Clinical and neuroradiological correlates of sleep in myotonic dystrophy type 1.

Abnormalities of sleep are common in myotonic dystrophy type 1 (DM1), but few previous studies have combined polysomnography with detailed clinical measures and brain imaging. In the present study, domiciliary polysomnography, symptom questionnaires and cognitive evaluation were undertaken in 39 DM1-affected individuals. Structural brain MRI was completed in those without contra-indication (n = 32). Polysomnograms were adequate for analysis in 36 participants. Sleep efficiency was reduced, and sleep architecture altered in keeping with previous studies. Twenty participants (56%) had moderate or severe sleep-disordered breathing (apnoea-hypopnoea index [AHI] ≥ 15). In linear modelling, apnoeas were positively associated with increasing age and male sex. AHI ≥ 15 was further associated with greater daytime pCO

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Déclaration de conflit d'intérêts

Conflicts of interest Within the last five years Professor Monckton has been a scientific consultant and/or received an honoraria/stock options/grants from AMO Pharma, Charles River, LoQus23, Small Molecule RNA, Triplet Therapeutics and Vertex Pharmaceuticals. Professor Monckton also had research contracts with AMO Pharma and Vertex Pharmaceuticals. Professor Monckton has received research grants/contracts from the European Union, CHDI, European Huntington Disease Network, Huntington Disease Society of America, National Institute of Health, Muscular Dystrophy UK and the Myotonic Dystrophy Support Group. Professor Monckton is on the Scientific Advisory Board of Myotonic (formerly the Myotonic Dystrophy Foundation) and EuroDyMA (European Dystrophia Myotonica Association), is a scientific advisor to the Myotonic Dystrophy Support Group and is a vice president of Muscular Dystrophy UK.; The remaining authors have no conflicts to declare.