Vocal Fold "Paralysis": An Early Sign in Multiple System Atrophy

Multiple system atrophy (MSA) is a neurodegenerative disease characterized by a cerebellar syndrome, autonomic dysfunction, and extrapyramidal signs. Extrapyramidal signs may manifest as parkinsonism as well as dystonia, which is the involuntary contraction of a muscle(s) resulting in an abnormal posture. MSA belongs to a family of diseases known as α-synucleinopathies which are associated with dream enactment reflecting REM sleep behavior disorder (RBD). In patients with MSA, dystonia or paresis may involve the laryngeal muscles resulting in vocal fold hypomobility. We identified four individuals presenting with vocal complaints and subsequently diagnosed with vocal fold "paralysis." Within one year, each patient developed neurologic symptoms and upon evaluation by a movement disorders specialist was diagnosed with probable MSA. Our findings highlight the importance of screening by otolaryngologists when patients are diagnosed with vocal fold hypomobility. Specifically, patients should be assessed for RBD by questioning others if he/she acts out their dreams. The presence of RBD raises clinical suspicion for a synucleinopathy such as MSA. Untreated patients with MSA experiencing nocturnal stridor and breathing disorders have an increased risk for sudden death. Therefore, early evaluation by a movement disorders specialist to promptly diagnose MSA may have a substantial effect on morbidity and mortality in this high-risk patient population.