Rett Syndrome (RTT, OMIM 312750), a unique rare neurodevelopmental disorder, mostly affects females and causes severe multi-disabilities including poor sleep. This meta-analysis systematically reviewed the polysomnographic (PSG) data of individuals with RTT on both sleep macrostructure and sleep respiratory indexes and compared them to literature normative values. Studies were collected from PubMed, Web of Science, PsycINFO, Ebsco, Scopus, and Cochrane Library till 26 April 2022. Across 13 included studies, the 134 selected RTT cases were mostly females being MECP2 (n = 41) and CDKL5 (n = 4) positive. They were further stratified by gene, age, and clinical features. Findings of comparison with literature normative values suggested shorter total sleep time (TST) and sleep onset latency (SOL), twice as long wake after sleep onset (WASO) with lower sleep efficiency (SEI) in RTT, as well as increased non-rapid eye movement stage 3 (stage N3) and decreased rapid eye movement sleep. Based on limited data per stratifications, we found in RTT cases <5 years old lower stage N3, and in RTT cases >5 years old less WASO and more WASO in the epileptic strata. However, meta-results generated from studies designed with comparison groups only showed lower stage N1 in RTT than in healthy comparison, together with similar SEI and stage N3 to primary snoring subjects. For sleep respiratory indexes, severe disordered sleep breathing was confirmed across roughly all RTT strata. We are the first study to meta-analyze PSG data of sub...
Wake After Sleep Onset RTT Cases Stage N3 Severe Nocturnal Hypoxemia Presence Of Epilepsy Lower Sleep Efficiency Sleep Macrostructure Apneic Events Sleep Onset Latency Total Sleep Time
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No potential conflict of interest was reported by the authors. The conception and design of the study, acquisition of data, analysis and interpretatio...Read More
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