S.P.25 Sleep disorders in type II SMA

Abstract: The aim of this study was to assess sleep disorders in children with type II SMA. More specifically, we wished to establish a profile of various aspects of sleep behavior and their possible relation with motor functional aspects or the presence of ventilator devices or ortheses used at night time. Fifty children with type II SMA (mean age: 9.5years) were included in the study. All were assessed using the Sleep Disturbance Scale for Children,SDSC, a questionnaire specifically developed to assess a variety of sleep-related behaviors in childhood. The questionnaire provides a total score and subscores related to specific aspects of sleep: Disorders of initiating and maintaining sleep, Sleep breathing disorders, Disorders of arousal, nightmares, Sleep wake transition disorders, Disorders of excessive somnolence, Sleep hyperhydrosis. All the patients were also assessed using Hammersmith Functional Motor Scale (HFMS). 74% of the patients had a total SDSC score within normal limits and 26% had scores that were considered outside the normal range. In a number of patients the total SDSC scores were normal but the subscores on one or more subscales were not and only 17/50 (34%) had normal scores on all the subscales. Sleep hyperhydrosis and disorders of initiating and maintaining sleep were more commonly found to be outside the normal range. We did not find any specific association with the presence of night splints or with functional motor levels. Although all but one of the children with abnormal scores on the sleep questionnaire had low scores on the HFMS the opposite was not always true as a number of other children with similar low scores on the HFMS had normal scores on the questionnaires. Our results suggest that sleep-related behaviors are frequent in type II SMA and they are mainly related to hyperhydrosis. [Copyright &y& Elsevier]