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Polyssomnography analisys in Moebius sequence syndrome: new insights on REM Sleep
Genetic syndromes that interfere with the functioning of the Central Nervous System (CNS) have indirect effects on the sleep-wake cycle. Brain alterations can be generalized as well as focal, making it possible to dissect the structures involved in sleep disorders, for example, in the topography of the mid-brain and high medulla for Moebius Syndrome (MS).
Rapid Eye Movement (REM) sleep is characterized by rapid eye movement and muscle atony. The absence of eye movements due to VI paralysis and the frequent hypotonia of the mandibular muscles in MS makes it difficult to accurately characterize REM sleep.
There are few case reports with the analysis of MS patients and the clinical presentation of narcolepsy, as also for the quantification and study of REM sleep in these group.
The aim is to analyze the sleep architecture of pediatric patients from a tertiary service with a rare syndrome and to correlate their findings with possible sleep disorders. Furthermore, to analyze REM sleep staging data and its clinical correlations.
We described a sequence of five clinical cases including pediatric patients, , ranging from 2 to 12 years old, with MS under multidisciplinary follow-up. The study was a retrospective analysis of video-polysomnographic recordings from a tertiary reference pediatric care service, performed by physicians specializing in Sleep Medicine using the criteria of the American Academy of Sleep Medicine. It was possible to describe the overall sleep pattern of the sample, as well as to quantify the staging periods with absence of atony in REM sleep by its index.
Most patients with MS showed a reduction in sleep efficiency with increased WASO, as well as changes in REM sleep parameters such as increased latency and an important reduction in its percentage index. Furthermore, it was possible to quantify the loss of tone during REM sleep through electromyographic recordings of lower limbs in two patients (Patients 4 and 5) and of witnessed sucking movements in one patient (Patient 2).
Studies in the literature provide promising data on the applicability of the loss of atony loss index in REM sleep, having been shown to be an electrophysiological marker of good accuracy for diagnosing pediatric narcolepsy. This increase in the REM sleep atony loss index reinforces the importance of considering this electrophysiological marker in the clinical evaluation.
Moebius Syndrome (MS); Rapid Eye Movement (REM) Sleep; Loss of REM Atonia
Relato de Caso
Faculdade de Medicina da USP - São Paulo - Brasil
Ana Paula Assunção Cecilio, Clarissa Bueno, Maria Joaquina Marques-Dias, Leticia Azevedo Soster