Abstracts

Rationale: Sturge-Weber Syndrome (SWS) typically presents with port-wine stain, seizures and motor difficulties. Comorbidities such as behaviour and sleep disturbances are described but not well understood in SWS. Our aim was to determine the frequency of these comorbidities and to evaluate possible predisposing factors. Methods: Case records were reviewed of 92 children with SWS presenting at a single tertiary centre between 2002 and 2015. Using a structured form, retrospective data collection included documentation of behaviour and sleep difficulties, use of standardised screening questionnaires including strength and difficulties questionnaire (SDQ), and recording of pial angioma, epilepsy history and social communication profiles. Results: Eighty-five percent (78/92) of children with SWS had a diagnosis of epilepsy and 63% of those with epilepsy (49/78) had a history of status epilepticus. Behavioural difficulties were reported in 46/92 children (50%). Analysing SDQ data (n=40) 44% reported emotional distress, 53% reported hyperactive inattention behaviour, 48% had difficulties with other children and 67% reported a high impact on the child's life. Sleep problems were encountered in 24 children (26%). Among these 45% had delayed sleep onset, 62% were waking during the night and 32% were rising early. 18/24 children (75%) were taking medication for sleep related problems. Melatonin was given in all cases and combined with chloral hydrate in 4 cases. Sleep and behavioural difficulties were closely linked: 21/46 children (46%) with behavioural difficulties also had sleep disturbances, whereas among those without behavioural problems sleep disturbances occurred in only 3/46 (7%; p < 0.01). Similarly the presence of social communication difficulties was more often associated with behavioural concerns (59%vs30%; p < 0.01) and with sleep disturbances (67%vs37%; p=0.01). Occurrence of behavioural or sleep problems was not related to the extent of pial angioma, the presence of hemiplegia, seizure frequency or a history of status epilepticus. Conclusions: Behavioural difficulties and sleep disturbances are frequently encountered in children with Sturge-Weber Syndrome independent of seizure frequency or the extent of the underlying vascular abnormality. Sleep and behaviour should be routinely assessed in SWS to facilitate intervention programmes and treatment strategies as early as possible. Funding: None