A 76 Year-Old Woman with Sleep and Waking Stridor, Sleep Talking, Orthostatic Hypotension, and Imbalance

Abstract

A 76-year-old woman presented with a three-month history of a harsh, high-pitched breathing noise during sleep, which had also recently begun to occur occasionally during the daytime while awake. Sleep talking had been noted for decades but recently had become louder and more agitated in character. No other nocturnal motor activity was noted. Over the last year, she had intermittent urinary urgency and incontinence; softer, imprecise speech; imbalance; and dizziness while standing, with severe supine hypertension. Midodrine was unhelpful. Her only medication was losartan 25 mg daily.On examination, there was moderate hypomimia, hypokinetic dysarthria, inspiratory and phonatory stridor (Video 1), moderate symmetric rigidity and bradykinesia, and a 30-mm systolic blood pressure drop from sitting to standing. Also noted was loud waking stridor, particularly notable during inspiration, with use of accessory respiratory muscles (sternocleidomastoid and neck strap muscles) indicating increased effort as the patient breathes against dysfunctional vocal cords (Video 1).Videolaryngoscopy showed significantly reduced vocal fold mobility bilaterally, with failure of abduction and paradoxical movement of true vocal fold tissue during inspiration. Autonomic reflex screen demonstrated severe orthostatic hypotension during head-up tilt table testing, and marked cardiovagal, cardiovascular adrenergic, and patchy postganglionic sympathetic sudomotor abnormalities. Thermoregulatory sweat testing demonstrated severe anhidrosis compatible with central autonomic failure. Brain MRI showed linear hyperintensities along the lateral aspects of the putamen (Figure 1)