Sleep-related breathing disorders in the Shy-Drager syndrome. Observations on investigation and management

Eur J Neurol. 1996 May;3(3):186-90. doi: 10.1111/j.1468-1331.1996.tb00421.x.


We report on 18 patients with the Shy-Drager syndrome who were referred for assessment of a sleep-related breathing disorder. The main symptoms of snoring, apnoea, stridor and daytime hypersomnolence were detailed and vocal cord movement graded by laryngoscopy as normal, mild weakness of abduction or near paralysis of vocal cord movement Sleep studies involving oximetry and observations were performed. Obstructive and central apnoeas were detected in six patients. Many patients with near paralysis of vocal cord abduction had normal sleep studies. Successful management strategies of obstructive cases included CPAP, nasal surgery and tracheostomy or arytenoidectomy alone or in combination. Sleep-related breathing disorders in the Shy-Drager syndrome may involve significant nocturnal desaturation; investigation with suitable medical or surgical treatment should be employed in these patients.