Sanfilippo syndrome is a rare degenerative disorder which has severe i
ntellectual and behavioural sequelae, commonly including sleep problem
s. A parental questionnaire was used to gather information on the slee
p 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 (st
aying up all night chewing the bedclothes or crying out suddenly), and
a few laughed or sang. Such problems may have been more severe in tho
se with Sanfilippo syndrome type B. In four of the families offered in
dividually tailored behaviour-management advice there was immediate im
provement, which was maintained at follow-up in two cases. These resul
ts demonstrate the usefulness of even such a minimal intervention, eve
n in a very difficult population such as this.