Sleep-Related Breathing Disorders in Patients With Multiple System Atrophy and Vocal Fold Palsy

Abstract

We performed sleep studies in eight patients with multiple system atrophy (MSA) and three patients with peripheral bilateral vocal fold palsy (PBVFP) and investigated stenosis of the upper airway tract during sleep in MSA patients with vocal fold palsy. Among the eight MSA patients in this study, five had definite glottic snoring and two others were suspected of having glottic snoring. Of the PBVFP patients, two had glottic snoring. Three of 11 patients died, and two of the three deaths occurred during sleep. Glottic snoring indicated a high degree of negative esophageal pressure. High negative esophageal pressure demonstrates severe narrowing of the upper airway tract. Therefore, glottic snoring should be considered a risk factor for sudden death in sleep. Repeated laryngoscopic examination is useful in evaluating the progressive process of vocal fold palsy while awake, but this examination performed only while awake is not enough to evaluate narrowing of the upper airway during sleep. Sleep studies that include the measurement of esophageal pressure can be very useful in evaluating the severity of narrowing in the upper airway tract. It is suspected that sudden nocturnal death in MSA patients is caused not only by abnormal respiration resulting from impairment of the respiratory center, but also by glottic obstruction caused by sputum or by edema of the vocal folds. We recommend treatment of respiratory disorders when loud laryngeal snoring occurs in patients with MSA, even if they do not complain of dyspnea while awake.

Respiratory aspects of neurological disease

“…In the related condition of multisystem atrophy, glottic narrowing during sleep has been suggested as a possible cause of sudden nocturnal death. This is not always detectable by fibreoptic examinations conducted while the patient was awake,156 157 and polysomnography may therefore be indicated in symptomatic patients. However, in another study of patients with progressive supranuclear palsy, frequent desaturations were not found 158…”

“…The cause of the acute encephalopathy of hypercapnia is thought in part to be due to the rapid rate of diffusion of CO 2 across the blood-brain barrier. This leads to an abrupt fall in pH in the CSF, which in turn inhibits the post-synaptic receptor of the excitatory neurotransmitter glutamate 156. Overvigorous mechanical ventilation to correct hypercapnia can also cause coma and seizures, perhaps due to hyperventilation induced cerebral vasoconstriction.…”

Obstructive sleep apnea syndrome in a patient with medulloblastoma

“…Degenerative disorders, such as multiple system atrophy, which involve the pons and cerebellum, have been implicated in both central (Salazar-Grueso et al, 1988) and obstructive sleep apnea (Adelman et al, 1984). The only case of multiple system atrophy with purely obstructive sleep apnea (Sadaoka et al, 1996), however, was also associated with xation of the right vocal fold in the paramedian position. Isolated cerebellar damage has never been shown to lead to purely obstructive sleep apnea, but one might imagine that it could on the basis of hypotonia.…”

Autonome Dysfunktion mit nächtlicher Dyspnoe (Gerhardt-Syndrom) im Rahmen einer multiplen Systematrophie

“…Als Ort der Obstruktion und des Schnarchens bei Patienten mit multipler Systematrophie wurden von Sadaoka et al [18] vor allem die Glottis, sog. Stimmbandschnarchen, aber auch der weiche Gaumen gefunden.…”

“…Es wurden zwar alternativ Versuche mit einer Lateralfixation eines Stimmbandes gemacht, aber die Tracheotomie gilt zurzeit als Mittel der Wahl [18]. Allerdings sind auch bei tracheotomierten Patienten mit multipler Systematrophie plötzliche nächtliche Todesfälle beschrieben worden, was auf eine Entgleisung der autonomen kardiorespiratorischen Regulationsmechanismen hindeuten könnte [13].…”