Case series

Anesthetic management of patients with spinal and bulbar muscular atrophy (Kennedy's disease)

Adam D. Niesen, Juraj Sprung, Y.s. Prakash, James Watson, Toby N. Weingarten

Research output: Contribution to journalArticle

6 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)136-141
Number of pages6
JournalCanadian Journal of Anesthesia
Volume56
Issue number2
DOIs
StatePublished - Feb 2009

Fingerprint

X-Linked Bulbo-Spinal Atrophy
Atrophic Muscular Disorders
Anesthetics
Laryngismus
Medical Records
Anesthesia
Neuromuscular Blocking Agents
General Anesthetics
Succinylcholine
Muscular Atrophy
Tracheostomy
Muscle Weakness
Motor Neurons
Pneumothorax
Tongue
Neurodegenerative Diseases
General Anesthesia
Edema
Extremities
Databases

ASJC Scopus subject areas

  • Anesthesiology and Pain Medicine

Cite this

Case series : Anesthetic management of patients with spinal and bulbar muscular atrophy (Kennedy's disease). / Niesen, Adam D.; Sprung, Juraj; Prakash, Y.s.; Watson, James; Weingarten, Toby N.

In: Canadian Journal of Anesthesia, Vol. 56, No. 2, 02.2009, p. 136-141.

Research output: Contribution to journalArticle

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