Stridor and death in multiple system atrophy

Patients with multiple system atrophy (MSA) have a mean survival of 8 to 10 years. Nocturnal stridor has been considered a poor prognostic feature. We analyzed demographic, clinical, and polysomnographic data and obtained follow-up information from 42 patients with MSA (30 with follow-up data) seen in a Sleep Disorders Center. Group I consisted of 17 patients with nocturnal stridor, including seven with daytime stridor. Group II consisted of 25 patients without stridor. Analysis of survival curves of 30 patients with follow-up information showed a significantly shorter survival from the sleep evaluation, but not from disease onset, for patients with stridor compared with those without. Nine of 11 patients with stridor died a median of 2 years from presentation and the only two survivors had undergone tracheostomy. Patients with daytime stridor and immobile vocal cords had especially poor prognoses. However, two of four patients with tracheostomies also died, as did six of 19 without stridor. We postulate that central hypoventilation and its complications may have been responsible for many of these other deaths. We conclude that stridor does carry a poor prognosis in patients with MSA but that there are also other causes of death. We recommend consideration of tracheostomy for patients with MSA who have stridor, but also assessment for central hypoventilation and appropriate management if it is present.