title: Case series: anesthetic management of patients with spinal and bulbar muscular atrophy (Kennedy's disease).
abstract: OBJECTIVE
  Kennedy's disease (KD) is a rare, X-linked recessive, neurodegenerative disorder of lower motor neurons characterized by progressive proximal limb and bulbar muscular atrophy with spontaneous laryngospasm, which may present an anesthetic risk. We undertook a computerized search of the Mayo Clinic medical records database between January 1996 and May 2008 for patients with KD undergoing general anesthesia. Medical records were reviewed for anesthetic techniques and perioperative complications.
  
  
  METHODS
  We identified six patients with KD, confirmed by DNA testing, who underwent 13 general anesthetics. Succinylcholine was used in two patients, and non-depolarizing neuromuscular blockers in seven cases, all without adverse effects. Although laryngospasm was not identified in any patient, one patient with advanced disease experienced postoperative glottic edema, worsening respiratory distress, bulbar dysfunction, requiring tracheostomy and prolonged ventilatory support. One patient experienced a pneumothorax.
  
  
  CONCLUSIONS
  The potential for bulbar dysfunction and muscle weakness in patients with KD places them at risk for perioperative complications from anesthesia. Anesthesia providers should be cognizant of the different potential anesthetic risk factors in these patients.
authors.affiliation: Department of Anesthesiology, College of Medicine, Mayo Clinic, 200 First Street S.W., Rochester, MN, 55905, USA.
authors.forename: Adam D
authors.initials: AD
authors.lastname: Niesen
authors.forename: Juraj
authors.initials: J
authors.lastname: Sprung
authors.forename: Y S
authors.initials: YS
authors.lastname: Prakash
authors.forename: James C
authors.initials: JC
authors.lastname: Watson
authors.forename: Toby N
authors.initials: TN
authors.lastname: Weingarten
mesh_terms: D000328:Adult; D000368:Aged; D000768:Anesthesia, General / Q000009:adverse effects / Q000379:methods*; D016208:Databases, Factual; D006801:Humans; D007826:Laryngismus / Q000209:etiology; D008297:Male; D008875:Middle Aged; D009134:Muscular Atrophy, Spinal / Q000150:complications / Q000503:physiopathology / Q000601:surgery*; D009467:Neuromuscular Depolarizing Agents / Q000009:adverse effects / Q000627:therapeutic use*; D003473:Neuromuscular Nondepolarizing Agents / Q000009:adverse effects / Q000627:therapeutic use*; D011183:Postoperative Complications / Q000517:prevention & control; D012189:Retrospective Studies; D013390:Succinylcholine / Q000009:adverse effects / Q000627:therapeutic use
chemical_list: D009467:Neuromuscular Depolarizing Agents; D003473:Neuromuscular Nondepolarizing Agents; D013390:Succinylcholine