TY - JOUR
T1 - Case series
T2 - Anesthetic management of patients with spinal and bulbar muscular atrophy (Kennedy's disease)
AU - Niesen, Adam D.
AU - Sprung, Juraj
AU - Prakash, Y. S.
AU - Watson, James C.
AU - Weingarten, Toby N.
N1 - Funding Information:
Acknowledgement We sincerely thank Dr. William Kennedy for his review of our manuscript. This work was entirely supported by the Department of Anesthesiology, Mayo Clinic, Rochester, MN, USA.
PY - 2009/2
Y1 - 2009/2
N2 - Purpose 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. Clinical features 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. Conclusion 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.
AB - Purpose 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. Clinical features 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. Conclusion 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.
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U2 - 10.1007/s12630-008-9018-1
DO - 10.1007/s12630-008-9018-1
M3 - Article
C2 - 19247761
AN - SCOPUS:64849103444
SN - 0832-610X
VL - 56
SP - 136
EP - 141
JO - Canadian Journal of Anesthesia
JF - Canadian Journal of Anesthesia
IS - 2
ER -