title: Surgical and postpartum hereditary brachial plexus attacks and prophylactic immunotherapy.
abstract: BACKGROUND
  Surgery and childbirth can trigger attacks of hereditary brachial plexus neuropathy (HBPN), and inflammation was suggested as a component of the pathogenesis.
  
  
  METHODS
  HBPN patients who underwent surgery or parturition from January 1, 1996 to December 31, 2009 were studied.
  
  
  RESULTS
  Twenty-five HBPN patients underwent 48 surgeries or parturitions. Seventeen patients (68%) had attacks, including 13 periprocedural and 7 postpartum by varied anesthesia types. Three patients who had 8 earlier combined attacks (after thyroidectomy, laminectomy, and Caesarean section) were given prophylactic immunosuppressive therapy (corticosteroids ± immunoglobulin). None suffered postoperative attacks, which is uncharacteristic of their prior experience. Five had perioperative attacks as their first HBPN manifestation. Median follow-up was 11 months (3-48 months). Attacks occurred in the operated limb (n = 6) or distant (n = 7) to surgical sites. All attacks interfered with daily living, with frequent incomplete recovery. Five patients had a SEPT9 mutation.
  
  
  CONCLUSIONS
  Corticosteroids may prevent parturition and surgical HBPN attacks in some patients. Diverse surgeries, anesthesia, and childbirth frequently trigger HBPN attacks.
authors.affiliation: Department of Neurology, Division of Peripheral Nerve Diseases and Medical Genetics, Mayo Clinic Foundation, 200 First Street S.W., Rochester, Minnesota, USA. klein.christopher@mayo.edu
authors.forename: Christopher J
authors.initials: CJ
authors.lastname: Klein
authors.forename: David W
authors.initials: DW
authors.lastname: Barbara
authors.forename: Juraj
authors.initials: J
authors.lastname: Sprung
authors.forename: Peter J
authors.initials: PJ
authors.lastname: Dyck
authors.forename: Toby N
authors.initials: TN
authors.lastname: Weingarten
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D020968:Brachial Plexus Neuritis; D020516:Brachial Plexus Neuropathies / Q000209:etiology* / Q000235:genetics / Q000517:prevention & control; D002585:Cesarean Section / Q000009:adverse effects*; D005260:Female; D006801:Humans; D007167:Immunotherapy; D007796:Laminectomy / Q000009:adverse effects*; D008297:Male; D008875:Middle Aged; D049590:Postpartum Period; D013965:Thyroidectomy / Q000009:adverse effects*