ISSN:1350-7540    

出版商:OVID    

年代:1997

数据来源: OVID

ISSN:1350-7540    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

Autoimmune ataxic neuropathies are a subset of the sensory ataxic neuropathies which are characterized by ataxia as the dominant presenting feature. The major known causes of autoimmune ataxic neuropathies include sensory variants of the Guillain-Barré syndrome, including Miller-Fisher syndrome, subsets of immunoglobulin M paraproteinaemic neuropathy, paraneoplastic neuropathy and the neuropathy associated with Sjögren's syndrome. Identified antigens as targets for autoantibodies include gangliosides, myelin associated glycoprotein, Hu antigen and extractable nuclear antigens. Some recent studies support the pathogenic role of anti-GD1b ganglioside antibody in autoimmune ataxic neuropathies. The major site of pathology in autoimmune ataxic neuropathies is in the dorsal root ganglion, but dorsal roots and peripheral nerve myelin and axons may also be affected.

ISSN:1350-7540    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

Paraneoplastic neuronopathies are presumed to be the result of an autoimmune attack directed at neuronal proteins, and both humoral and cell-mediated mechanisms have been postulated. The lower motor neuron syndrome after irradiation to the spinal column is caused by a proximal motor polyradiculopathy. Prevention of brachial plexopathy after radiotherapy for breast cancer may be accomplished by lower doses and surgical management of the axilla. Polymerase chain reaction casts doubt on the distinction between neoplastic and paraneoplastic mechanisms of neuromuscular manifestations of lymphoproliferative diseases. Shared antigenic components may underlie the association between inflammatory neuropathy and malignant melanoma. Advances in chemotherapy strategies against responsive tumors are hindered by the toxic effects of agents on peripheral nerves.

ISSN:1350-7540    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

Epidemiological studies have documented a high prevalence of diabetic neuropathy. The risk of lower leg amputation is increased three to four times in patients with clinical signs of neuropathy and ankle weakness is more common than hitherto recognized. Increased nerve hydration and increased expression of low affinity p75 receptor for neurotrophins suggest new therapeutic potentials for the prevention of diabetic neuropathy.

ISSN:1350-7540    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

Proximal childhood spinal muscular atrophy is a common autosomal recessive disorder that results in degeneration of lower motor neurons of the spinal cord. The defective gene, survival of motor neuron, encodes a novel protein with a putative role in RNA metabolism. Further work is required to define clearly the mechanism by which the survival of motor neuron gene defect would result in motor neuron degeneration.

ISSN:1350-7540    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

The most common forms of inherited demyelinating neuropathy in humans are caused by mutations in the genes encoding protein zero, peripheral myelin protein 22 kDa, and connexin32, all of which are expressed by myelinating Schwann cells and are components of the myelin sheath. The phenotype of myelinating Schwann cells depends on the maintenance of axon-Schwann cell interactions, because axonal degeneration also leads to the breakdown of the myelin sheath and dedifferentiation of the previously myelinating cells into ‘denervated’ Schwann cells, which are essential for axonal regeneration. Several transcription factors have been shown to play critical roles in regulating the phenotype of Schwann cells, including SCIP/tst-1/Oct-6 andKrox-20, both of which are required for the normal development of the myelinating phenotype.

ISSN:1350-7540    

出版商:OVID    

年代:1997

数据来源: OVID

ISSN:1350-7540    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

Congenital myasthenic syndromes are a rare group of heterogeneous disorders affecting neuromuscular transmission. Recent identification and in-vitro functional analysis of some of the genetic mutations that cause these disorders correlates with previous electrophysiological, biochemical, pathological and therapeutic studies, and has advanced our understanding of neuromuscular transmission.

ISSN:1350-7540    

出版商:OVID    

年代:1997

数据来源: OVID

摘要:

Mitochondrial disorders may be caused by mutations either in mitochondrial or in nuclear genes involved in the synthesis or regulation of respiratory chain subunits. The unique nature of the mitochondrial genome calls for a different approach to genetic counselling and risk analysis.

ISSN:1350-7540    

出版商:OVID    

年代:1997

数据来源: OVID