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Rett Syndrome and Sleep

Reference work entry

Abstract

Rett syndrome is one of the most common causes of complex disability in girls. It is characterized by early neurological regression that severely affects motor, cognitive, and communication skills, by autonomic dysfunction and often a seizure disorder. It is a monogenic X-linked dominant neurodevelopmental disorder related to mutation in MECP2, which encodes the methyl-CpG binding protein MeCP2. We discuss the clinical aspects with special emphasis on sleep macrostructure and epilepsy but also sleep ventilatory evaluation and its potential role in the development of peculiar signs of Rett syndrome as social interaction and language development on one hand and the ventilatory impairment often recorded in RTT on the other hand. We review current perspectives in clinical management alongside.

Keywords

Sleep Problem Rett Syndrome Epileptiform Activity Epileptic Activity MECP2 Gene 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer Science+Business Media New York 2014

Authors and Affiliations

  1. 1.Department of NeurologyUniversité Libre de Bruxelles (ULB), Hôpital Universitaire des Enfants Reine FabiolaBrusselsBelgium

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