title: An analogue assessment of repetitive hand behaviours in girls and young women with Rett syndrome.
abstract: BACKGROUND
  Rett syndrome is a neurodevelopmental disorder that almost exclusively affects females. In addition to neurodevelopmental regression and loss of hand skills, apraxia, deceleration of head growth, and increasing spasticity and scoliosis, a number of behavioural features are also seen, including stereotypic hand movements, hyperventilation and breath holding. The aim of the study was to investigate the extent to which analogue environmental conditions affected the frequency of repetitive hand behaviour in eight girls and young women with Rett syndrome.
  
  
  METHODS
  The frequency of repetitive hand movements was observed every 10 s for four 4-min sessions under the following conditions: Continuous Adult Attention, Adult Demands, Stimulation and No Stimulation.
  
  
  RESULTS
  The frequency of repetitive hand movements was high -- they occurred in above 60% of all intervals in all conditions for all participants and at nearly 100% for some participants in some conditions. For one participant the frequency of repetitive hand movements was somewhat reduced in the Stimulation condition; for another it was relatively increased in the No Stimulation condition.
  
  
  CONCLUSIONS
  Overall, environmental manipulations had relatively limited effects on repetitive hand behaviours. Repetitive hand behaviour in Rett syndrome may be maintained by automatic reinforcement or neurochemical processes and may not be primarily influenced by contingent reinforcement.
authors.affiliation: St. Margaret's School, The Children's Trust, Tadworth, Surrey, UK. lwales@thechildrenstrust.org.uk
authors.forename: L
authors.initials: L
authors.lastname: Wales
authors.forename: T
authors.initials: T
authors.lastname: Charman
authors.forename: R H
authors.initials: RH
authors.lastname: Mount
mesh_terms: D000293:Adolescent; D005260:Female; D006225:Hand / Q000502:physiology*; D006801:Humans; D015518:Rett Syndrome / Q000150:complications*; D012720:Severity of Illness Index; D019956:Stereotypic Movement Disorder / Q000175:diagnosis* / Q000209:etiology* / Q000628:therapy; D014743:Videotape Recording