Direct Observation of the Behaviour of Females with Rett Syndrome

Abstract

The aim was to observe the behaviour of a sample of females with RTT and explore how it was organized in relation to environmental events. Ten participants, all with a less severe form of classic (n = 9) or atypical (n = 1) Rett syndrome (RTT), were filmed at home and at school or day centre. Analysis used real-time data capture software. Observational categories distinguished engagement in social and non-social pursuits, hand stereotypies, self-injury and the receipt of attention from a parent, teacher or carer. Associations between participant behaviour and intake variables and receipt of attention were explored. Concurrent and lagged conditional probabilities between behavioural categories and receipt of attention were calculated. Receipt of adult attention was high. Engagement in activity using the hands was associated with a less severe condition and greater developmental age. Engagement in activity, whether using the hands or not, and social engagement were positively associated with receipt of support. The extent of hand stereotypies varied greatly across participants but was independent of environmental events. Six participants self-injured. There was some evidence that self-injury was related to adult attention. Participants appeared to experience a carer and attention rich environment and their levels of engagement seemed high as a result. As in the more general literature, engagement in activity was related to personal development and to social support. Self-injury contrasted with hand stereotypies in having possible environmental function.

Movement disorders in patients with Rett syndrome: A systematic review of evidence and associated clinical considerations

“…As presented in Table 1, the 43 studies captured information across the movement disorder ecosystem in patients with RTT. About 42% of the studies used video observations to assess movement disorders 12,16–32 . Others used questionnaires as the primary assessment method 11,33–36 or as an adjunct with other methods 18 .…”

“…This theme underscores the influence of symptoms and stressors on movement disorders. The environment is important in managing negative behavior associated with self‐injury in RTT 18 . High‐stress conditions can also lead to visible signs of displeasure in the faces and the vocalization of patients with RTT 19 and could have implications on how movement behaviors can be affected by these signs.…”

“…About 42% of the studies used video observations to assess movement disorders. 12 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 Others used questionnaires as the primary assessment method 11 , 33 , 34 , 35 , 36 or as an adjunct with other methods. 18 The sample was also diverse, from an analysis of 1074 patients with RTT from the RTT Natural History Study, 10 to a study exploring a few cases.…”

“… 12 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 Others used questionnaires as the primary assessment method 11 , 33 , 34 , 35 , 36 or as an adjunct with other methods. 18 The sample was also diverse, from an analysis of 1074 patients with RTT from the RTT Natural History Study, 10 to a study exploring a few cases. 37 The data obtained allowed a thematic analysis to be performed and are presented in the next section.…”

“…The environment is important in managing negative behavior associated with self‐injury in RTT. 18 High‐stress conditions can also lead to visible signs of displeasure in the faces and the vocalization of patients with RTT 19 and could have implications on how movement behaviors can be affected by these signs. Evidence further suggests that environmental and sensory influences might have an important role in reducing the incidence of secondary disabilities associated with abnormal movements in RTT.…”