conclusion 252
Further reading 252
Part 8 Infectious diseases 253
58 Acute bacterial meningitis 253
Epidemiology 253
Etiology 253
Pathophysiology 254
Pathology 254
Clinical features 254
Investigations 255
Imaging 255
Cerebrospinal fluid 255
Differential diagnosis 255
Treatment 255
Empirical antibiotic treatment 255
Specific treatment 255
Role of steroids and glycerol 256
Prevention 256
Prognosis 256
Complications 256
Further reading 256
59 Brain abscess 257
Pathophysiology 257
Clinical features 258
Investigations 258
Treatment 258
Further reading 259
60 Subdural empyema 260
Epidemiology 260
Pathophysiology 260
Clinical features 260
Investigations 260
Imaging studies 260
Laboratory studies 261
Treatment 262
Further reading 262
61 Epidural abscess 263
Epidemiology 263
Pathophysiology 263
Clinical features 263
Investigations 263
Treatment 264
Further reading 264
62 Septic cerebral venous sinus thrombosis 265
Infection?related/septic cerebral venous sinus thrombosis 265
Anatomy and pathophysiology 265
Microbial etiology 265
Thrombophilia and septic CVT 266
Clinical presentation 266
Differential diagnosis 266
Radiological findings 266
Other investigations 266
Management 266
Outcome and prognosis 267
Further reading 268
63 Encephalitis due to bacterial infections 269
Mycoplasma pneumoniae 269
Listeria monocytogenes 269
Whipple’s disease 270
Cat?scratch disease 270
Brucellosis 271
Legionnaires’ disease 271
Recommendations 271
Further reading 271
64 Mycobacterium tuberculosis and avium 272
Epidemiology 272
Pathophysiology 272
Tuberculosis meningitis 272
Tuberculomas 273
Clinical features 273
Tuberculous meningitis 273
Tuberculomas 273
Investigations 273
Tuberculous meningitis 273
Tuberculomas, abscesses, and other complications 273
Treatment 273
Medication 273
Neurosurgery 274
Mycobacterium avium 274
Further reading 274
65 Leprosy 275
Bacteriology 275
Epidemiology 275
Pathogenesis 275
Immunity 275
Clinical features 276
Leprosy reactions 277
Diagnosis 277
Other investigations 278
Differential diagnosis 278
Antileprotic treatment 278
Treatment of leprosy reactions 278
Further reading 278
66 Neurosyphilis 279
Epidemiology 279
Pathophysiology 279
Clinical features 279
Neuropsychiatric 279
Stroke 280
Encephalopathy with seizures 280
Spinal cord stroke 280
Meningitis 280
Tabes dorsalis 280
HIV and neurosyphilis 280
Ocular syphilis 280
Investigations 280
Treatment 280
Treatment of HIV?negative patients with neurosyphilis 280
Treatment of HIV?positive patients with neurosyphilis 280
Follow?up 281
Further reading 281
67 Lyme disease 282
Epidemiology 282
Demographics 282
Tick vector 282
Organism 282
Clinical expression 282
Target organs 282
Disease stages 283
Extraneural manifestations 283
Neurological Lyme disease 283
Diagnosis 283
Treatment 284
Prevention 284
Tick co?pathogens 284
Pathogenesis 284
Pathology 284
Pathophysiology 284
Chronic Lyme disease/post?treatment Lyme disease syndrome 285
Further reading 285
68 Introduction to protozoans of thecentral nervous system 286
69 Amoebic disease of the central nervous system 287
Epidemiology 287
Pathophysiology 287
Clinical features 288
Investigations 288
Treatment 288
Further reading 289
70 Toxoplasmosis of the central nervous system 290
Epidemiology 290
Pathophysiology 290
Clinical features 290
Primary toxoplasmosis 290
Congenital toxoplasmosis 291
Immunocompromised patients 291
Retinochoroiditis 291
Investigations 291
Treatment 291
Further reading 293
71 Cerebral malaria 294
Epidemiology 294
Pathology 294
Pathophysiology 294
Sequestration 294
Cytoadherence 294
Cytokines 294
Other factors 294
Pathogenesis of coma 294
Cerebral malaria caused by P. vivax 294
Clinical features 295
Post?malaria neurological syndromes(PMNS) 296
Poor prognostic factors 296
Diagnosis 296
Treatment 297
Resuscitation 297
Antimalarial treatment 297
Option 1: Artesunate 297
Option 2: Artemether 297
Option 3: Quinine 297
Supportive treatment 297
Further reading 298
72 Trypanosomiasis 299
American trypanosomiasis 299
Epidemiology 299
Clinical features 299
Pathophysiology of chronic Chagas’ disease 299
Investigations 299
Treatment 300
Human African trypanosomiasis 300
Epidemiology 300
Pathophysiology 300
Clinical features 301
Investigations 301
Treatment 301
Further reading 301
73 Cestodes 302
Neurocysticercosis 302
Epidemiology 303
Pathophysiology 303
Clinical features 303
Diagnosis 304
Treatment 304
Echinococcosis 305
Epidemiology 305
Pathophysiology 305
Clinical features 305
Diagnosis 305
Treatment 306
Further reading 306
74 Trematodes: Schistosomiasis 307
Epidemiology 307
Pathogenesis 307
Clinical features 307
Cerebral schistosomiasis 307
Spinal schistosomiasis 307
Investigations 307
Treatment 309
Further reading 309
75 Nematodes 310
Trichinosis 310
Epidemiology 310
Pathophysiology 310
Clinical features 311
Investigations 311
Treatment 311
Gnathostomiasis 311
Epidemiology 311
Pathophysiology 311
Clinical features 311
Investigations 311
Treatment 312
Angiostrongyliasis 312
Epidemiology 312
Pathophysiology 312
Clinical features 312
Investigations 312
Treatment 312
Further reading 312
76 Fungal infections of the centralnervous system 313
Candidiasis 313
Aspergillosis 313
Cryptococcosis 314
Coccidioidomycosis 314
Mucormycosis (zygomycosis) 315
Histoplasmosis 315
Blastomycosis 316
Fungal infections associated with contaminated steroid injections 316
Further reading 316
77 Rickettsial disease 317
Epidemiology 317
Pathophysiology 319
Clinical features 319
Spotted fever group rickettsioses 319
Typhus group rickettsioses 320
Investigations 321
Treatment 321
Further reading 321
Part 9 Prion Diseases and Neurovirology 322
78 Prion diseases 322
Epidemiology 322
Pathophysiology 322
Clinical features 323
Sporadic Creutzfeldt–Jakob disease 323
Genetic transmissible spongiform encephalopathies 323
Iatrogenic Creutzfeldt–Jakob disease 323
Kuru 323
Variant Creutzfeldt–Jakob disease 323
Investigations 323
Electrophysiological studies 323
Cerebrospinal fluid 324
Neuroimaging 324
Genetic analysis 324
Brain biopsy 324
Postmortem investigations 324
Treatment 324
Preventive measures 324
Further reading 325
79 Acute, recurrent and chronic viral meningitis 326
Epidemiology 326
Pathophysiology 327
Clinical features 327
Acute viral meningitis 327
Recurrent viral meningitis 328
Chronic viral meningitis 328
Differential diagnosis 328
Investigations 329
Treatment 329
Recovery and prognosis 330
Further reading 330
80 Acute viral encephalitis 331
Overview of acute encephalitis 331
Epidemiology 331
Pathophysiology 331
Clinical features 332
Investigations 332
Treatment 333
Specific viral encephalitides: Rabies 334
Epidemiology 334
Pathophysiology 334
Clinical features 334
Furious rabies 334
Dumb rabies 334
Investigations 335
Treatment 335
Japanese encephalitis 335
Epidemiology 335
Pathophysiology 335
Clinical features 335
Investigations 335
Treatment 336
West Nile encephalitis 336
Epidemiology 336
Pathophysiology 336
Clinical features 336
Investigations 336
Treatment 337
Tick?borne encephalitis 337
Epidemiology 337
Pathophysiology 337
Clinical features 337
Laboratory features 337
Management 337
St. Louis encephalitis 337
Epidemiology 337
Pathology 338
Clinical features 338
Investigation 338
Treatment 338
LaCrosse encephalitis 338
Epidemiology 338
Pathophysiology 338
Clinical features 338
Investigations 338
Treatment 338
Alphaviruses 338
Western equine virus 338
Venezuelan equine virus 339
Eastern equine virus 339
Investigations 339
Treatment 339
Other encephalitides 339
Dengue virus 339
Nipah virus 339
Enterovirus and EV71 340
Rift Valley encephalitis 340
Powassan and deer tick virus encephalitis 340
Australian encephalitis: Murray Valley encephalitis and Kunjin encephalitis 340
Hendra virus 340
Further reading 341
81 Neurological complications of human herpesvirus infections 342
Herpes simplex virus (HSV-1) encephalitis 342
Clinical features 342
Investigations 342
Treatment 343
HSV-2 infection 343
Clinical presentations 343
Encephalitis 343
Aseptic meningitis 343
Radiculopathy 344
Varicella zoster virus 344
Clinical presentations 344
Zoster 344
Investigations 346
Cytomegalovirus 347
Clinical presentations 347
Guillain–Barré syndrome 347
Polyradiculopathy 347
Ventriculoencephalitis 347
Encephalitis 347
Treatment 347
Epstein–Barr virus 347
Clinical features 347
Investigations 347
Human herpesvirus-6 (HHV-6) encephalitis 348
Clinical features 348
Investigations 348
Treatment 348
Acknowledgments 348
Further reading 348
82 Chronic viral diseases of the central nervous system 349
Epidemiology 349
Pathophysiology 349
Clinical features 349
Investigations 350
Serology and cerebrospinal fluid analysis 350
Magnetic resonance imaging 350
Electroencephalography 350
Prognosis and treatment 350
Other viruses that cause chronic progressive panencephalitis 350
Conclusion 351
Acknowledgments 351
Further reading 351
83 Progressive multifocal leukoencephalopathy 352
Epidemiology 352
PML and HIV/AIDS 352
PML in other populations 352
Geographic differences 353
Pathophysiology 353
Pathogenesis 353
Clinical features 353
PML–immune reconstitution inflammatory syndrome 354
Prognosis 354
Other forms of infection 354
Investigations 354
Imaging 354
Laboratory studies 354
Histopathology 354
Treatment 354
Prevention (risk?mitigation strategies) 354
Further reading 355
84 Biology of HIV and overview of AIDS 356
History and overview 356
Viral molecular epidemiology 356
Viral structure 356
Pathogenesis 357
Course of infection and clinical features 357
Acute HIV infection 358
Early HIV 358
Advanced HIV/AIDS 358
Neurological sequelae of HIV infection 358
Viral dynamics 358
Treatment and management 359
Conclusion 359
Further reading 359
85 HIV encephalitis and myelopathy 360
HIV?associated cognitive impairment 360
Terminology 360
Epidemiology 360
Pathophysiology 361
Clinical features 361
Investigations 361
Therapy 362
HIV?associated myelopathies 362
Epidemiology 362
Pathophysiology 363
Clinical features 363
Investigations 363
Treatment 363
Further reading 363
86 Neuromuscular complications of HIV 365
Epidemiology 365
Pathophysiology 365
Clinical features 365
Myopathies 365
Neuropathies 366
HIV?DSP 366
Inflammatory demyelinating polyneuropathies (IDP) 367
Polyradiculopathy 367
Mononeuropathy multiplex 367
Conclusion 367
Further reading 367
87 Opportunistic infections in HIV-positive subjects and AIDS patients 368
Opportunistic infections: Diffuse complications 368
Cryptococcal meningitis 368
Epidemiology 368
Pathophysiology 368
Clinical features 368
Investigations 368
Treatment 368
Tuberculous meningitis 369
Epidemiology 369
Pathophysiology 369
Clinical features 369
Investigations 369
Treatment 369
Cytomegalovirus encephalitis 369
Epidemiology 369
Pathophysiology 369
Clinical features 369
Investigations 370
Treatment 370
Opportunistic infections: Focal complications 370
Toxoplasmosis 370
Epidemiology 370
Pathophysiology 370
Clinical features 370
Investigations 370
Treatment 370
Tuberculosis 370
Clinical features 370
Pathophysiology 370
Investigations 370
Treatment 371
Progressive multifocal leukoencephalopathy 371
Epidemiology 371
Pathophysiology 371
Clinical features 371
Investigations 371
Treatment 371
HIV?associated lymphoma 371
Primary central nervous system lymphoma 371
Treatment 372
Further reading 372
88 Neurological complications of human T?cell lymphotropic virus type?1 infection 374
Epidemiology 374
Pathophysiology 374
Clinical features 375
HTLV?1?associated neurological complex 375
HTLV?1?associated polymyositis (HAPm) 375
HTLV?1?associated polyneuropathy (HAPn) 375
HTLV?1?associated dysautonomia (HAD) 375
Amyotrophic lateral sclerosis?like syndrome associated with HTLV?1 (ALS?HTLV) 375
Chronic diffuse encephalomyelopathy 375
Investigations 376
Differential diagnosis 376
Treatment 376
Further reading 376
Part 10 Demyelinating Disorders 377
89 Multiple sclerosis 377
Epidemiology 377
Pathophysiology 378
Pathology 378
Pathogenesis and etiology 379
Clinical presentation 380
Symptoms, signs, and course 380
Investigations 381
Treatment 383
Maintenance of general health and prevention or lowering the risk of MS 383
Treatment of symptoms 383
Treatment of relapses 384
Disease modifying therapies 384
Treatment of clinically isolated syndrome 386
Treatment of SPMS, PPMS, and PRMS 386
Overview of treatment of relapsing forms of MS 386
Treatment pipeline 386
Further reading 386
90 Multiple sclerosis in children 387
Epidemiology 387
Pathophysiology 387
Clinical features 387
Investigations 388
MRI 388
Laboratory tests 389
Differential diagnosis 389
Treatment 389
Acute treatment 389
Disease?modifying therapies 389
Multidisciplinary team approach 390
Future directions 390
Further reading 390
91 Neuromyelitis optica spectrum disorders 391
Current status 391
Pathology and pathophysiology 391
Clinical, imaging, and laboratory features 392
Serological testing 393
Diagnosis 393
Seropositive NMOSD 394
Seronegative NMOSD or of unknown serostatus 394
Optic neuritis 394
Myelitis 396
Area postrema syndromes 396
Acute brainstem or diencephalic syndromes 396
Treatment 396
Further reading 397
92 Acute disseminated encephalomyelitis 398
Clinical features 398
Laboratory findings in ADEM 398
MRI features of ADEM 398
Differential diagnosis 399
Treatment 400
Prognosis 400
Pathobiology 401
Further reading 401
93 Isolated inflammatory demyelinating syndromes 402
Features common to all isolated inflammatory demyelinating syndromes 402
Epidemiology 402
Pathophysiology 402
Clinical features 402
Investigations 403
Treatment 403
The clinical syndromes 403
Optic neuritis 403
(Transverse) myelitis 403
Brainstem inflammatory lesions 405
Cerebral hemisphere inflammatory lesions 405
Further reading 405
94 Osmotic demyelination syndromes 406
Epidemiology 406
Pathophysiology 406
Clinical features 406
Investigations 407
Treatment 407
Further reading 407
95 Concentric sclerosis (Baló’s disease) 408
Epidemiology 408
Pathophysiology 408
Clinical features 408
Investigations 409
Laboratory findings 409
Imaging 409
Treatment 409
Further reading 409
Part 11 Specific Toxicities and Deficiencies 410
96 Neurotoxicology 410
Heavy metal intoxication 410
Arsenic 410
Lead 410
Manganese 411
Mercury 412
Elemental mercury 412
Inorganic mercury salts 412
Organomercury compounds 412
Organic solvents or compounds 413
Acrylamide 413
Carbon disulfide 413
N?hexane and methyl?n?butyl ketone 414
Toluene 415
Thallium 415
Gases 415
Carbon monoxide 415
Hydrogen sulfide 416
Nitrous oxide 416
Pesticides 417
Organophosphates 417
Organic tin 417
Defoliant toxins 417
2, 4?Dichlorophenoxyacetic acid 417
Animal toxins 417
Marine toxins including ciguatoxin, saxitoxin, andtetrodotoxin 417
Plant toxins 418
Datura 418
Mushrooms 418
Podophyllotoxin (Bajiaolian) 419
Bacterial toxins 419
Botulinum toxin 419
Tetanus 419
Illicit drugs 419
1?Methyl?4?phenyl?1,2,3,6?tetrahydropyridine 419
Specific drugs 420
Disulfiram 420
Cyanide 420
Hexachlorophene 420
Methanol 420
Hypervitaminosis 421
Vitamin A 421
Regional?specific toxins 421
Polychlorinated biphenyls 421
Dimethylamine?borane 421
Tetramethylammonium hydroxide 421
Conclusions 422
Further reading 422
97 Alcohol?related neurological disorders 423
Pharmacological aspects 423
Acute alcohol intoxication 423
Alcohol withdrawal 424
Alcohol?related dementia 424
Alcoholic cerebellar degeneration 424
Marchiafava–Bignami disease 425
Alcoholic amblyopia 425
Alcoholic myopathy 425
Alcoholic polyneuropathy 425
Fetal alcohol syndrome 427
Further reading 427
98 Vitamin deficiencies 428
Group B vitamins 428
Thiamine 428
Nicotinic acid/niacin 429
Cobalamin 429
Pyridoxine 430
Pantothenic acid 430
Vitamin B deficiency syndromes 430
Thiamine deficiency 430
Nicotinic acid/niacin deficiency 431
Pyridoxine deficiency 434
Pantothenic acid deficiency 434
Other vitamins 434
Vitamin E 434
Vitamin C 434
Vitamin A 435
Vitamin D 435
Other vitamin deficiencies 435
Vitamin E deficiency 435
Vitamin A deficiency 435
Vitamin C deficiency 435
Vitamin D deficiency 435
Vitamin K deficiency 436
Conclusion 436
Further reading 436
99 Starvation, Strachan’s syndrome, and postgastroplasty syndrome 438
Starvation 438
Clinical features 438
Treatment 438
Strachan’s syndrome 438
Pathophysiology 439
Clinical features 439
Investigations 439
Differential diagnosis 439
Treatment 439
Postgastroplasty syndrome 439
Pathophysiology 440
Cinical features 440
Investigations 441
Treatment 441
Prognosis 441
Further reading 442
Part 12 Peripheral Neuropathies 443
100 Peripheral neuropathies overview 443
Epidemiology 443
Pathophysiology and clinical features 444
Investigations 444
Treatment 445
Further reading 446
101 Hereditary neuropathies 447
Hereditary motor and sensory neuropathy 447
Epidemiology 447
Pathophysiology 447
Clinical features 449
Investigations 450
Hereditary sensory and autonomic neuropathy 450
Clinical features 450
Investigations 451
Distal hereditary motor neuropathy 451
Other hereditary neuropathies 452
Refsum’s disease 452
Familial amyloid polyneuropathy 452
Friedreich’s ataxia 452
Hereditary disorders of lipid metabolism 452
Fabry’s disease 452
Porphyrias 452
Treatment 452
Further reading 452
102 Acquired neuropathies 453
Immune?mediated neuropathies 453
Guillain–Barré syndrome 453
Epidemiology 453
Pathophysiology 453
Clinical features 453
Investigations 454
Treatment 454
Chronic inflammatory demyelinating polyneuropathy 455
Epidemiology 455
Pathophysiology 455
Clinical features 455
Investigations 455
Treatment 456
Multifocal motor neuropathy 457
Epidemiology 457
Pathophyisology 457
Clinical features 457
Investigations 457
Treatment 457
Neuropathies with monoclonal gammopathies 457
Epidemiology 458
Pathophysiology 458
Clinical features 458
Vasculitic neuropathies 458
Epidemiology 458
Pathophysiology 459
Clinical features 459
Investigations 459
Treatment 459
Metabolic neuropathies 459
Diabetic neuropathy 459
Epidemiology 460
Pathophysiology 460
Clinical features 460
Investigations 460
Treatment 460
Neuropathy from B12 deficiency 461
Epidemiology 461
Pathophysiology 461
Clinical features 461
Investigations 461
Treatment 461
Other metabolic and endocrine neuropathies 461
Infectious neuropathies 461
Neuropathy from nutritional causes andalcoholism 462
Further reading 462
103 Plexopathies and mononeuropathies 463
Plexopathies 463
Epidemiology 463
Clinical features: Cervical plexus 463
Clinical features: Brachial plexus 463
Clinical features: Lumbosacral plexus 465
Investigations 466
Treatment 466
Mononeuropathies (entrapmentneuropathies) 466
Epidemiology 466
Clinical features 466
Investigations 468
Treatment 468
Further reading 468
Part 13 Neuromuscular Disorders 469
104 Amyotrophic lateral sclerosis 469
Epidemiology 469
Pathophysiology 469
Clinical features 470
Investigations 470
Treatment 471
Further reading 472
105 Progressive muscular atrophy 473
Epidemiology 473
Pathophysiology 473
Clinical features 473
Differential diagnosis 473
Multifocal motor neuropathy 473
Multiple radiculopathies 473
Inclusion body myositis 473
Brachial amyotrophic diplegia (O’Sullivan?McLeod syndrome or flail arm syndrome) 474
Monomelic amyotrophy (Hirayama syndrome) 474
Lead toxicity 474
X?linked bulbospinal muscular atrophy (Kennedy’s disease) 474
Poliomyelitis and post?polio syndrome 474
Brachial plexitis 474
Shingles 474
Adult?onset SMA (SMA type 4) 474
Adult?type hexosamidase A deficiency (late?onset Tay–Sachs disease) 474
Madras motor neuron disease 474
Hereditary motor neuropathy 474
Treatment 474
Further reading 474
106 Primary lateral sclerosis 475
Epidemiology 475
Pathophysiology 475
Clinical features 475
Investigations 476
Treatment 476
Further reading 476
107 Hereditary spastic paraplegia 477
Epidemiology 479
Pathophysiology 479
Clinical features 479
Classification based on genotyping 479
Investigations 480
Treatment 480
Further reading 481
108 Spinal muscular atrophy 482
Epidemiology 482
Pathophysiology 482
Clinical features 482
Proximal SMA 482
Non?proximal SMA 484
Investigations 485
Treatment 485
Future prospects 485
Further reading 485
109 Post polio syndrome 486
Epidemiology 486
Disease mechanisms 486
Clinical features 487
Investigations 487
Treatment 487
Further reading 488
110 Limb?girdle muscular dystrophies 489
Epidemiology 489
Pathophysiology 489
Clinical features 491
Investigations 491
Differential diagnosis 492
Treatment 492
Further reading 492
111 Dystrophinopathies 493
Epidemiology 493
Pathophysiology 493
Clinical features 494
Duchenne muscular dystrophy 494
Becker muscular dystrophy 494
Female carriers of dystrophinopathy 494
Investigations 495
Treatment 495
Further reading 495
112 Facioscapulohumeral muscular dystrophy 496
Epidemiology 496
Pathophysiology 496
Clinical features 496
Investigations 496
Treatment 497
Further reading 497
113 Myotonic dystrophies 498
Epidemiology 498
Pathophysiology 498
Clinical features 498
Myotonic dystrophy type 1 498
Myotonic dystrophy type 2 499
Investigations 499
Treatment 500
Further reading 501
114 Oculopharyngeal muscular dystrophy 502
Epidemiology 502
Pathophysiology 502
Clinical features 502
Investigations 503
Treatment 504
Further reading 504
115 Emery–Dreifuss syndrome and laminopathies 505
Epidemiology 505
Pathophysiology 505
Clinical features 505
Investigations 506
Treatment 506
Conclusion 507
Further reading 507
116 Muscle channelopathies 508
Non?dystropic myotonias 508
Epidemiology 508
Pathophysiology 508
Clinical features 508
Investigations 510
Treatment 510
Conclusion 510
Further reading 510
117 Congenital dystrophies and myopathies 511
Epidemiology 512
Pathophysiology 512
Clinical features 513
Congenital muscular dystrophy 513
Laminin ??2 (merosin)–deficient CMD 513
Ullrich CMD (collagen VI–deficient CMD) 513
Rigid spine congenital muscular dystrophy 513
Congenital muscular dystrophies caused by glycosylation defects of ??dystrophycan (dystroglycanopathy) 513
Congenital myopathies 513
Central core disease 513
Nemaline (rod) myopathy 514
Centronuclear myopathies 514
Congenital myopathy with fiber?type disproportion 514
Investigations 514
Treatment 515
Further reading 515
118 Congenital myasthenic syndromes 516
Epidemiology 516
Pathophysiology 516
Clinical features 516
Investigations 517
Treatment 517
Further reading 519
119 Distal myopathies 520
Epidemiology 520
Pathophysiology 520
Clinical, pathological, and genetic features 520
Investigations 522
Treatment 522
Conclusions 522
Further reading 522
120 Polymyositis 523
Clinical manifestations 523
Association conditions 523
Immunopathogenesis 524
Differential diagnosis 524
Diagnosis 524
Therapy 525
Steroids and nonsteroidal immunosuppressive agents 525
Intravenous immunoglobulin 526
Treatment failures and new agents 526
Prognosis 526
Further reading 526
121 Dermatomyositis 527
Clinical manifestations 527
Imunopathogenesis 528
Diagnosis 529
Treatment 529
Prognosis and complications 530
Further reading 530
122 Inclusion body myositis 531
Epidemiology 531
Pathophysiology 531
Clinical features 531
Investigations 531
Treatment and management 532
Glucocorticoids and cytotoxic agents 532
Immunotherapy 533
Other therapies 533
Conclusions 533
Further reading 533
123 Immune?mediated necrotizing myopathies 534
Epidemiology 534
Pathophysiology 534
Clinical features 535
Treatment and management 536
Further reading 536
124 Toxic myopathies 537
Alcohol?associated myopathy 537
Statin myopathy 537
Corticosteroid myopathy 539
Critical illness myopathy and malignanthyperthermia 539
Further reading 539
125 Critical illness neuromuscular disorders 540
Epidemiology 540
Pathophysiology 540
Clinical features 540
Investigations 540
Treatment and management 541
Further reading 541
126 Exercise intolerance and myoglobinuria 542
Testing of exercise intolerance 542
Handgrip exercise tests 542
Testing of work capacity 542
Myoglobinuria 543
Causes of myoglobinuria 543
Hereditary causes of myoglobinuria 543
Disorders of carbohydrate metabolism 543
Disorders of fatty acid oxidation 543
Mitochondrial disorders 544
Muscular dystrophies 544
Acquired causes of myoglobinuria 544
Further reading 545
127 Muscle cramps 546
Pathophysiology 546
Clinical features 546
Investigations 547
Treatment 548
Acknowledgments 548
Further reading 548
128 Myasthenia gravis 549
Epidemiology 549
Pathophysiology 549
Neuromuscular junction transmission and the acetylcholine receptor 549
Acetylcholine receptor antibody and the autoimmune disorder 549
The role of the thymus 550
Clinical manifestations 550
Symptoms and signs 550
Associated disorders 550
Investigations 550
Antibody detection 550
Electrophysiological studies 550
Diagnosis 550
Treatment 551
Physiological or symptomatic therapy 551
Immunosuppressive and immunomodulatory therapy 551
Drugs that can worsen MG 551
Emergency care of myasthenic patients 551
Further reading 552
129 Lambert–Eaton myasthenic syndrome 553
Epidemiology 553
Pathophysiology 553
Clinical features 553
Investigations 554
Treatment 554
Further reading 554
130 Neuromuscular transmission disorders caused by toxins and drugs 555
Botulism 555
Organophosphate intoxication 556
Medication?induced neuromuscular junction disorders 556
Conclusion 556
Further reading 556
Part 14 Neurogenetics 557
131 Genetics in neurology 557
Basic definitions 557
Mendelian genetics 557
Autosomal dominant inheritance 557
Autosomal recessive inheritance 557
X?linked dominant inheritance 557
X?linked recessive inheritance 557
Codominant inheritance 558
Other genetic forms of inheritance 558
Reduced penetrance 558
Variable expressivity 558
Trinucleotide repeats 558
Fragile X syndrome 558
Fragile X–associated tremor/ataxia syndrome 558
Kennedy’s disease 558
Genomic imprinting 559
Mitochondrial disorders 559
Gene dosage disorders 559
Susceptibility genes 559
Neurocutaneous disorders 560
Neurofibromatosis 560
Von Hippel–Lindau disease 560
Tuberous sclerosis 560
Sturge–Weber syndrome 560
Xeroderma pigmentosum 560
Incontinentia pigmenti 561
Other genetic disorders 561
Ataxia telangiectasia 561
Cerebrotendinosis xanthomatosis 561
Treatment for genetic disorders 561
Enzyme replacement 561
Mechanism?based therapy 561
Tuberous sclerosis 561
Fragile X syndrome 562
Phenylketonuria 562
X?adrenoleukodystrophy 562
Maple syrup urine disease 562
Nonsense suppression therapy 562
Gene therapy 562
Somatic gene therapy 563
Germline gene therapy 563
Further reading 563
Part 15 Neuro?otology 564
132 Neuro?otology 564
Epidemiology 564
International considerations in patients with neuro?otological disorders 564
Pathophysiology 564
Clinical approach to patients with dizziness 565
History of present illness 565
General medical examination 566
General neurological examination 566
Neuro?otological examination 566
Common presentations of dizziness 568
Acute severe dizziness 568
Recurrent attacks of dizziness 569
Recurrent positional dizziness 569
Non?specific dizziness 569
Imbalance 569
Management of the patient with dizziness 570
Investigations 570
Imaging studies 570
Vestibular laboratory tests 571
Auditory testing 571
Common presentations of hearing loss 571
Asymmetric sensorineural hearing loss 571
Sudden sensorineural hearing loss 571
Hearing loss with age 571
Genetic hearing loss 571
Common presentations of tinnitus 571
Conclusion 572
Further reading 572
Part 16 Neuro?ophthalmology 573
133 Neuro?ophthalmology 573
Clinical approach to visual loss 573
Transient visual loss 573
Duration 573
Pattern of visual loss 574
Ocular disorders 574
Sudden visual loss 574
Acute to subacute visual loss 575
Optic neuritis 575
Neuromyelitis optica spectrum disorder 575
Leber’s hereditary optic neuropathy 575
Neuroretinitis 576
Idiopathic orbital inflammatory syndrome 576
Thyroid?associated orbitopathy 576
Acute glaucoma 576
Radiation optic neuropathy 576
Methanol toxicity 577
Chronic visual loss 577
Clinical approach to double vision 579
Horizontal diplopia 579
Horizontal diplopia with other neurological symptoms 579
Vertical diplopia without ptosis 579
Vertical diplopia with ptosis 579
Vertical diplopia with bilateral ptosis 579
Vertical diplopia with other neurological disorders 579
Treatment of diplopia 580
Clinical approach to pupillary abnormalities 580
Small pupil 580
Unilateral small pupil 580
Bilateral small pupils 580
Large pupil 580
Further reading 581
Part 17 Neuro?oncology 583
134Neuro?oncology overview 583
135 High?grade astrocytomas 585
Epidemiology 585
Genetics 585
Clinical features 585
Investigations 585
Radiological assessment 585
Pathology and molecular biology assessment 585
Prognostic factors 586
Treatment 586
Symptomatic management 586
Surgery 586
Radiotherapy 587
First?line treatment 587
Treatment at recurrence 587
Conclusion 588
Further reading 588
136 Low?grade astrocytomas 589
Epidemiology and clinical features 589
Pathophysiology and molecular biology 589
Investigations 589
Prognosis 590
Treatment and management of astrocytomas 590
Overall approach 590
What is the reliability of the neuroradiological diagnosis “presumptive low?grade glioma”? 590
What evidence is available to decide at what moment diagnosis should be obtained and treatment should be initiated? 591
Which patients should undergo early diagnosis and treatment? 591
Surgery 591
Radiation therapy 591
Chemotherapy 591
Further reading 592
137 Low?grade and anaplastic oligodendrogliomas 593
Epidemiology 593
Pathophysiology 593
Macroscopic features 593
Molecular features 594
Clinical features 594
Radiographic features 594
Treatment 595
Low?grade oligodendroglioma 595
Radiotherapy 595
Chemotherapy 596
High?grade oligodendroglioma 596
Initial management 596
Management at progression 596
Conclusion 597
Further reading 597
138 Brainstem glioma 598
Epidemiology 598
Pathophysiology 598
Clinical features 598
Investigations 598
Treatment 598
Surgery 598
Radiation therapy 599
Chemotherapy 599
Adults 599
Emerging therapies 599
Further reading 599
139 Intracranial ependymoma 600
Treatment 600
Surgery 600
Radiotherapy 600
Chemotherapy 601
Recurrent ependymoma 601
Conclusion 601
Further reading 601
140 Nerve sheath tumors 602
Epidemiology and pathophysiology 602
Benign nerve sheath tumors 602
Neurofibromatosis 603
Clinical features and investigations 603
Treatment 604
Further reading 604
141 Meningiomas 605
Epidemiology 605
Pathophysiology 605
Gonadal hormones 605
Histology 605
Clinical presentation 605
Investigations 606
Treatment 606
Surgery 606
Radiation 606
Chemotherapy 606
Outcomes 607
Further reading 607
142 Adult medulloblastoma 608
Epidemiology 608
Clinical features 608
Investigations 608
Staging 608
Tumor biology 608
SHH group 609
Other groups in adults 609
Treatment 609
Surgery 609
Radiation therapy 609
Chemotherapy 609
Further reading 610
143 Primary central nervous systemlymphoma 611
Epidemiology 611
Pathophysiology 611
Clinical features 611
Investigations 611
Treatment 612
Salvage therapy 613
Treatment?related toxicity 613
HIV/AIDS?related central nervous system lymphoma 613
Treatment guidelines, clinical trials, and future direction 613
Further reading 614
144 Brain metastases 615
Epidemiology 615
Pathophysiology 615
Clinical features 615
Prognosis 615
Investigations 616
Treatment 616
Medical management 616
Surgery 616
Radiotherapy 616
Radiation therapy and radiosensitizers 616
Radiosurgery 616
Systemic treatment 617
Management in common solid tumors 617
Non?small cell lung cancer 617
Small cell lung cancer 617
Breast cancer 617
Malignant melanoma 617
Germ cell tumors 618
Conclusion 618
Further reading 618
145 Leptomeningeal metastases 619
Pathophysiology 619
Clinical features 619
Investigations 619
Imaging 619
CSF examination 619
Prognosis 619
Treatment and management 620
Symptomatic treatment 620
Surgery 620
Radiotherapy 620
Chemotherapy and targeted therapy 620
Conclusion 621
Further reading 621
146 Spinal epidural metastases 622
Epidemiology 622
Pathophysiology 622
Clinical features 622
Investigations 622
Differential diagnosis 623
Treatment 623
Prognosis 624
Pediatric epidural metastases 624
Further reading 624
147 General approach to the diagnosis and treatment of paraneoplastic neurological disorders 625
Diagnosis 625
Treatment 625
Specific syndromes 626
Paraneoplastic cerebellar degeneration 626
Paraneoplastic encephalomyelitis 626
Limbic encephalitis and brainstem encephalitis 627
Paraneoplastic sensory neuronopathy 627
Paraneoplastic opsoclonus?myoclonus 627
Further reading 627
Part 18 Sleep Disorders 628
148 Insomnia 628
Epidemiology 628
Pathophysiology 628
Clinical features 628
Investigations 628
Treatment 629
Drug therapy 629
Psychological and behavioral therapy 630
Overall management strategies in the treatment of insomnia 630
Specific behavioral treatments 630
Conclusion 630
Further reading 630
149 Narcolepsy 631
Epidemiology 631
Pathophysiology 631
Clinical features 631
Excessive daytime sleepiness 632
Cataplexy 632
Sleep paralysis 632
Hallucinations 632
Sleep fragmentation 632
Automatic behaviors 632
Investigations 632
Treatment 633
Further reading 633
150 Idiopathic hypersomnia 634
Epidemiology 634
Pathophysiology 634
Clinical features 634
Excessive daytime sleepiness 634
Autonomic dysfunction 635
Other features 635
Differential diagnosis 635
Diagnostic assessment 635
Treatment 635
Further reading 635
151 Obstructive sleep apnea 636
Epidemiology 636
Pathophysiology 636
Risk factors 636
Clinical features 637
Investigations 637
Treatment 637
Positive airway pressure 637
Surgery 637
Oral appliances 638
Hypoglossal nerve stimulators 638
Further reading 638
152 Restless legs syndrome and periodic limb movement disorders 639
Epidemiology 639
Pathophysiology 639
Clinical features 639
RLS 639
Diagnostic criteria (ICSD?3, 2014) 640
Periodic leg movements (PLM) and periodic limb movement disorder (PLMD) 640
Investigations 640
Treatment 640
Augmentation 641
Further reading 641
153 Circadian rhythm sleep–wake disorders 642
Epidemiology 642
Pathophysiology 642
Clinical features 642
Investigations 642
Diagnostic criteria that must be met for all CRSWD 643
Classification of rhythm sleep–wake disorders 643
Jet lag disorder 643
Circadian sleep–wake disorder not otherwise specified 643
Treatment 643
Conclusions 643
Acknowledgments 643
Further reading 643
154 Arousal disorders 644
Epidemiology and risk factors 644
Pathophysiology 644
Clinical features and differential diagnoses 644
Investigations 645
Treatment 645
Clinical course 645
Conclusion 645
Further reading 645
155REM sleep behavior disorder 646
Epidemiology and risk factors 646
Pathophysiology 646
Clinical features and differential diagnoses 646
Investigations 647
Treatment 647
Clinical course 647
Conclusion 647
Further reading 647
156 Paroxysmal nocturnal dystonia 648
Clinical features 648
Genetics 648
Differential diagnosis 648
Investigations 648
Treatment 648
Further reading 649
157 Sleep abnormalities in neurological disorders 650
Sleep?disordered breathing 650
Stroke 650
Neuromuscular disease 650
Headache syndromes 650
Restless legs syndrome and periodic limb movement disorder 650
Neuropathy 651
Parkinson’s disease 651
Parasomnias 651
NREM parasomnias 651
REM behavior disorder 651
Pathological hypersomnias 651
Narcolepsy 651
Idiopathic hypersomnia 651
Further reading 651
Part 19 Spinal Cord Disorders 652
158 Spinal cord disorders 652
Cervical disc disease 653
Lumbar disc disease 653
Natural course of disc herniation 654
Diagnosis 654
Treatment 654
Cervical spondylosis 654
Lumbar spinal stenosis 655
Paget’s disease 655
Fibrous dysplasia 656
Other compressive disorders 657
Investigation 657
Further reading 657
159 Ischemic and congestive myelopathies 658
Acute ischemic myelopathy 658
Congestive myelopathy 659
Further reading 660
160 Syringomyelia 661
Epidemiology 661
Pathophysiology 661
Clinical features 662
Sensory signs 662
Motor signs 662
Autonomic signs 662
Investigations 662
Treatment 662
Further reading 663
Part 20 Pediatric Neurology 664
161 Neonatal neurology 664
Hypoxic ischemic encephalopathy 664
Intraventricular hemorrhage 664
Infection 665
Inborn errors of metabolism 665
Seizures 665
Neurotoxin exposure 665
Further reading 666
162 Neurodevelopmental disorders 667
Developmental delay and intellectual disability 667
Clinical evaluation 667
Diagnostic approach 667
Management 668
Selected genetic neurodevelopmental disorders 668
Tuberous sclerosis complex 668
Neurological features 668
Diagnostic approach 668
Management 668
Neurofibromatosis type 1 669
Neurological features 669
Diagnostic approach 669
Management 669
Rett syndrome 669
Neurological features 669
Diagnostic approach 670
Management 670
Conclusion 670
Further reading 670
163 Floppy infant syndrome 671
Clinical features and evaluation 671
Laboratory investigations 671
Approach for genetic diagnosis 672
Special considerations for cost?effective genetic analysis 673
Therapeutic approach 673
Conclusion 674
Further reading 674
164 Storage disorders 675
Lipidoses 675
Neuronal ceroid lipofuscinosis 675
Gangliosidoses 676
Sphingomyelinoses 677
Cerebrosidoses 678
Mucolipidoses 678
Fabry disease 679
Mucopolysaccharidosis 679
Mucopolysaccharidosis type I (Hurler syndrome?type IH, Scheie syndrome?type IS, and Hurler–Scheie syndrometypeIH/S) 679
Mucopolysaccharidosis type II (Hunter syndrome) 679
Mucopolysaccharidosis type III (Sanfilippo syndrome) 680
Mucopolysaccharidosis type IV (Morquio syndrome) 680
Mucopolysaccharidosis type VI (Maroteaux–Lamy syndrome) 680
Mucopolysaccharidosis type VII (Sly syndrome) 680
Glycogenoses 680
Glycogen storage disease type I (von Gierke’s disease) 680
Glycogen storage disease type II (Pompe disease) 680
Glycogen storage disease type V (McArdle disease) 681
Leukodystrophies 681
Pelizaeus–Merzbacher disease 681
Cockayne syndrome 681
Alexander disease 681
Canavan disease 681
Krabbe disease (globoid cell leukodystrophy) 682
Metachromic leukodystrophy 682
Adrenal leukodystrophy 682
Refsum disease 682
Other lipidoses 682
Abetalipoproteinemia 682
Tangier disease 683
Further reading 683
165 Fatty acid oxidation disorders 684
Physiology 684
Epidemiology and genetics 684
Clinical features and pathophysiology 685
Acyl?CoA dehydrogenase deficiencies 685
Carnitine deficiency 685
Carnitine palmitoyltransferase deficiency 686
Investigations 686
Treatment and management 686
Prenatal diagnosis 687
Conclusion 687
Further reading 687
166 Disorders of amino acid, organic acid, and ammonia metabolism 688
Neonatal disorders 688
Maple syrup urine disease 688
Epidemiology 688
Clinical features 688
Diagnosis 689
Treatment 689
Urea cycle disorders 689
Epidemiology 689
Clinical features 689
Pathophysiology and treatment 689
Propionic and methylmalonic acidemia 690
Epidemiology 690
Clinical features 690
Treatment 690
Progressive diseases of infancyand childhood 691
Phenylketonuria 691
Epidemiology 691
Clinical features 691
Treatment 692
Tetrahydrobiopterin?deficient hyperphenylalaninemia 692
Epidemiology 692
Diagnosis 692
Treatment 692
Homocystinuria (homocystinemia) 692
Epidemiology 693
Treatment 693
Disorders of lysine and tryptophan metabolism: Glutaric acidemia type I 693
Epidemiology 693
Pathophysiology 693
Clinical features 693
Treatment 693
Biotinidase deficiency 694
Pathophysiology 694
Clinical features 694
Treatment 694
Further reading 694
167 Pediatric neurotransmitter diseases 695
Pathophysiology 695
Monoamine neurotransmitter disorders 695
DOPA?responsive dystonias 695
GTP?cyclohydrolase I deficiency (GCH?1 deficiency, Segawa disease) 696
Tyrosine hydroxylase deficiency 696
Sepiapterin reductase deficiency 697
Other monoamine neurotransmitter disorders 697
GABA neurotransmitter disorders 697
Pyridoxine?dependent epilepsy 697
GABA transaminase deficiency 698
Conclusion 698
Further reading 698
168 Mitochondrial encephalomyopathies 699
Mitochondrial genetics 699
Genomic organization 699
Replication, transcription, and translation of mtDNA 699
Mitochondrial DNA inheritance and transmission 700
Heteroplasmy and threshold effect 701
Segregation 701
Genetic and functional classification 701
Disorders of mtDNA 701
Kearns–Sayre syndrome 701
Progressive external ophthalmoplegia 702
Pearson syndrome 702
Mitochondrial encephalopathy, lactic acidosis, and stroke?like episodes 702
Myoclonic epilepsy and ragged red fibers 703
Leber’s hereditary optic neuropathy syndrome 703
Neuropathy, ataxia, retinitis pigmentosa syndrome 703
Maternally-inherited Leigh Syndrome 704
Disorders of nDNA 704
Leigh syndrome 704
Autosomal dominant or recessive progressive external ophthalmoplegia 704
Mitochondrial neurogastrointestinal encephalomyopathy 704
Hepatocerebral syndromes 706
Ataxia syndromes 706
Encephalomyopathy syndromes 707
Mitochondrial leukodystrophies 707
Neurodegenerative disorders 707
Epidemiology 707
Neurological manifestations 707
Diagnosis 708
History 708
Physical examination 709
Investigations 709
Basic laboratory tests 709
Neuroimaging 709
Neurophysiological studies 709
Exercise physiology 710
Muscle biopsy 710
Molecular genetic testing 710
Mitochondrial disease scales 711
Therapy 711
Symptomatic therapy 711
Pharmacological therapy 711
Removal of noxious metabolites 711
Administration of electron acceptors 711
Administration of vitamins and cofactors 711
Administration of oxygen radical scavengers 711
Gene therapy 712
Cytoplasmic transfer 712
Nucleoside and nucleotide treatment 712
Mitochondrial biogenesis modulators 712
Conclusion 712
Further reading 712
169 Disorders resulting from transporter defects 713
Disorders of copper transporters 713
Wilson’s disease 713
Clinical features 713
Pathophysiology 714
Diagnosis 714
Treatment 714
Menkes disease 715
MEDNIK syndrome 716
Manganese transporter deficiency 716
Clinical features 716
Pathophysiology 717
Diagnosis 717
Treatment 717
Carnitine O?palmitoyltransferase 2 deficiency 717
Clinical features 717
Pathophysiology 717
Diagnosis 717
Treatment 717
GLUT1 deficiency syndrome 717
Clinical features 718
Pathophysiology 718
Diagnosis 718
Treatment 718
Hereditary folate malabsorption 718
Clinical features 718
Pathophysiology 718
Diagnosis 718
Treatment 719
Conclusion 719
Further reading 719
170 The porphyrias 720
Pathogenesis of neurological dysfunction 720
Epidemiology 721
Clinical features 721
Erythropoietic porphyrias 721
Hepatic porphyrias 721
Neurological manifestations 722
Extra?neurological manifestations 722
Differential diagnosis 722
Porphyric attacks caused by antiepileptic medications 722
Treatment 722
Conclusion 723
Acknowledgments 723
Further reading 723
Part 21 Central Nervous System Trauma, Pain, and Headache 724
171 Traumatic brain injury 724
Epidemiology 724
Pathophysiology 724
Clinical features 725
Mild traumatic brain injury/concussion 725
Moderate to severe traumatic brain injury 725
Investigations 725
Treatment 726
Mild traumatic brain injury/concussion 726
Moderate to severe traumatic brain injury 726
Chronic management of sequelae 727
Further reading 727
172 Spinal injury 728
Epidemiology 728
Pathophysiology 728
Clinical features 729
Investigations 729
Treatment 730
Further reading 730
173 Whiplash injury 731
Epidemiology 731
Pathophysiology 731
Clinical features 731
Investigations 732
Treatment 732
Conclusion 732
Further reading 732
174 Decompression sickness 733
Epidemiology 733
Pathophysiology 733
Clinical features 733
Treatment 734
Further reading 734
175 Pain 735
Classification of pain 735
Pathophysiology of pain 735
Nociceptors 735
Steps in the pain pathway 736
Ascending modulation 736
Descending modulation 736
Neuropathic pain 737
Visceral pain 737
Neuralgias and neuropathic pain syndromes 738
Entrapment neuropathies 738
Complex regional pain syndromes 739
Central pain syndromes 739
Pain due to systemic disorders 739
Pain in patients with cancer 739
Pain due to undiagnosed medical disease 739
Approach to the patient with pain 739
Treatment 739
Opioids 740
Further reading 742
176 Headache 743
Migraine headache 743
Epidemiology 743
Pathophysiology 743
Clinical features 743
Differential diagnosis 744
Investigations 744
Treatment 744
Tension?type headache 744
Pathophysiology 744
Clinical features 744
Differential diagnosis 745
Investigations 745
Treatment 745
Cluster headache 745
Epidemiology 745
Pathophysiology 745
Clinical features 745
Differential diagnosis 745
Investigations 745
Treatment 745
Idiopathic intracranial hypertension 745
Epidemiology 745
Pathophysiology 745
Clinical features 745
Differential diagnosis 746
Investigations 746
Treatment 746
Low?pressure headache 746
Epidemiology 746
Pathophysiology 746
Clinical features 746
Investigations 746
Treatment 746
Other headache syndromes 746
Cough and exercise headache 746
Benign thunderclap headache 747
Further reading 747
177 Facial and neck pain 748
Facial pain 748
Trigeminal neuralgia 748
Glossopharyngeal–vagoglossopharyngeal neuralgia 749
Neck pain and headache 749
Occipital neuralgia 750
Carotidynia 750
Cervicogenic headache 750
Further reading 751
178 Chronic fatigue syndrome 752
Epidemiology 752
Etiology 752
Pathophysiology 752
Diagnosis 752
Proposed diagnostic criteria for ME/CFS 752
Treatment 753
Further reading 753
179 Fibromyalgia 754
Epidemiology 754
Pathogenesis 754
Diagnosis 754
Clinical features 754
Differential diagnosis 755
Management 755
Further reading 756
Index 757
Supplemental Images 779
EULA 786

Produkt-Details

• Erscheint lt. Verlag: 13.4.2016
• Sprache: englisch
• Themenwelt: Medizin / Pharmazie ► Medizinische Fachgebiete ► Neurologie
• Schlagworte: Clinical Neurology • CNS Trauma • Daniel D. Truong • dementias • demyelinating disorders • Diagnosis • Disorders of Cerebrovacular Autonomic Control • Global Trends • Headache • Infectious Diseases • Inflammatory Vasculopathies • International Neurology • Management • Medical Science • Medizin • movement disorders • Neurogenetic • Neurological Disease • Neurologie • Neurology • Neuromuscular disorders • Neuro-oncology • neuroophthalmology • neurootology • Pain • Paroxysmal disorders • pediatric neurology • peripheral neuropathies • Prion diseases and Neurovirology • Robert P. Lisak • Roongroj Bhidayasiri • Sleep Disorders • Specific toxicities and deficiencies • spinal cord • The Vasculitides • Vascular • William M. Carroll
• ISBN-10: 1-118-77735-2 / 1118777352
• ISBN-13: 978-1-118-77735-0 / 9781118777350