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Beschreibung

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

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Dedication

To my many teachers and mentors over the years, most especially Lewis Rowland, Robert Fishman, Sidney Carter, Donald Silberberg, Donald Schotland, and Arthur Asbury in neurology, Burton Zweiman in immunology and clinical immunology, Marian Kies in neuroscience, neurochemistry and glial biology, and Elvin Kabat in immunology. To my collaborators, colleagues, students, residents, fellows, and patients over the years. And finally and most importantly to my family, including my late parents Irving and Sylvia Lisak, my sister Nancy Lisak Sager, my wife Deena, my children Ilene and Michael, and my grandchildren Samuel, Isabella, Vivienne, and Sophie.

RPL

To my mother Cam Tran, my wife Diane Truong, and my friend Marcia Manker for whose support I am indebted, to my associates Thong Nguyen, Mayank Pathak, Karen Frei, Anumantha Kanthasamy, Rae Matsumoto, KK Tai, Phuong Pham, Steven Jenkins, Dave Brown and Christina Nguyen whose loyalty I cherish, and to Emily Gelskey, Suzanne Mellor, Gianni Pezzoli, Khalid Sheikh, Erik Wolters and Rick Warren from whose wisdom I learn.

DDT

To my wife Kathryn and our daughters Gemma, Bonita, and Laura, I thank you for your support, encouragement, and forbearance, and I thank all who have contributed so unstintingly to make this textbook truly unique.

WMC

To my beloved grandmother Pranom Chivakiat, my parents Mitr and Nisaratana Bhidayasiri, my family, Nucharee Yoovidhya and Bhiradej Yoovidhya Bhidayasiri, for their continuing support, love, and understanding, all my teachers of neurology, and lastly my patients who have taught me much about neurology.

RB

International Neurology

Second edition

Edited by

Robert P. Lisak MD 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

Clinical Professor, University of California, Riverside Riverside, CA, USA The Truong Neuroscience Institute Orange Coast Memorial Medical Center Fountain Valley, CA, USA

William M. Carroll MBBS MD FRACP FRCP(E)

Clinical Professor of Neurology, Western Australian Neuromuscular Research Institute University of Western Australia, Sir Charles Gairdner Hospital, Perth, Western Australia Australia

Roongroj Bhidayasiri MD FRCP FRCPI

Professor of Neurology and Director Chulalongkorn Center of Excellence for Parkinson’s Disease & Related Disorders, Faculty of Medicine, Chulalongkorn University Hospital Bangkok, Thailand

Department of Rehabilitation Medicine, Juntendo University, Tokyo, Japan

Foreword by John Walton (Lord Walton of Detchant)

This edition first published 2016 © 2016 by John Wiley & Sons, Ltd

Registered office: John Wiley & Sons, Ltd, The Atrium, Southern Gate, Chichester, West Sussex, PO19 8SQ, UK

Editorial offices: 9600 Garsington Road, Oxford, OX4 2DQ, UK The Atrium, Southern Gate, Chichester, West Sussex, PO19 8SQ, UK 111 River Street, Hoboken, NJ 07030-5774, USA

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The right of the author to be identified as the author of this work has been asserted in accordance with the UK Copyright, Designs and Patents Act 1988.

All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or by any means, electronic, mechanical, photocopying, recording or otherwise, except as permitted by the UK Copyright, Designs and Patents Act 1988, without the prior permission of the publisher.

Designations used by companies to distinguish their products are often claimed as trademarks. All brand names and product names used in this book are trade names, service marks, trademarks or registered trademarks of their respective owners. The publisher is not associated with any product or vendor mentioned in this book. It is sold on the understanding that the publisher is not engaged in rendering professional services. If professional advice or other expert assistance is required, the services of a competent professional should be sought.

The contents of this work are intended to further general scientific research, understanding, and discussion only and are not intended and should not be relied upon as recommending or promoting a specific method, diagnosis, or treatment by health science practitioners for any particular patient. The publisher and the author make no representations or warranties with respect to the accuracy or completeness of the contents of this work and specifically disclaim all warranties, including without limitation any implied warranties of fitness for a particular purpose. In view of ongoing research, equipment modifications, changes in governmental regulations, and the constant flow of information relating to the use of medicines, equipment, and devices, the reader is urged to review and evaluate the information provided in the package insert or instructions for each medicine, equipment, or device for, among other things, any changes in the instructions or indication of usage and for added warnings and precautions. Readers should consult with a specialist where appropriate. The fact that an organization or Website is referred to in this work as a citation and/or a potential source of further information does not mean that the author or the publisher endorses the information the organization or Website may provide or recommendations it may make. Further, readers should be aware that Internet Websites listed in this work may have changed or disappeared between when this work was written and when it is read. No warranty may be created or extended by any promotional statements for this work. Neither the publisher nor the author shall be liable for any damages arising herefrom.

Library of Congress Cataloging-in-Publication Data

Names: Lisak, Robert P., editor. | Truong, Daniel, editor. |     Carroll, William M., editor. |     Bhidayasiri, Roongroj, editor. Title: International neurology / edited by Robert P. Lisak, Daniel D. Truong,     William M. Carroll, Roongroj Bhidayasiri ; foreword by John Walton (Lord     Walton Of Detchant). Description: Second edition. | Chichester, West Sussex ; Hoboken, NJ : John     Wiley & Sons, Inc., 2016. | Includes bibliographical references and index. Identifiers: LCCN 2015046045 (print) | LCCN 2015048295 (ebook) | ISBN     9781118777367 (cloth) | ISBN 9781118777350 (pdf) | ISBN 9781118777343     (epub) Subjects: | MESH: Nervous System Diseases | Neurology—methods Classification: LCC RC346 (print) | LCC RC346 (ebook) | NLM WL 140 | DDC     616.8—dc23 LC record available at http://lccn.loc.gov/2015046045

A catalogue record for this book is available from the British Library.

Wiley also publishes its books in a variety of electronic formats. Some content that appears in print may not be available in electronic books.

Contents

Editors

List of contributors

Foreword

Endorsement from the World Federation of Neurology

Preface

PART 1 Vascular Disorders

1 Overview of stroke

2 Ischemic stroke and transient events, TIA

History

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Conclusions

Further reading

3 Atherothrombotic disease

Site of atheroslerosis

Mechanisms of stroke and clinical manifestations

Investigations

Management of large vessel atherosclerosis

Further reading

4 Occlusive disease of small penetrating arteries

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

5 Ischemic white matter disease (Binswanger’s disease)

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

6 Brain embolism

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

7 Systemic hypotensive injury (border-zone infarction)

Epidemiology

Pathophysiology

Clinical features

Investigation

Treatment

Further reading

8 Dissection of the cervicocerebral arteries

Introduction and epidemiology

Etiology and pathophysiology

Clinical features

Investigations

Treatment

Further reading

9 Coagulation and Hematological Disorders in Stroke

Hyperviscosity disorders

Prothrombotic disorders

Homocysteinemia

Antiphospholipid syndrome

Platelet dysfunction disorders

Other coagulopathies

Investigations

Treatment

Further reading

10 Hemorrhagic strokes

Primary intracerebral hemorrhage

Subarachnoid hemorrhage

Cerebral arteriovenous malformation

Cerebral amyloid angiopathy

Further reading

11 Strokes in children and young adults

Etiology and epidemiology

Clinical features

Investigations

Treatment

Further reading

12 Other cerebrovascular syndromes

Stroke and migraine

Stroke and substance abuse

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) and related conditions

Hypertensive encephalopathy posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstrictive syndrome (RCVS)

Periprocedural, postoperative, and in-hospital stroke

Rare stroke syndromes

Conclusion

Further reading

13 Cerebral venous disease

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

14 Spinal cord strokes

Spinal cord vascular supply

Spinal cord ischemia and infarction

Causes of spinal cord ischemia

Spinal vascular malformations

Investigations and management

Further reading

PART 2 Disorders of Cerebrovascular Autonomic Control

15 Reversible cerebral vasoconstriction syndrome (RCVS)

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Conclusion

Further reading

16 Posterior reversible encephalopathy syndrome (PRES)

Epidemiology

Pathophysiology

Etiology

Clinical features

Investigations

Treatment

Prognosis

Conclusion

Further reading

PART 3 The Vasculitides

17 Extracranial and intracranial granulomatous arteritis (giant cell arteritis)

Epidemiology

Pathogenesis

Clinical manifestations

Diagnostic approaches to GCA

Treatment

Prognosis

Further reading

18 Intracranial granulomatous arteritis (primary angiitis of the CNS)/idiopathic CNS vasculitis

Introduction and nomenclature

Clinical course and diagnosis

Treatment

Future developments

Further Reading

19 Takayasu’s arteritis

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

20 Polyarteritis nodosa, eosinophilic granulomatosis with polyangiitis, and overlap vasculitis syndrome

Polyarteritis nodosa (PAN)

Eosinophilic granulomatosis with polyangiitis (EGPA)

Overlap vasculitis

Further reading

21 Granulomatosis with polyangiitis (GPA)

Epidemiology

Clinical features

Investigations

Treatment

Further reading

22 Cerebrovascular disease associated with antiphospholipid antibodies

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

23 Thromboangiitis obliterans (Buerger’s disease)

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

24 Susac syndrome

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

PART 4 Inflammatory Vasculopathies

25 SLE, rheumatoid arthritis, and Sjögren’s syndrome

Systemic lupus erythematosus (SLE)

Rheumatoid arthritis

Sjögren’s syndrome

Further reading

26 Systemic sclerosis

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

27 Mixed connective tissue disease

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

28 Behcet’s syndrome and nervous system involvement

Epidemiology

Pathophysiology

Clinical features

Investigations for NBS

Differential diagnosis

Treatment of NBS

Prognosis

Further reading

29 Sarcoidosis

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

30 Autoimmune encephalitis with neuronal cell surface antibodies

Antibodies and ancillary tests

Clinical spectrum

Differential diagnoses

Treatment

Further reading

PART 5 Paroxysmal Disorders

31 Epilepsy overview

Classification

Epidemiology

Age and seizure types

Mortality

Pathophysiology

Risks and etiology

Investigations

Differential diagnosis

Treatment/management

Special populations

Conclusions

Further reading

32 Structural, genetic and unknown generalized epilepsies and syndromes

Ohtahara syndrome (EIEE)

Severe myoclonic epilepsy in infancy (SMEI) or Dravet syndrome

West syndrome (WS)

Lennox–Gastaut syndrome (LGS)

Epilepsy with myoclonic absences (EMA)

Inflammatory epilepsies

Further reading

33 Genetic (primary) idiopathic generalized epilepsies

Advances in the classification of epilepsy

Advances in molecular genetics

Febrile seizure (FS)

Severe myoclonic epilepsy of infancy (SMEI)

Childhood absence epilepsy (CAE)

Juvenile absence epilepsy (JAE)

Juvenile myoclonic epilepsy (JME)

Novel progressive myoclonic epilepsy (PME)

De novo

mutations

Advances in technologies

Further reading

34 Localization-related epilepsies

Idiopathic LRE

Monogenic focal epilepsies

Further reading

35 Neurodiagnostic tools for the paroxysmal disorders

Electrophysiology

Neuroimaging

Functional imaging

MRS

Other techniques

Conclusion

Further reading

36 Neuropharmacology of antiepileptic drugs

History of antiepileptic drugs

Mechanisms of action for antiepileptic drugs

Rationale for antiepileptic drug selection: Choice of initial drug

Other conditions treated with antiepileptic drugs

Failure of initial treatment: Rationale for replacement of initial drug or combination AED therapy

AEDs in the treatment of status epilepticus

Complications of antiepileptic drug therapy

Elective discontinuation of antiepileptic drugs

Herbal treatments as AEDs

Further reading

37 Surgical treatment of epilepsy

Investigations and indications for epilepsy surgery

Semi-invasive and invasive video EEG

Operations

Acknowledgments

Further reading

PART 6 Dementia

38 Dementia overview

Acknowledgment

39 Mild cognitive impairment and Alzheimer’s disease

Mild cognitive impairment

Alzheimer’s disease

Use of biomarkers for MCI and AD

Treatment

Further reading

40 Parkinson’s Disease Dementia, Dementia with Lewy Bodies, and Other Synucleinopathies

Parkinson’s disease dementia

Dementia with Lewy bodies

Multiple system atrophy

Further reading

41 Specific vascular syndromes

Cerebral amyloid angiopathy

Hereditary small vessel syndromes

Poststroke dementia

Further reading

42 Vascular cognitive impairment

Epidemiology

Neuropathology and pathophysiology of VCI

Clinical features

Clinical evaluation

Treatment

Conclusions

Further reading

43 Frontotemporal dementia

Epidemiology

Pathophysiology

Genetics

Behavioral variant frontotemporal dementia

Primary progressive aphasia

Cross-cultural issues

Treatment

Acknowledgment

Further reading

44 Progressive supranuclear palsy, corticobasal syndrome, and other tauopathies

Pathophysiology

Progressive supranuclear palsy syndrome

Corticobasal syndrome

Other tauopathies (FTLD-tau)

Future directions

Further reading

45 Amyotrophic lateral sclerosis and other TDP-43 proteinopathies

Amyotrophic lateral sclerosis

Frontotemporal dementias

Conclusion

Further reading

46 Metabolic, toxic, infectious, inflammatory, and other dementias

Normal pressure hydrocephalus

Toxic and metabolic disorders

Infectious causes

Genetic causes

Autoimmune disorders

Conclusion

Further reading

PART 7 Movement Disorders

47 Movement disorders: An overview

Diagnostic recommendations

Further reading

48 Tremor

Clinical features

Epidemiology

Pathophysiology

Investigations

Treatment

Further reading

49 Parkinsonism

Progressive supranuclear palsy

Corticobasal syndrome

Multiple system atrophy

Dementia with Lewy bodies

Frontotemporal dementia

Vascular parkinsonism

Normal pressure hydrocephalus

Parkinsonism dementia complex of Guam

Drug-induced parkinsonism

Toxin-induced parkinsonism

Infectious and postinfectious parkinsonism

Further reading

50 Parkinson’s disease

Neuropathology

Clinical presentation

Imaging

Differential diagnosis

Treatment

Conclusion

Further reading

51 Dystonia

Epidemiology

Pathobiology

Classification

Clinical features

Adult-onset focal dystonias

Oppenheim dystonia

Dopa-responsive dystonia (DRD)

Acquired and heredodegenerative dystonias

Treatment

Further reading

52 Chorea and related disorders

Classification

Hereditary causes

Parainfectious and autoimmune causes

Drug-related causes

Vascular causes

Metabolic causes

Infectious causes

Diagnosis

Treatment

Conclusion

Further reading

53 Myoclonus

Classification

Features of major myoclonic disorders

Evaluation

Treatment

Further reading

54 Tics and Tourette syndrome

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

55 Ataxia

Clinical features

Pathophysiology

Cerebellar ataxic disorders

Diagnosis

Management

Further reading

56 Drug-induced movement disorders

Acute syndromes

Subacute syndromes

Chronic or tardive syndromes

Further reading

57 Paroxysmal movement disorders

Historical aspects

Epidemiology

Pathophysiology

Clinical features

Investigations/diagnosis

Treatment

Conclusion

Further reading

PART 8 Infectious Diseases

58 Acute bacterial meningitis

Epidemiology

Etiology

Pathophysiology

Pathology

Clinical features

Investigations

Treatment

Prevention

Prognosis

Complications

Further reading

59 Brain abscess

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

60 Subdural empyema

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

61 Epidural abscess

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

62 Septic cerebral venous sinus thrombosis

Infection-related/septic cerebral venous sinus thrombosis

Anatomy and pathophysiology

Microbial etiology

Thrombophilia and septic CVT

Clinical presentation

Differential diagnosis

Radiological findings

Other investigations

Management

Outcome and prognosis

Further reading

63 Encephalitis due to bacterial infections

Mycoplasma pneumoniae

Listeria monocytogenes

Whipple’s disease

Cat-scratch disease

Brucellosis

Legionnaires’ disease

Recommendations

Further reading

64 Mycobacterium tuberculosis and avium

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Mycobacterium avium

Further reading

65 Leprosy

Bacteriology

Epidemiology

Pathogenesis

Immunity

Clinical features

Leprosy reactions

Diagnosis

Other investigations

Differential diagnosis

Antileprotic treatment

Treatment of leprosy reactions

Further reading

66 Neurosyphilis

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

67 Lyme disease

Epidemiology

Demographics

Tick vector

Organism

Clinical expression

Extraneural manifestations

Neurological Lyme disease

Diagnosis

Treatment

Prevention

Tick co-pathogens

Pathogenesis

Chronic Lyme disease/post-treatment Lyme disease syndrome

Further reading

68 Introduction to protozoans of the central nervous system

69 Amoebic disease of the central nervous system

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

70 Toxoplasmosis of the central nervous system

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

71 Cerebral malaria

Epidemiology

Pathology

Pathophysiology

Pathogenesis of coma

Cerebral malaria caused by P.

vivax

Clinical features

Post-malaria neurological syndromes (PMNS)

Poor prognostic factors

Diagnosis

Treatment

Further reading

72 Trypanosomiasis

American trypanosomiasis

Human African trypanosomiasis

Further reading

73 Cestodes

Neurocysticercosis

Echinococcosis

Further reading

74 Trematodes: Schistosomiasis

Epidemiology

Pathogenesis

Clinical features

Investigations

Treatment

Further reading

75 Nematodes

Trichinosis

Gnathostomiasis

Angiostrongyliasis

Further reading

76 Fungal infections of the central nervous system

Candidiasis

Aspergillosis

Cryptococcosis

Coccidioidomycosis

Mucormycosis (zygomycosis)

Histoplasmosis

Blastomycosis

Fungal infections associated with contaminated steroid injections

Further reading

77 Rickettsial disease

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

PART 9 Prion Diseases and Neurovirology

78 Prion diseases

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Preventive measures

Further reading

79 Acute, recurrent and chronic viral meningitis

Epidemiology

Pathophysiology

Clinical features

Differential diagnosis

Investigations

Treatment

Recovery and prognosis

Further reading

80 Acute viral encephalitis

Overview of acute encephalitis

Specific viral encephalitides: Rabies

Japanese encephalitis

West Nile encephalitis

Tick-borne encephalitis

St. Louis encephalitis

LaCrosse encephalitis

Alphaviruses

Other encephalitides

Further reading

81 Neurological complications of human herpesvirus infections

Herpes simplex virus (HSV-1) encephalitis

HSV-2 infection

Varicella zoster virus

Cytomegalovirus

Epstein–Barr virus

Human herpesvirus-6 (HHV-6) encephalitis

Acknowledgments

Further reading

82 Chronic viral diseases of the central nervous system

Epidemiology

Pathophysiology

Clinical features

Investigations

Prognosis and treatment

Other viruses that cause chronic progressive panencephalitis

Conclusion

Acknowledgments

Further reading

83 Progressive multifocal leukoencephalopathy

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Prevention (risk-mitigation strategies)

Further reading

84 Biology of HIV and overview of AIDS

History and overview

Viral molecular epidemiology

Viral structure

Pathogenesis

Course of infection and clinical features

Viral dynamics

Treatment and management

Conclusion

Further reading

85 HIV encephalitis and myelopathy

HIV-associated cognitive impairment

HIV-associated myelopathies

Further reading

86 Neuromuscular complications of HIV

Epidemiology

Pathophysiology

Clinical features

Myopathies

Neuropathies

Conclusion

Further reading

87 Opportunistic infections in HIV-positive subjects and AIDS patients

Opportunistic infections: Diffuse complications

Opportunistic infections: Focal complications

HIV-associated lymphoma

Further reading

88 Neurological complications of human T-cell lymphotropic virus type-1 infection

Epidemiology

Pathophysiology

Clinical features

Investigations

Differential diagnosis

Treatment

Further reading

PART 10 Demyelinating Disorders

89 Multiple sclerosis

Epidemiology

Pathophysiology

Clinical presentation

Investigations

Treatment

Further reading

90 Multiple sclerosis in children

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

91 Neuromyelitis optica spectrum disorders

Current status

Pathology and pathophysiology

Clinical, imaging, and laboratory features

Serological testing

Diagnosis

Treatment

Further reading

92 Acute disseminated encephalomyelitis

Clinical features

Laboratory findings in ADEM

MRI features of ADEM

Differential diagnosis

Treatment

Prognosis

Pathobiology

Further reading

93 Isolated inflammatory demyelinating syndromes

Features common to all isolated inflammatory demyelinating syndromes

The clinical syndromes

Further reading

94 Osmotic demyelination syndromes

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

95 Concentric sclerosis (Baló’s disease)

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

PART 11 Specific toxicities and deficiencies

96 Neurotoxicology

Heavy metal intoxication

Organic solvents or compounds

Gases

Pesticides

Defoliant toxins

Animal toxins

Plant toxins

Bacterial toxins

Illicit drugs

Specific drugs

Hypervitaminosis

Regional-specific toxins

Conclusions

Further reading

97 Alcohol-related neurological disorders

Pharmacological aspects

Acute alcohol intoxication

Alcohol withdrawal

Alcohol-related dementia

Alcoholic cerebellar degeneration

Marchiafava–Bignami disease

Alcoholic amblyopia

Alcoholic myopathy

Alcoholic polyneuropathy

Fetal alcohol syndrome

Further reading

98 Vitamin deficiencies

Group B vitamins

Vitamin B deficiency syndromes

Other vitamins

Other vitamin deficiencies

Conclusion

Further reading

99 Starvation, Strachan’s syndrome, and postgastroplasty syndrome

Starvation

Strachan’s syndrome

Postgastroplasty syndrome

Further reading

PART 12 Peripheral Neuropathies

100 Peripheral neuropathies overview

Epidemiology

Pathophysiology and clinical features

Treatment

Further reading

101 Hereditary neuropathies

Hereditary motor and sensory neuropathy

Hereditary sensory and autonomic neuropathy

Distal hereditary motor neuropathy

Other hereditary neuropathies

Treatment

Further reading

102 Acquired neuropathies

Immune-mediated neuropathies

Multifocal motor neuropathy

Metabolic neuropathies

Infectious neuropathies

Neuropathy from nutritional causes and alcoholism

Further reading

103 Plexopathies and mononeuropathies

Plexopathies

Mononeuropathies (entrapment neuropathies)

Further reading

PART 13 Neuromuscular Disorders

104 Amyotrophic lateral sclerosis

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

105 Progressive muscular atrophy

Epidemiology

Pathophysiology

Clinical features

Differential diagnosis

Treatment

Further reading

106 Primary lateral sclerosis

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

107 Hereditary spastic paraplegia

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

108 Spinal muscular atrophy

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Future prospects

Further reading

109 Post polio syndrome

Epidemiology

Disease mechanisms

Clinical features

Investigations

Treatment

Further reading

110 Limb-girdle muscular dystrophies

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

111 Dystrophinopathies

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

112 Facioscapulohumeral muscular dystrophy

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

113 Myotonic dystrophies

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

114 Oculopharyngeal muscular dystrophy

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

115 Emery–Dreifuss syndrome and laminopathies

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Conclusion

Further reading

116 Muscle channelopathies

Non-dystropic myotonias

Periodic paralyses

Conclusion

Further reading

117 Congenital dystrophies and myopathies

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

118 Congenital myasthenic syndromes

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

119 Distal myopathies

Epidemiology

Pathophysiology

Clinical, pathological, and genetic features

Investigations

Treatment

Conclusions

Further reading

120 Polymyositis

Clinical manifestations

Association conditions

Immunopathogenesis

Differential diagnosis

Diagnosis

Therapy

Prognosis

Further reading

121 Dermatomyositis

Clinical manifestations

Imunopathogenesis

Diagnosis

Treatment

Prognosis and complications

Further reading

122 Inclusion body myositis

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment and management

Conclusions

Further reading

123 Immune-mediated necrotizing myopathies

Epidemiology

Pathophysiology

Clinical features

Treatment and management

Further reading

124 Toxic myopathies

Alcohol-associated myopathy

Statin myopathy

Corticosteroid myopathy

Critical illness myopathy and malignant hyperthermia

Further reading

125 Critical illness neuromuscular disorders

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment and management

Further reading

126 Exercise intolerance and myoglobinuria

Testing of exercise intolerance

Myoglobinuria

Further reading

127 Muscle cramps

Pathophysiology

Clinical features

Investigations

Treatment

Acknowledgments

Further reading

128 Myasthenia gravis

Epidemiology

Pathophysiology

Clinical manifestations

Investigations

Diagnosis

Treatment

Further reading

129 Lambert–Eaton myasthenic syndrome

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

130 Neuromuscular transmission disorders caused by toxins and drugs

Botulism

Organophosphate intoxication

Medication-induced neuromuscular junction disorders

Conclusion

Further reading

PART 14 Neurogenetics

131 Genetics in neurology

Basic definitions

Mendelian genetics

Other genetic forms of inheritance

Neurocutaneous disorders

Other genetic disorders

Treatment for genetic disorders

Further reading

PART 15 Neuro-otology

132 Neuro-otology

Epidemiology

International considerations in patients with neuro-otological disorders

Pathophysiology

Clinical approach to patients with dizziness

Common presentations of dizziness

Management of the patient with dizziness

Investigations

Common presentations of hearing loss

Common presentations of tinnitus

Conclusion

Further reading

PART 16 Neuro-ophthalmology

133 Neuro-ophthalmology

Clinical approach to visual loss

Clinical approach to double vision

Clinical approach to pupillary abnormalities

Further reading

PART 17 Neuro-oncology

134 Neuro-oncology overview

135 High-grade astrocytomas

Epidemiology

Genetics

Clinical features

Investigations

Prognostic factors

Treatment

Conclusion

Further reading

136 Low-grade astrocytomas

Epidemiology and clinical features

Pathophysiology and molecular biology

Investigations

Prognosis

Treatment and management of astrocytomas

Further reading

137 Low-grade and anaplastic oligodendrogliomas

Epidemiology

Pathophysiology

Clinical features

Radiographic features

Treatment

Conclusion

Further reading

138 Brainstem glioma

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Adults

Emerging therapies

Further reading

139 Intracranial ependymoma

Treatment

Conclusion

Further reading

140 Nerve sheath tumors

Epidemiology and pathophysiology

Clinical features and investigations

Treatment

Further reading

141Meningiomas

Epidemiology

Pathophysiology

Clinical presentation

Investigations

Treatment

Outcomes

Further reading

142 Adult medulloblastoma

Epidemiology

Clinical features

Investigations

Treatment

Further reading

143 Primary central nervous system lymphoma

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

144 Brain metastases

Epidemiology

Pathophysiology

Clinical features

Prognosis

Investigations

Treatment

Management in common solid tumors

Conclusion

Further reading

145 Leptomeningeal metastases

Pathophysiology

Clinical features

Investigations

Prognosis

Treatment and management

Conclusion

Further reading

146 Spinal epidural metastases

Epidemiology

Pathophysiology

Clinical features

Investigations

Differential diagnosis

Treatment

Prognosis

Further reading

147 General approach to the diagnosis and treatment of paraneoplastic neurological disorders

Diagnosis

Treatment

Specific syndromes

Further reading

PART 18 Sleep Disorders

148 Insomnia

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Conclusion

Further reading

149 Narcolepsy

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

150 Idiopathic hypersomnia

Epidemiology

Pathophysiology

Clinical features

Differential diagnosis

Diagnostic assessment

Treatment

Further reading

151 Obstructive sleep apnea

Epidemiology

Pathophysiology

Risk factors

Clinical features

Investigations

Treatment

Further reading

152 Restless legs syndrome and periodic limb movement disorders

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

153 Circadian rhythm sleep–wake disorders

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Conclusions

Acknowledgments

Further reading

154 Arousal disorders

Epidemiology and risk factors

Pathophysiology

Clinical features and differential diagnoses

Investigations

Treatment

Clinical course

Conclusion

Further reading

155 REM sleep behavior disorder

Epidemiology and risk factors

Pathophysiology

Clinical features and differential diagnoses

Investigations

Treatment

Clinical course

Conclusion

Further reading

156 Paroxysmal nocturnal dystonia

Clinical features

Genetics

Differential diagnosis

Investigations

Treatment

Further reading

157 Sleep abnormalities in neurological disorders

Sleep-disordered breathing

Restless legs syndrome and periodic limb movement disorder

Parasomnias

Pathological hypersomnias

Further reading

PART 19 Spinal Cord Disorders

158 Spinal cord disorders

Cervical disc disease

Lumbar disc disease

Natural course of disc herniation

Diagnosis

Treatment

Cervical spondylosis

Lumbar spinal stenosis

Paget’s disease

Fibrous dysplasia

Other compressive disorders

Investigation

Further reading

159 Ischemic and congestive myelopathies

Acute ischemic myelopathy

Congestive myelopathy

Further reading

160 Syringomyelia

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

PART 20 Pediatric Neurology

161 Neonatal neurology

Hypoxic ischemic encephalopathy

Intraventricular hemorrhage

Infection

Inborn errors of metabolism

Seizures

Neurotoxin exposure

Further reading

162 Neurodevelopmental disorders

Developmental delay and intellectual disability

Selected genetic neurodevelopmental disorders

Conclusion

Further reading

163 Floppy infant syndrome

Clinical features and evaluation

Laboratory investigations

Approach for genetic diagnosis

Special considerations for cost-effective genetic analysis

Therapeutic approach

Conclusion

Further reading

164 Storage disorders

Lipidoses

Mucopolysaccharidosis

Glycogenoses

Leukodystrophies

Other lipidoses

Further reading

165 Fatty acid oxidation disorders

Physiology

Epidemiology and genetics

Clinical features and pathophysiology

Investigations

Treatment and management

Prenatal diagnosis

Conclusion

Further reading

166 Disorders of amino acid, organic acid, and ammonia metabolism

Neonatal disorders

Progressive diseases of infancy and childhood

Further reading

167 Pediatric neurotransmitter diseases

Pathophysiology

Monoamine neurotransmitter disorders

GABA neurotransmitter disorders

Conclusion

Further reading

168 Mitochondrial encephalomyopathies

Mitochondrial genetics

Genetic and functional classification

Epidemiology

Neurological manifestations

Diagnosis

Therapy

Conclusion

Further reading

169 Disorders resulting from transporter defects

Disorders of copper transporters

Manganese transporter deficiency

Further reading

170 The porphyrias

Pathogenesis of neurological dysfunction

Epidemiology

Clinical features

Differential diagnosis

Porphyric attacks caused by antiepileptic medications

Treatment

Conclusion

Acknowledgments

Further reading

PART 21 Central Nervous System Trauma, Pain, and Headache

171 Traumatic brain injury

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

172 Spinal injury

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Further reading

173 Whiplash injury

Epidemiology

Pathophysiology

Clinical features

Investigations

Treatment

Conclusion

Further reading

174 Decompression sickness

Epidemiology

Pathophysiology

Clinical features

Treatment

Further reading

175 Pain

Classification of pain

Pathophysiology of pain

Visceral pain

Neuralgias and neuropathic pain syndromes

Entrapment neuropathies

Complex regional pain syndromes

Central pain syndromes

Pain due to systemic disorders

Approach to the patient with pain

Treatment

Further reading

176 Headache

Migraine headache

Tension-type headache

Cluster headache

Idiopathic intracranial hypertension

Low-pressure headache

Other headache syndromes

Further reading

177 Facial and neck pain

Facial pain

Neck pain and headache

Further reading

178 Chronic fatigue syndrome

Epidemiology

Etiology

Pathophysiology

Diagnosis

Treatment

Further reading

179 Fibromyalgia

Epidemiology

Pathogenesis

Diagnosis

Clinical features

Differential diagnosis

Management

Further reading

Index

EULA

List of Tables

Chapter 2

Table 2.1

Table 2.2

Chapter 4

Table 4.1

Table 4.2

Chapter 6

Table 6.1

Table 6.2

Chapter 13

Table 13.1

Chapter 14

Table 14.1

Chapter 15

Table 15.1

Chapter 16

Table 16.1

Chapter 18

Table 18.1

Chapter 19

Table 19.1

Chapter 20

Table 20.1

Chapter 25

Table 25.1

Table 25.2

Table 25.3

Chapter 26

Table 26.1

Chapter 28

Table 28.1

Table 28.2

Chapter 30

Table 30.1

Chapter 31

Table 31.1

Table 31.2

Table 31.3

Table 31.4

Chapter 32

Table 32.1

Chapter 33

Table 33.1

Chapter 36

Table 36.1

Table 36.2

Table 36.3

Chapter 40

Table 40.1

Table 40.2

Table 40.3

Chapter 42

Table 42.1

Chapter 43

Table 43.1

Table 43.2

Chapter 47

Table 47.1

Table 47.2

Chapter 48

Table 48.1

Table 48.2

Table 48.3

Table 48.4

Chapter 49

Table 49.1

Table 49.2

Table 49.3

Table 49.4

Table 49.5

Chapter 50

Table 50.1

Table 50.2

Chapter 51

Table 51.1

Table 51.2

Table 51.3

Chapter 52

Table 52.1

Table 52.2

Table 52.3

Chapter 53

Table 53.1

Chapter 54

Table 54.1

Chapter 55

Table 55.1

Chapter 57

Table 57.1

Chapter 58

Table 58.1

Table 58.2

Chapter 59

Table 59.1

Table 59.2

Chapter 60

Table 60.1

Chapter 61

Table 61.1

Chapter 65

Table 65.1

Table 65.2

Chapter 66

Table 66.1

Chapter 67

Table 67.1

Chapter 69

Table 69.1

Table 69.2

Chapter 71

Table 71.1

Table 71.2

Chapter 73

Table 73.1

Table 73.2

Chapter 74

Table 74.1

Chapter 75

Table 75.1

Chapter 77

Table 77.1

Table 77.2

Chapter 78

Table 78.1

Chapter 79

Table 79.1

Table 79.2

Table 79.3

Table 79.4

Chapter 80

Table 80.1

Table 80.2

Table 80.3

Table 80.4

Table 80.5

Chapter 82

Table 82.1

Chapter 83

Table 83.1

Chapter 84

Table 84.1

Chapter 86

Table 86.1

Chapter 88

Table 88.1

Chapter 89

Table 89.1

Table 89.2

Table 89.3

Chapter 90

Table 90.1

Chapter 91

Table 91.1

Chapter 92

Table 92.1

Table 92.2

Table 92.3

Chapter 98

Table 98.1

Chapter 99

Table 99.1

Chapter 100

Table 100.1

Chapter 101

Table 101.1

Table 101.2

Chapter 102

Table 102.1

Chapter 104

Table 104.1

Chapter 107

Table 107.1

Chapter 108

Table 108.1

Chapter 110

Table 110.1

Chapter 111

Table 111.1

Chapter 115

Table 115.1

Table 115.2

Chapter 117

Table 117.1

Table 117.2

Chapter 118

Table 118.1

Table 118.2

Table 118.3

Chapter 119

Table 119.1

Chapter 124

Table 124.1

Chapter 126

Table 126.1

Chapter 127

Table 127.1

Table 127.2

Chapter 131

Table 131.1

Chapter 132

Table 132.1

Chapter 133

Table 133.1

Chapter 135

Table 135.1

Chapter 137

Table 137.1

Chapter 139

Table 139.1

Chapter 142

Table 142.1

Chapter 143

Table 143.1

Chapter 145

Table 145.1

Chapter 147

Table 147.1

Chapter 148

Table 148.1

Chapter 149

Table 149.1

Table 149.2

Chapter 150

Table 150.1

Chapter 152

Table 152.1

Chapter 161

Table 161.1

Chapter 162

Table 162.1

Table 162.2

Chapter 163

Table 163.1

Chapter 164

Table 164.1

Chapter 166

Table 166.1

Chapter 168

Table 168.1

Table 168.2

Table 168.3

Table 168.4

Table 168.5

Chapter 169

Table 169.1

Chapter 170

Table 170.1

Chapter 171

Table 171.1

Chapter 173

Table 173.1

Chapter 175

Table 175.1

Table 175.2

Table 175.3

Chapter 178

Table 178.1

Chapter 179

Table 179.1

Table 179.2