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dc.contributor.authorJensen, Synnøve
dc.contributor.authorAbeler, Karin Louise
dc.contributor.authorFriborg, Oddgeir
dc.contributor.authorRösner, Assami
dc.contributor.authorOlsborg, Caroline
dc.contributor.authorMellgren, Svein Ivar
dc.contributor.authorMüller, Kai Ivar
dc.contributor.authorRosenberger, Andreas Dybesland
dc.contributor.authorVold, Monica Linea
dc.contributor.authorArntzen, Kjell Arne
dc.description.abstractLimb-girdle muscular dystrophy R9 (LGMDR9) is a progressive and disabling genetic muscle disease. Sleep is relevant in the patient care as it impacts on health, functioning, and well-being. LGMDR9 may potentially afect sleep by physical or emotional symptoms, myalgia, or sleep-disordered breathing (SDB) through cardiorespiratory involvement. The objective was to investigate the occurrence of insomnia and unrecognized or untreated SDB in LGMDR9, associated factors, and relationships with fatigue and health-related quality of life (HRQoL). All 90 adults in a Norwegian LGMDR9 cohort received questionnaires on sleep, fatigue, and HRQoL. Forty-nine of them underwent clinical assessments and 26 without mask-based therapy for respiration disorders additionally underwent polysomnography (PSG) and capnometry. Among 77 questionnaire respondents, 31% received mask-based therapy. The prevalence of insomnia was 32% of both those with and without such therapy but was signifcantly increased in fatigued respondents (54% vs 21%). Insomnia levels correlated inversely with mental HRQoL. Among 26 PSG candidates, an apnea–hypopnea index (AHI)≥5/h was observed in 16/26 subjects (≥15/h in 8/26) with median 6.8 obstructive apneas and 0.2 central apneas per hour of sleep. The AHI was related to advancing age and an ejection fraction<50%. Sleep-related hypoventilation was detected in one subject. Fatigue severity did not correlate with motor function or nocturnal metrics of respiration or sleep but with Maximal Inspiratory Pressure (r=− 0.46). The results indicate that insomnia and SDB are underrecognized comorbidities in LGMDR9 and associated with HRQoL impairment and heart failure, respectively. We propose an increased attention to insomnia and SDB in the interdisciplinary care of LGMDR9. Insomnia and pulmonary function should be examined in fatigued patients.en_US
dc.identifier.citationJensen, Abeler, Friborg, Rösner, Olsborg, Mellgren, Müller, Rosenberger, Vold, Arntzen. Insomnia and sleep-disordered breathing in FKRP-related limb-girdle muscular dystrophy R9. The Norwegian LGMDR9 cohort study (2020). Journal of Neurology. 2023en_US
dc.identifier.cristinIDFRIDAID 2182499
dc.publisherSpringer Natureen_US
dc.relation.journalJournal of Neurology
dc.rights.holderCopyright 2023 The Author(s)en_US
dc.rightsAttribution 4.0 International (CC BY 4.0)en_US
dc.titleInsomnia and sleep-disordered breathing in FKRP-related limb-girdle muscular dystrophy R9. The Norwegian LGMDR9 cohort study (2020)en_US
dc.typeJournal articleen_US
dc.typePeer revieweden_US

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Attribution 4.0 International (CC BY 4.0)
Except where otherwise noted, this item's license is described as Attribution 4.0 International (CC BY 4.0)