Sleep-related problems and sleep disorders in ataxia telangiectasia

Abstract

Objective: Ataxia telangiectasia (AT) is a rare neurodegenerative disorder and sleep-related problems may be expected. We investigated sleep-related problems in children with AT in relation to their clinical status.

Methods: We administered Children's Sleep Habits Questionnaire and Sleep Disturbance Scale for Children to patients with AT (n = 46) and a matched healthy control (HC) (n = 92). We defined clinical subgroups of AT as mild (n = 10) and moderate/severe (n = 36) according to patients' clinical scores. We compared the results between the groups.

Results: The median age was 130.5 (104.8-175.0) months in AT patients and 125.5 (85.0-177.0) months in HC. The frequency (6.5 % in AT, 0 % in HC), risk of sleep disturbance (60 % in AT, 25 % in HC), and sleep problems (80 % in AT, 50 % in HC) were higher in AT than in HC. As the male/female ratio differed between AT and HC in our study, we applied further analyses adjusting for gender and age; AT patients were almost 4.5 times more likely to develop sleep disturbances and 6 times more likely to have sleep problems than HC. Sleep-related problems decreased with increasing age in HC; sleep problems partially decreased in AT, but sleep disturbances did not decrease in AT.

Conclusion: Sleep architecture, behavior, and habits may be disturbed in AT, justifying the inclusion of sleep screening tools in the clinical follow-up of these patients even if no symptoms are reported in the medical history. Further clinical studies in large cohorts are needed to develop sleep screening tools specific to AT and similar neurodegenerative diseases.

Keywords: Ataxia-telangiectasia; Cerebellum; Child; sleep disturbance; sleep habits; sleep problems.