Vocal cord abductor paralysis in spinocerebellar ataxia type 1

Vocal cord abductor paralysis (VCAP) is considered a sign of a poor prognosis in neurodegenerative diseases, because severe laryngeal dysfunction by VCAP may result in acute airway obstruction and require emergency tracheotomy.1 Although VCAP is a cardinal feature in multiple system atrophy (MSA), it has not been reported in several types of spinocerebellar ataxia with dominant inheritance. We evaluated the movements of the vocal cords of seven patients with SCA1 by laryngofibroscopy. Seven unrelated patients with SCA1 who had the expanded CAG repeat of ataxin-1 were investigated. There were two men and five women ranging in age from 27 to 67 years old (mean 44.5 years). Spouses and other family members, in addition to the patients, were questioned about events of stridor, dyspnoea, and dysphagia. Vocal cord movement was examined by laryngofibroscopy and recorded during inspiration and phonation. The rating scale used to evaluate maximal abduction of the vocal …