Stridor and death in multiple system atrophy

Patients with multiple system atrophy (MSA) have a mean survival of 8 to 10 years. Nocturnal strider has been considered a poor prognostic feature. We analyzed demographic, clinical, and polysomnographic data and obtained fol low-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 nocturna l strider, including seven with daytime strider. Group II consisted of 25 p atients without strider. Analysis of survival curves of 30 patients with fo llowup information showed a significantly shorter survival from the sleep e valuation, but not from disease onset, for patients with strider compared w ith those without. Nine of 11 patients with strider died a median of 2 year s from presentation and the only two survivors had undergone tracheostomy. Patients with daytime strider and immobile vocal cords had especially poor prognoses. However, two of four patients with tracheostomies also died, as did six of 19 without strider. We postulate that central hypoventilation an d its complications may have been responsible for many of these other death s. We conclude that strider does carry a poor prognosis in patients with MS A but that there are also other causes of death. We recommend consideration of tracheostomy for patients with MSA who have strider, but also assessmen t for central hypoventilation and appropriate management if it is present.