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Lookup NU author(s): Dr Kirstie Anderson
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Angelman syndrome is a neurodevelopmental disorder characterized by severe learning difficulties, epilepsy, and a typical behavioral phenotype. The diagnostic criteria state that 20-80% of individuals have decreased sleep need and abnormal sleep-wake cycles. A wide variety of sleep problems have been reported, including reduced total sleep time, frequent night awakenings and nocturnal enuresis. Most previous reports have used sleep questionnaires to assess the frequency of various sleep disorders. Most patients studied have been children or adolescents and only one previous study has used complex sleep studies (polysomnography). We report three adult sisters with Angelman's syndrome who have been assessed with sleep diaries, actigraphy and, in one case, overnight polysomnography. Despite sleep diaries showing prolonged sleep with a mean of 9 h a night with few nocturnal arousals, the actigraphy in all three patients showed increased sleep fragmentation and in one the polysomnography was strikingly abnormal, with a greatly reduced total sleep time and very frequent, predominantly obstructive sleep apneas fragmenting night sleep (desaturation index 63.3 of total sleep time). Despite over 10 h in bed, she had only 69.5 min of actual sleep. There was no evidence of a circadian rhythm disorder. This is the first report of polysomnography in an adult with Angelman syndrome and it highlights the need to look for the presence of sleep apnea in this group, which may be an under-recognized cause of nocturnal sleep disturbance.
Author(s): Anderson KN, Pilsworth S, Jamieson S, Ray J, Shneerson JM, Lennox GG
Publication type: Article
Publication status: Published
Journal: Sleep and Biological Rhythms
ISSN (print): 1446-9235
ISSN (electronic): 1479-8425
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