Multiple system atrophy laryngeal stridor treated with radiofrequency-assisted posterior cordotomy and arytenoidectomy

Abstract

Stridor is a harsh, high-pitched breathing sound caused by the rapid and turbulent airfow through a narrow airway. In patients with multiple system atrophy (MSA), stridor represents a supportive feature for clinical diagnosis and a prognostic marker when present during wakefulness and associated with bilateral vocal fold immobility (BVFI), due to the risk of upper airway obstruction and sudden death during sleep [1]. Current treatment recommendations when continuous positive airway pressure (CPAP) ventilation is inefective are limited to tracheostomy [1]. However, there is a scarcity of less-invasive alternatives for functionally independent patients who wish to avoid tracheostomy. Here we present a 55-year-old male patient with MSA who developed continuous stridor with dyspnea on exertion and was treated with posterior cordotomy and arytenoidectomy using radiofrequency (RF) through a microlaryngoscopic approach. The patient was diagnosed with MSA due to poorly levodopa-responsive Parkinsonism with onset at age 49 and an MDS-Unifed Parkinson’s Disease Rating Scale (MDS-UPDRS) III score of 48 points despite being under chronic levodopa therapy up to 1000 mg/day. At age 51, he developed urogenital failure, with erectile dysfunction, urinary urgency, and incomplete voiding, that at age 53 was followed by urinary incontinence requiring intermittent catheterization. On diagnostic assessment, brain magnetic resonance imaging (MRI) showed slight putaminal atrophy with an incomplete hyperintense lateral rim, an orthostatic stress test revealed nonsymptomatic neurogenic orthostatic hypotension, and a whole exome sequencing test revealed a variant of uncertain signifcance (VUS) in the LRRK2 gene (LRRK2 c.4536+3A>G, ClinVar: RCV000873745.6) that was interpreted as unrelated to the diagnosis.