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.
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Table of Contents
Section 1 Introduction 1. Prelude to the peripheral neuropathies Section 2 Structure and function 2. Microscopic anatomy: normal structures; 3. Gross anatomy and development of the peripheral nervous system; 4. Physiology and pathophysiology of myelinated nerve fibers; 5. Biology of Schwann cells; 6. Neurophysiological approach to disorders of peripheral nerve; 7. Testing autonomic functions; 8. Imaging of the peripheral nervous system; 9. The nerve biopsy: indications, technical aspects and contribution; 10. The cutaneous nerve biopsy: Technical aspects, indications, and contribution; 11. Antibody testing in peripheral nerve disorders; 12. DNA testing in hereditary neuropathies Section 3 Clinical aspects 13. Examination and clinical care of the patient with neuropathy; 14. How to explore a patient with chronic axonal neuropathy; 15. Evaluation of a patient with suspected chronic demyelinating polyneuropathy; 16. Sensory-motor assessment in clinical research trials; 17. Management of painful neuropathies Section 4 Plexus and compression lesions 18. Diagnosis of brachial and lumbosacral plexus lesions; 19. Compression and entrapment neuropathies; 20. Facial nerve palsy and hemifacial spasm Section 5 Inflammatory root and nerve lesions 21. The Guillain-Barré syndrome; 22. Chronic inflammatory demyelinating polyneuropathy; 23. Treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP); 24. Multifocal motor neuropathy 25. Neuropathy and monoclonal gammopathy Section 6 Neuropathy in connective tissue disorders 26. Vasculitic neuropathy; 27. Sarcoidosis of the peripheral nervous system Section 7 Neuropathy in infectious disorders 28. Leprous neuropathy; 29. HIV peripheral neuropathy; 30. Human t-cell leukemia virus (HTLV) associated neuropathy; 31. Herpes virus infection of the peripheral nervous system; 32. Lyme neuroborreliosis Section 8 Neuropathy and metabolic disorders 33. Diabetic neuropathy; 34. Biology of diabetic neuropathy; 35. Uremic neuropathy; 36. Porphyric neuropathy; 37. Fabry's disease; 38. Transthyretin familial amyloid polyneuropathy; 39. Hereditary gelsolin amyloidosis Section 9 Neuropathy and malignancy 40. Malignant cell infiltration in the peripheral nervous system; 41. Paraneoplastic neuropathy Section 10 Cryptogenic, Traumatic and Iatrogenic neuropathies 42. Drug induced neuropathies; 43. Late radiation injury to peripheral nerves; 44. Neuromuscular complications of critical illness; 45. The surgery of peripheral nerves (including tumours); 46. Neuropathy in the elderly Section 11. Familial neuropathies 47. Dominant Charcot-Marie-Tooth syndrome and cognate disorders; 48. Recessively transmitted predominantly motor neuropathies; 49. Early onset childhood monogenic neuropathies; 50. Hereditary sensory and autonomic neuropathies; 51. Peripheral nerve involvement in hereditary cerebellar and multisystem degenerative disorders; 52. Giant axonal neuropathy; 53. Neurofibromatosis 1(NF1), diagnosis and management; 54. Neurofibromatosis type 2 (NF2) diagnosis and management
