title: Inflammation and neuropathic attacks in hereditary brachial plexus neuropathy.
abstract: OBJECTIVE
  To study the role of mechanical, infectious, and inflammatory factors inducing neuropathic attacks in hereditary brachial plexus neuropathy (HBPN), an autosomal dominant disorder characterised by attacks of pain and weakness, atrophy, and sensory alterations of the shoulder girdle and upper limb muscles.
  
  
  METHODS
  Four patients from separate kindreds with HBPN were evaluated. Upper extremity nerve biopsies were obtained during attacks from a person of each kindred. In situ hybridisation for common viruses in nerve tissue and genetic testing for a hereditary tendency to pressure palsies (HNPP; tomaculous neuropathy) were undertaken. Two patients treated with intravenous methyl prednisolone had serial clinical and electrophysiological examinations. One patient was followed prospectively through pregnancy and during the development of a stereotypic attack after elective caesarean delivery.
  
  
  RESULTS
  Upper extremity nerve biopsies in two patients 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 patients intravenous methyl prednisolone ameliorated symptoms (largely pain), but with tapering of steroid dose, signs and symptoms worsened. Elective caesarean delivery did not prevent a typical postpartum attack.
  
  
  CONCLUSIONS
  Inflammation, probably immune, appears pathogenic for some if not all attacks of HBPN. Immune modulation may be useful in preventing or reducing the neuropathic attacks, 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 HBPN disorders.
authors.affiliation: Peripheral Neuropathy Research Center, Mayo Clinic, Rochester, Minnesota 55905, USA. klein.christopher@mayo.edu
authors.forename: C J
authors.initials: CJ
authors.lastname: Klein
authors.forename: P J B
authors.initials: PJ
authors.lastname: Dyck
authors.forename: S M
authors.initials: SM
authors.lastname: Friedenberg
authors.forename: T M
authors.initials: TM
authors.lastname: Burns
authors.forename: A J
authors.initials: AJ
authors.lastname: Windebank
authors.forename: P J
authors.initials: PJ
authors.lastname: Dyck
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D020516:Brachial Plexus Neuropathies / Q000150:complications* / Q000188:drug therapy / Q000235:genetics* / Q000473:pathology / Q000503:physiopathology; D002648:Child; D004594:Electrophysiology; D005260:Female; D006801:Humans; D007249:Inflammation / Q000209:etiology / Q000473:pathology; D008297:Male; D008775:Methylprednisolone / Q000008:administration & dosage; D008875:Middle Aged; D010375:Pedigree; D010523:Peripheral Nervous System Diseases / Q000209:etiology* / Q000473:pathology; D011247:Pregnancy; D011248:Pregnancy Complications / Q000209:etiology
chemical_list: D000893:Anti-Inflammatory Agents; D008775:Methylprednisolone