Peripheral Nerve Disorders
- 1st Edition, Volume 115 - September 11, 2013
- Editors: Gérard Said, Christian Krarup
- Language: English
- Hardback ISBN:9 7 8 - 0 - 4 4 4 - 5 2 9 0 2 - 2
- eBook ISBN:9 7 8 - 0 - 4 4 4 - 6 3 3 5 5 - 2
Disorders of the peripheral nervous system (PNS) are the cause of prominent neurological symptoms including weakness, sensory loss, pain and autonomic dysfunction associated wi… Read more
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.
- 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
Clinical neurologists and research neuroscientists
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
- Edition: 1
- Volume: 115
- Published: September 11, 2013
- Language: English
GS
Gérard Said
Affiliations and expertise
Médecin Consultant, Department of Neurology, Hôpital de la Salpétrière, Paris, FranceCK
Christian Krarup
Affiliations and expertise
Department of Clinical Neurophysiology, Rigshospitalet, Copenhagen, DenmarkRead Peripheral Nerve Disorders on ScienceDirect