Recognizing and treating Rett syndrome in schools

Abstract

A review of the literature on Rett syndrome (RS) for school-based professionals is presented from a behavioral perspective. A description of RS is provided, including distinctive physical, behavioral, and emotional features, diagnostic criteria for classic and formes frustes forms of RS, and stages of the disorder. The similarities and differences between RS and autism are reviewed. Controversies regarding the classification of RS are presented, including whether RS should be classified as a neurological or psychiatric condition and whether RS should remain classified as a pervasive developmental disorder. Last, a variety of options for the treatment of RS are discussed.

Key issues in Rett syndrome: emotional, behavioural and autonomic dysregulation (EBAD) - a target for clinical trials

“…In RTT, the emotional state of the individual can be further exacerbated by the physical difficulties observed, for example, epileptic seizures can lead to a heightened emotional state and often leads to anxiety [ 69 ] but can also include screaming, labile mood and uncontrollable crying [ 67 , 70 ]. Regarding lifespan, emotion and behaviour are thought to change during the time course of RTT [ 71 , 72 ]. Behavioural dysregulation can present with increased stereotypies, repetitive rocking, scratching, self-injurious or self-stimulatory behaviour, and agitation.…”

Social‐emotional instability in individuals with Rett syndrome: parents' experiences with second stage behaviour

“…The social‐emotional behaviour in particular leads to questions in parents of children with RTT. The children's behaviour may not only differ from the behaviour of typically developing children but also according to the stage of RTT (Kaufmann et al ; Wanzek et al ). Children in the early stages of RTT especially may display emotional and behavioural disturbances (such as sleeping problems or screaming).…”

The effects of sleep disturbance on school performance: A preliminary investigation of children attending elementary grades

“…The data available so far on the link between sleep disorders and school performance arise mainly from samples of children attending middle or high school (Eliasson et al., 2002; Giannotti et al., 2002; Millman, 2005; Perez-Chada et al., 2007; Sadeh et al., 2002; Shin et al., 2003; Wolfson & Carskadon, 2003) or from clinically referred samples (Wanzek, Jenson, & Houlihan, 2012) as well as from children who have experienced crises or natural disasters (Widyatmoko, Tan, Seyle, Mayawati, & Silver, 2011). However, little is known about the possible association between sleep problems and academic achievement in young children.…”