Peripheral Nerve Disorders

Peripheral Nerve Disorders

1st Edition - August 17, 2013

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  • Editors: Gérard Said, Christian Krarup
  • Hardcover ISBN: 9780444529022
  • eBook ISBN: 9780444633552

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Description

Disorders of the peripheral nervous system (PNS) are the cause of prominent neurological symptoms including weakness, sensory loss, pain and autonomic dysfunction associated with deficits, morbidity and mortality. These disorders may be primary hereditary or cryptogenic neurologic disorders confined to the PNS or part of the pathology of both the central nervous system and the PNS. Most PNS disorders are secondary to other system disorders and may be responsive to treatment of the primary disease. Important advances have been obtained in several areas including molecular genetics, biochemistry, immunology, morphology and physiology that have enhanced our understanding of the causes and consequences of damage to peripheral nerve. Understanding of both these groups of PNS diseases has greatly expanded over recent years and has led to important advances of treatment both to protect and to repair damages of peripheral nerve. This volume provides an overview of the state-of-the-art of examination, diagnosis and treatment of these very diverse disorders and will be of interest to both the research and clinical neuroscience and neurology communities.

Key Features

  • Covers both hereditary and cryptogenic neurologic disorders
  • Includes advances in the basic science of PNS from molecular genetics, biochemistry, immunology, morphology and physiology
  • Detailed coverage of neuropathy in connective tissue disorders, infectious disorders, metabolic disorders and malignancy

Readership

Clinical neurologists and research neuroscientists

Table of Contents

  • Handbook of Clinical Neurology 3rd Series

    Foreword

    Preface

    Contributors

    Section 1: Introduction

    Chapter 1. Prelude to the peripheral neuropathies

    Section 2: Structure and function

    Chapter 2. Microscopic anatomy: normal structure

    The nerve trunk

    Development

    Aging

    Conclusions

    References

    Chapter 3. Gross anatomy and development of the peripheral nervous system

    Introduction

    Gross anatomy

    Development of the PNS

    Conclusion

    References

    Chapter 4. Physiology and pathophysiology of myelinated nerve fibers

    Introduction

    Axonal structure and function

    Fluctuations in excitability with impulse transmission

    Assessment of nerve excitability in a clinical setting

    Mechanisms of conduction block

    Impulse conduction in demyelinating neuropathies

    Multifocal motor neuropathy, axonal hyperpolarization, and conduction block

    Conclusions

    References

    Chapter 5. Biology of Schwann cells

    Introduction

    Schwann cell development and differentiation

    Biology of Schwann cell myelination

    Nonmyelinating (remak) Schwann cells

    Schwann cells in wallerian degeneration and regeneration

    Conclusions and perspectives

    References

    Chapter 6. Neurophysiological approach to disorders of peripheral nerve

    Introduction

    Pathological changes in peripheral nerve disorders

    Electromyography (EMG)

    Nerve conduction studies

    Special studies

    Relation between clinical manifestations and neurophysiological findings

    Differential diagnosis of the patient with peripheral nerve disorders

    Mononeuropathies/multiple mononeuropathies

    Polyneuropathies with axonal loss and an acute/subacute onset (subtype 3A)

    Polyneuropathies with axonal loss and slow onset (subtype 3B)

    Demyelinating polyneuropathy with acute/subacute onset (subtype 3C)

    Demyelinating polyneuropathy with slow onset (subtype 3D)

    References

    Chapter 7. Testing the autonomic nervous system

    Introduction

    History

    Background physiology

    Molecular determinants of autonomic control

    Clinical tests of autonomic function

    Tests of sympathetic adrenergic function

    Assessment of sympathetic cholinergic sudomotor function

    Research tests

    References

    Chapter 8. Imaging of the peripheral nervous system

    Introduction

    Magnetic resonance imaging

    Acknowledgment

    References

    Chapter 9. The nerve biopsy: indications, technical aspects, and contribution

    Introduction: indications

    Surgical procedure

    Laboratory techniques

    Interpretation

    Diagnostic findings

    Conclusions

    References

    Chapter 10. The cutaneous nerve biopsy: technical aspects, indications, and contribution

    Introduction

    Technical aspects of skin biopsy for studying cutaneous nerve fibers

    Indications for skin biopsy to evaluate cutaneous nerve fibers

    Contribution of cutaneous nerve biopsy in peripheral nerve disease

    Conclusions

    References

    Chapter 11. Antibody testing in peripheral nerve disorders

    Part 1

    Part 2

    Part 3

    References

    Chapter 12. DNA testing in hereditary neuropathies

    Introduction

    Phenotypic Guides to Genetic Testing

    Detailed Guide to Genetic Testing

    Methods of Genetic Testing

    Advances in Genetic Testing

    Genetic Counseling

    Conclusions and Future Developments

    Acknowledgments

    References

    Further Reading

    Section 3: Clinical aspects

    Chapter 13. Examination and clinical care of the patient with neuropathy

    Introduction

    Motor abnormalities

    Autonomic dysfunction in peripheral neuropathy

    Synthesis of clinical manifestations

    Electrodiagnostic examination

    Imaging of the peripheral nervous system

    Cerebrospinal fluid examination

    DNA testing in hereditary neuropathies

    Indication of biopsy of sensory nerves

    Conclusion

    References

    Chapter 14. How to explore a patient with a chronic axonal polyneuropathy

    Introduction

    Epidemiology

    Diagnosis

    Role of laboratory testing

    Role of genetic testing

    Role of clinical autonomic testing

    Role of nerve biopsy

    Role of skin biopsy

    Etiologies of chronic polyneuropathies

    Conclusion

    References

    Chapter 15. Evaluation of a patient with suspected chronic demyelinating polyneuropathy

    Introduction

    Clinical evaluation findings suggesting chronic demyelinating neuropathy

    Laboratory investigations

    Neuroimaging

    Role of pathological assessment

    Summary

    References

    Chapter 16. Sensory-motor assessment in clinical research trials

    Introduction

    The clinimetric aspects of sensory-motor evaluation

    Measures of motor function in clinical research

    Measures of sensory function in clinical research

    Conclusions

    Acknowledgments

    References

    Chapter 17. Management of painful neuropathies

    Introduction

    Classification of peripheral neuropathies

    Animal models in painful neuropathies

    Prevention of painful neuropathies

    Treatment of underlying neuropathy

    Symptomatic pharmacological treatment of neuropathic pain

    Nonpharmacological treatment of neuropathic pain

    Disease-specific treatment recommendations

    Treatment algorithm

    General treatment principles

    Advances in the treatment of painful neuropathies

    References

    Section 4: Plexus and compression lesions

    Chapter 18. Diagnosis of brachial and lumbosacral plexus lesions

    Clinical introduction

    Brachial plexus lesions

    Specific disorders of the brachial plexus

    Lumbosacral plexus lesions

    Specific disorders of the lumbosacral plexus

    Symptomatic treatment and prognosis of plexopathies

    References

    Chapter 19. Compression and entrapment neuropathies

    Introduction

    Upper limb

    Lower limb

    Acknowledgment

    References

    Chapter 20. Facial nerve palsy and hemifacial spasm

    Introduction

    Functionally relevant anatomical particularities of the facial nerve

    peripheral facial palsy

    Hemifacial spasm

    Clinical and electrophysiological differences between postparalytic facial syndrome and essential hemifacial spasm

    Treatment options

    References

    Section 5: Inflammatory root and nerve lesions

    Chapter 21. The Guillain–Barré syndrome

    Introduction

    Epidemiology

    Clinical features and antecedent infections

    Diagnosis

    Pathophysiology

    Experimental model

    Treatment

    Acknowledgments

    References

    Chapter 22. Chronic inflammatory demyelinative polyneuropathy

    Introduction

    Epidemiology

    Clinical manifestations of CIDP

    Precipitating factors

    Age of patients

    Neurological manifestations at onset

    Neurological manifestations at referral or at steady state

    Clinical variants of CIDP

    Clinical course and prognosis

    Electrophysiological data

    Neuroimaging

    Morphological findings

    Immunological factors

    Differential diagnosis

    Treatment

    References

    Chapter 23. Treatment of chronic inflammatory demyelinating polyradiculoneuropathy

    Introduction

    Corticosteroids

    Plasma Exchange

    Immunoadsorption

    Intravenous immunoglobulin (IVIg)

    Azathioprine

    Cyclophosphamide

    Methotrexate

    Interferon-Beta

    Ciclosporin

    Alemtuzumab

    Rituximab

    Stem cell transplantation

    Practice of clinical treatment in CIDP

    Summary

    Acknowledgment

    References

    Chapter 24. Multifocal motor neuropathy

    Introduction

    History

    Clinical features

    Course and prognosis

    Differential diagnoses

    Diagnostic criteria

    Treatment

    Immunopathogenesis of multifocal motor neuropathy

    Axonal dysfunction in MMN

    Conclusion

    References

    Chapter 25. Neuropathy and monoclonal gammopathy

    Introduction

    Monoclonal gammopathy

    Neuropathy and monoclonal gammopathy

    Neuropathy and IgM monoclonal gammopathy

    Neuropathy and IgG monoclonal gammopathy

    Neuropathy and IgA monoclonal gammopathy

    Poems syndrome

    References

    Section 6: Neuropathy in connective tissue disorders

    Chapter 26. Vasculitic neuropathy

    Introduction

    Classification and epidemiology

    Clinical aspects

    Treatment

    References

    Chapter 27. Sarcoidosis of the peripheral nervous system

    Introduction

    History

    Epidemiology

    Genetics

    Neurobiological basis

    Symptoms and signs of sarcoid neuropathy

    Sarcoidosis: a multisystem disease

    Central nervous system and meningeal involvement in sarcoidosis

    Other organs most often involved in sarcoidosis

    Course

    Electrophysiology

    Pathology

    Diagnosis

    Treatment

    Conclusion

    References

    Section 7: Neuropathy in infectious disorders

    Chapter 28. Leprous neuropathy

    Introduction

    Epidemiology

    Mycobacterium leprae

    Pathogenesis

    Genetic studies

    Transmission

    Classification

    Sensory motor manifestations

    Clinical forms

    Immunological status

    Nerve enlargement

    Reactions

    Relapse

    Leprosy and HIV

    Diagnosis

    Differential diagnosis

    Treatment

    Rehabilitation

    Prevention

    Conclusion

    References

    Chapter 29. HIV peripheral neuropathy

    Introduction

    Epidemiology of the peripheral neuropathies

    Clinical presentations

    AIDP and CIDP

    Mononeuropathies, mononeuropathies multiplex, and cranial neuropathies

    Herpes zoster

    Autonomic neuropathy

    Lumbosacral polyradiculomyelopathy

    Amyotrophic lateral sclerosis-like motor neuropathy

    References

    Chapter 30. Human T-cell leukemia virus (HTLV)-associated neuropathy

    Introduction

    Epidemiology

    HTLV-1 Virus infection and the immune response

    Clinical features

    HTLV-1 Infection diagnosis

    Neurophysiological findings in HTLV-1

    Pathology

    HTLV Neuropathy and coinfections

    Differential diagnosis

    Treatment and prevention

    Conclusion

    References

    Chapter 31. Herpes virus infection of the peripheral nervous system

    Introduction

    The three neurotropic herpes viruses

    Peripheral nervous system disorders due to human cytomegalovirus

    Treatment

    Disclosure

    References

    Chapter 32. Lyme neuroborreliosis

    List of abbreviations

    Introduction

    Epidemiology and etiology

    Clinical features of lyme neuroborreliosis

    Pathogenesis and pathology of lyme neuroborreliosis

    Diagnostic laboratory findings in lyme neuroborreliosis

    Electrophysiological findings in lyme neuroborreliosis

    Diagnostic neuroimaging in lyme neuroborreliosis

    Differential diagnoses of lyme neuroborreliosis

    Therapy and prevention

    Post-Lyme disease syndrome and “chronic lyme disease”

    References

    Section 8: Neuropathy and metabolic disorders

    Chapter 33. Diabetic neuropathy

    Introduction

    Epidemiology

    Clinical aspects of diabetic neuropathy

    Nerve conduction studies

    The pathology of diabetic neuropathies

    Additional causes of neuropathy in diabetic patients

    Pathophysiology of diabetic neuropathy

    Treatment of diabetic neuropathy

    Conclusion

    References

    Chapter 34. Biology of diabetic neuropathy

    Introduction

    Linking clinical manifestations to cellular mechanisms

    Neuronal injury mechanisms downstream of hyperglycemia pathways and loss of insulin signaling

    Other significant factors leading to neuropathic mechanisms in diabetes

    Conclusion

    References

    Chapter 35. Uremic neuropathy

    Introduction

    Uremic polyneuropathy

    Role for hyperparathyroidism?

    Serum k+ between periods of dialysis

    Conclusion

    References

    Chapter 36. Porphyric neuropathy

    Abbreviations

    Introduction

    Historical perspectives

    Heme biosynthesis pathway

    Types of acute porphyria

    Clinical features of porphyria

    Laboratory investigations

    Treatment and management

    Pathophysiological mechanisms of neurotoxicity

    Experimental models of porphyric neuropathy

    Biophysical changes in the axon in porphyric neuropathy

    Future directions

    Conclusions

    References

    Chapter 37. Fabry disease

    Introduction

    Historical aspects

    Epidemiology

    Clinical manifestations

    Peripheral neuropathy and pathophysiology

    Genetics

    Diagnosis

    Treatment

    Basic research

    Conclusions

    References

    Chapter 38. Transthyretin familial amyloid polyneuropathy

    Introduction

    Historical aspects

    Genetics

    Neurobiological basis: pathophysiology of amyloid formation

    Clinical features

    Course

    Electrophysiology

    Pathology

    Diagnosis

    Management of patients

    Conclusion

    References

    Chapter 39. Hereditary gelsolin amyloidosis

    Introduction

    Epidemiology

    Genealogy

    Clinical findings

    Results of special investigations in hereditary gelsolin amyloidosis

    Morphological and immunohistochemical findings

    Biochemistry and molecular genetics

    Pathogenesis

    Diagnosis of hereditary gelsolin amyloidosis

    Treatment and follow-up of hereditary gelsolin amyloidosis

    Conclusion

    Note added in proof

    References

    Section 9: Neuropathy and malignancy

    Chapter 40. Malignant cell infiltration in the peripheral nervous system

    Introduction

    Cranial nerve neuropathies

    Nerve roots

    Nerve plexus

    Mononeuropathies

    Paraneoplastic polyneuropathies

    Conclusion

    Acknowledgment

    References

    Chapter 41. Paraneoplastic neuropathy

    Introduction

    Types of neuropathy

    Antibodies associated with paraneoplastic neuropathy

    Pathogenesis

    Diagnosis

    Treatment

    Conclusions

    References

    Section 10: Cryptogenic, traumatic and iatrogenic neuropathies

    Chapter 42. Drug-induced neuropathies

    Abbreviations

    Introduction

    Pathophysiology

    Tumor necrosis factor-α antagonists

    Chemotherapeutic agents

    Bortezomib

    Antiretroviral drugs

    Antibiotics

    Statins

    Amiodarone

    Conclusion

    References

    Chapter 43. Late radiation injury to peripheral nerves

    Introduction

    Pathophysiology

    Neurotoxicity according to the affected anatomical site

    Treatment

    Radiation-induced peripheral nerve tumors

    Conclusion

    Acknowledgments

    References

    Chapter 44. Neuromuscular complications of critical illness

    Introduction

    Historical perspective

    Critical illness, sepsis, multiple organ failure, and systemic inflammatory response syndrome

    Acquired weakness in the ICU

    Diagnostic criteria for acquired weakness in the ICU

    Diagnostic criteria for critical illness polyneuropathy

    Other acquired neuropathic conditions in ICU patients

    Critical illness myopathy

    Diagnostic criteria for critical illness myopathy

    Specific varieties of CIM

    Clinical approach to critical illness weakness

    Risk factors for developing acquired weakness in the ICU

    Pathophysiology of critical illness polyneuropathy and critical illness myopathy

    Respiratory dysfunction in acquired weakness in the ICU

    Persistent weakness and long-term outcome of ICUAW

    References

    Chapter 45. The surgery of peripheral nerves (including tumors)

    Introduction

    The history of peripheral nerve surgery

    Peripheral nerve degeneration and regeneration

    Traumatic peripheral nerve injuries and their management

    Peripheral nerve tumors

    Functional peripheral nerve surgery

    Future developments

    References

    Futher reading

    Chapter 46. Peripheral neuropathy in the elderly

    Introduction

    General approach to the patient with symptomatic neuropathy

    Acquired demyelinating neuropathies

    Poems

    Conclusions

    Acknowledgments

    References

    Section 11: Familial neuropathies

    Chapter 47. Dominant Charcot–Marie–Tooth syndrome and cognate disorders

    Abbreviations

    Genes

    Introduction

    Historical notes, eponyms, and acronyms

    Charcot–marie–tooth neuropathy

    Recurrent focal neuropathies

    Management

    Concluding remarks and future perspectives

    Acknowledgments

    References

    Chapter 48. Recessively transmitted predominantly motor neuropathies

    Introduction

    CMT4: Recessive/demyelinating

    CMT2: Recessive/axonal

    Recessive CMTX

    Recessive dHMN

    Conclusion

    Acknowledgment

    References

    Chapter 49. Early onset (childhood) monogenic neuropathies

    Abbreviations

    Introduction

    “Neurological” and “genetic” classifications of hereditary neuropathies in childhood

    Neuropathies secondary to a hereditary general disease

    Primary neuropathies: hereditary motor sensory neuropathies, hereditary sensory-autonomous neuropathies, and hereditary motor neuropathies

    Spinal muscular atrophy

    Syndromic neuropathies; congenital neuropathies

    References

    Chapter 50. Hereditary sensory and autonomic neuropathies

    Introduction

    Historical remarks

    Current classification of HSN/HSAN

    Management and treatment

    Conclusions

    References

    Chapter 51. Peripheral nerve involvement in hereditary cerebellar and multisystem degenerative disorders

    Historical perspective

    Ackowledgments

    References

    Chapter 52. Giant axonal neuropathy

    Introduction

    Clinical features

    Electromyography

    Muscle and nerve biopsy

    Cerebral MRI

    Neuropathology

    Molecular aspects

    Treatment

    Concluding remarks

    Acknowledgments

    References

    Chapter 53. Neurofibromatosis type 1 (NF1): diagnosis and management

    Introduction

    History of neurofibromatosis 1

    Diagnostic criteria for neurofibromatosis 1

    Epidemiology

    Differential diagnosis

    Clinical manifestations of neurofibromatosis 1

    The eye

    Gastrointestinal complications

    The skin

    Bone

    Cardiovascular problems

    Genetic counseling

    Future directions

    References

    Chapter 54. Neurofibromatosis type 2 (NF2): diagnosis and management

    Introduction

    History

    Epidemiology

    Genetics

    Molecular biology

    Clinical features

    Vestibular schwannomas

    Intracranial meningiomas

    Spinal tumors

    Neuropathy

    Ocular disease

    Cutaneous disease

    Determinants of disease phenotype

    Differential diagnosis

    Management

    Screening for NF2

    Survival rates

    Conclusion

    References

    Index

Product details

  • No. of pages: 1008
  • Language: English
  • Copyright: © Elsevier 2013
  • Published: August 17, 2013
  • Imprint: Elsevier
  • Hardcover ISBN: 9780444529022
  • eBook ISBN: 9780444633552

About the Editors

Gérard Said

Affiliations and Expertise

Médecin Consultant, Department of Neurology, Hôpital de la Salpétrière, Paris, France

Christian Krarup

Affiliations and Expertise

Department of Clinical Neurophysiology, Rigshospitalet, Copenhagen, Denmark

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