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Sleep problems in children with Sanfilippo syndrome
G A Colville1, J P Watters, W Yule
1Department of Child Psychology and Psychiatry, St George's Hospital, London, UK.
Insights
Most children with Sanfilippo syndrome experience significant sleep problems and unusual nighttime behaviors. Tailored behavior management offered some families immediate, though not always sustained, improvements.
Area of Science:
- Neuroscience
- Genetics
- Pediatrics
Background:
- Sanfilippo syndrome is a rare genetic disorder causing severe neurodevelopmental and behavioral issues.
- Sleep disturbances and unusual nighttime behaviors are common in children with Sanfilippo syndrome.
Abstract:
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.