Abstract
FSHD is one of the most frequent forms of muscular dystrophy characterized by an early involvement of facial and scapular muscles with eventual spreading to pelvic and lower limb muscles. Few studies have been dedicated to sleep disorders in FHSD. Our objective was to explore subjective sleep quality in FSHD patients. Adults with genetically proven FSHD were included in the study. They were compared to a control group of subjects with no myopathy, no past neurological disease nor instable chronic disease history. The Pittsburgh sleep quality index (PSQI), the Epworth sleepiness scale (ESS), the clinical severity scale (CSS), the fatigue severity scale and the hospital anxiety and depression scale were used to evaluate the severity of the sleep disorder, the subjective sleep quality, the prevalence of daytime sleepiness, the anxiety and the depression in patients and controls. Pulmonary function and MRC score were also assessed. Mann Withney test, Spearman correlation and Fisher test were used for the statistics analysis. 22 FSHD and 36 controls were included in the study. Both groups were similar as for age, sexe, body mass index and respiratory support. 50% of FSHD patients presented with vital capacity p p = 0.04). The latter was also correlated with depression, fatigue and bad dreams. Surprisingly, we noticed a negative correlation between ESS and CSS: the loss of activity in severe patients could result in harmony with sleep needs. FSHD patients have altered subjective sleep quality as compared with control population. Anxiety mainly accounts for this, while severity of the disease and respiratory involvement do not affect it.
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