OBJECTIVE: To study the role of mechanical, infectious, and inflammatory factors inducing @entity1109 in @entity11438 ( @entity11438 ), an @entity74 characterised by attacks of @entity158 and @entity425 , @entity194 , and sensory alterations of the shoulder girdle and upper limb muscles. METHODS: Four @entity1 from separate kindreds with @entity11438 were evaluated. Upper extremity nerve biopsies were obtained during attacks from a @entity1 of each kindred. In situ hybridisation for common viruses in nerve tissue and genetic testing for a @entity7577 ( @entity7577 ; @entity16 ) were undertaken. Two @entity1 treated with intravenous @entity1667 had serial clinical and electrophysiological examinations. One @entity1 was followed prospectively through pregnancy and during the development of a stereotypic attack after elective caesarean delivery. RESULTS: Upper extremity nerve biopsies in two @entity1 showed prominent perivascular inflammatory infiltrates with vessel wall disruption. Nerve in situ hybridisation for viruses was negative. There were no tomaculous nerve changes. In two @entity1 intravenous @entity1667 ameliorated symptoms ( @entity158 ), but with tapering of @entity134 dose, signs and symptoms worsened. Elective caesarean delivery did not prevent a typical postpartum attack. CONCLUSIONS: @entity32 , probably immune, appears pathogenic for some if not all attacks of @entity11438 . Immune modulation may be useful in preventing or reducing the @entity1109 , although controlled trials are needed to establish efficacy, as correction of the mutant gene is still not possible. The genes involved in immune regulation may be candidates for causing @entity74 .

@entity32 and XXXX in @entity11438 .

@entity1 :: ('9606', 'Species') :: ['patients', 'patient', 'person'] @entity1667 :: ('MESH:D008775', 'Chemical') :: ['methyl prednisolone'] @entity32 :: ('MESH:D007249', 'Disease') :: ['Inflammation'] @entity11438 :: ('MESH:D020968', 'Disease') :: ['HBPN', 'hereditary brachial plexus neuropathy'] @entity1109 :: ('MESH:D012678', 'Disease') :: ['neuropathic attacks'] @entity16 :: ('MESH:D009422', 'Disease') :: ['tomaculous neuropathy'] @entity425 :: ('MESH:D018908', 'Disease') :: ['weakness'] @entity7577 :: ('MESH:C536965', 'Disease') :: ['hereditary tendency to pressure palsies', 'HNPP'] @entity134 :: ('CHEBI:35341', 'Chemical') :: ['steroid'] @entity194 :: ('MESH:D001284', 'Disease') :: ['atrophy'] @entity74 :: ('MESH:D030342', 'Disease') :: ['HBPN disorders', 'autosomal dominant disorder'] @entity158 :: ('MESH:D010146', 'Disease') :: ['largely pain', 'pain']
@entity1109 :: (MESH:D012678,Disease) :: ['neuropathic attacks']
