Sleep problems in children with Sanfilippo syndrome.

Sleep problems in children with Sanfilippo syndrome.

Colville GA; Watters JP; Yule W; Bax M

Developmental Medicine And Child Neurology

1996

Child; Female; Humans; Male; Questionnaires; Longitudinal Studies; Time Factors; Preschool; Sleep Disorders/etiology; Sleep Disorders/therapy; Mucopolysaccharidosis III/classification; Mucopolysaccharidosis III/complications

Sanfilippo syndrome is a rare degenerative disorder which has severe intellectual and behavioural sequelae, commonly including sleep problems. A parental questionnaire was used to gather information on the sleep patterns of 80 children with Sanfilippo syndrome (mean age 10 years 2 months). The majority were found to have sleep problems (78%). Many also exhibited other distressing and unusual night time behaviours (staying up all night, chewing the bedclothes or crying out suddenly), and a few laughed or sang. Such problems may have been more severe in those with Sanfilippo syndrome type B. In four of the families offered individually tailored behaviour-management advice there was immediate improvement, which was maintained at followup in two cases. These results demonstrate the usefulness of even such a minimal intervention, even in a very difficult population such as this.

1996

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