Nachtelijke stridor bij multipele systeematrofie

Background

Multiple system atrophy is a neurodegenerative disorder with parkinsonism, cerebellar ataxia and autonomic dysfunction. The occurrence of nocturnal stridor in patients with multiple system atrophy is associated with a decreased life expectancy. This is what makes adequate treatment so important. Nevertheless, stridor goes often unrecognized and there is frequent uncertainty about the therapeutic options.

Case

We present a 58-year-old woman with multiple system atrophy and nocturnal stridor. Direct laryngoscopy showed bilateral paresis of the vocal cords and glottic stenosis. Since the outcome of treatment with continuous positive airway pressure (CPAP) was unsatisfactory, a tracheotomy was performed after which the stridor disappeared.

Conclusion

Polysomnography and laryngoscopy are the important diagnostic tools for this condition, after the clinical interview. Tracheotomy used to be the only therapeutic option for stridor. However, recent studies show a comparable effectiveness of non-invasive treatment with CPAP, provided there is a correct indication. The choice of therapy is a multidisciplinary process in which the neurologist, the sleep medicine specialist, the otolaryngologist, and the patient are involved.

Conflict of interest: S. Overeem is member of the UCB Xyrem Advisory Board and has held lectures for which his institute received payment from UCB Pharma, Boehringer Ingelheim and Novartis. Financial support: none declared.