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Graham A. Glass, MD; Keith A. Josephs, MST, MD; J. Eric Ahlskog, PhD, MD

Arch Neurol. 2006;63:978-981.

Background  Respiratory stridor, sleep-disordered breathing, and respiratory insufficiency are part of the clinical spectrum of multiple-system atrophy (MSA). We have encountered cases where these were presenting symptoms, with the diagnosis of MSA being initially unrecognized.

Objective  To describe cases in which breathing difficulties were the initial and primary manifestation of MSA.

Design  Database review from January 1, 1996, through October 31, 2005.

Setting  Mayo Clinic, Rochester, Minn.

Patients  All patients diagnosed as having MSA, cross-referenced for apnea, hypopnea, or hypoventilation. On review, we included only cases in which respiratory dysfunction was the primary initial clinical event in MSA, excluding equivocal cases.

Interventions  None.

Main Outcome Measures  Characteristics and clinical course of patients.

Results  Six cases were identified in which substantial respiratory insufficiency occurred as an early, presenting symptom of MSA. Three patients had been examined emergently for acute respiratory distress before the ultimate diagnosis of MSA; the other 3 patients were diagnosed as having obstructive sleep apnea unresponsive to therapy, with bilateral vocal cord paralysis found on ear, nose, and throat examination. Stridor was noted early in the course in all. All patients required tracheostomy, and all eventually developed features consistent with probable MSA.

Conclusions  Multiple-system atrophy may occasionally present as primary respiratory failure or dysfunction, with initially mild motor and autonomic symptoms. Otherwise unexplained central respiratory failure, bilateral vocal cord paralysis, stridor, or refractory central sleep apnea should prompt consideration of MSA.

Author Affiliations: Department of Neurology, Mayo Clinic, Rochester, Minn.

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