Abstract
BACKGROUND AND OBJECTIVES: Few studies have investigated sleep features in people with alternating hemiplegia of childhood (AHC). In this study, we present a bespoke survey of individuals with AHC to characterize sleep disturbances in this condition. METHODS: A cross-sectional survey was disseminated through the UK and Italian AHC family associations, addressing their registered families with at least 1 child or adult with a confirmed diagnosis of AHC by their national reference centers and who had consented to be contacted by email. The entire questionnaire implemented in the online surveys was organized into 3 main sections and included the Pittsburgh Sleep Quality Index (PSQI) scale. This scale ranges from 0 to 21, with higher scores indicating poorer sleep quality. Internal consistency of the questionnaire was assessed using Cronbach alpha statistic. RESULTS: Forty-eight of 54 families with AHC (88.89%) filled out at least 1 item of the AHC-Sleep Questionnaire, and 36 of 54 (66.67%) completed the whole questionnaire. Eighteen of 44 people (40.91%) from our cohort reported having a problem with sleep, which was persistent for 10 of 44 (22.7%). Twenty-five of 44 people (56.82%) reported multiple arousals during a typical night, and for 9 of 25 (36%), these could be very prolonged. For 16 of 40 (40%) and 19 of 40 (47.5%), plegic spells resolved or improved, respectively, during sleep, whereas 5 of 40 (12.5%) reported no effect of sleep on these spells. The mean global PSQI score in 40 people was 5.40 (SD ± 4.69). Fifteen of 40 individuals (37.5%) had a global PSQI score ≥5. PSQI scores were significantly higher in those individuals who completed the questionnaire during summer (n = 6) compared with those who completed them during winter (n = 33) (p = 0.011, Mann-Whitney test). We found a strong correlation between AHC paroxysmal spell burden and PSQI score (Spearman r = 0.39, p < 0.01). The survey demonstrated a high degree of internal consistency, with a Cronbach alpha of 0.88 (95% CI 0.83-0.91). DISCUSSION: We dissected sleep issues and reported sleep disruption, including elevated global PSQI scores, in a significant proportion of individuals with AHC. The main implication of this study is the importance of conducting comprehensive and regular assessments of sleep in both children and adults with AHC.