Sleep quality, restless legs syndrome and daytime sleepiness in adults with 5q-spinal muscular atrophy

This is the authors' abstract. We don't add key points for this paper.

BackgroundThis study aimed to investigate sleep quality, restless legs syndrome (RLS), and excessive daytime sleepiness in adults with spinal muscular atrophy (SMA) and their relationships with motor function, quality of life (QoL), fatigue, and depression.MethodsWe included 43 adults with SMA (31 non-ambulatory; 11 using non-invasive ventilation) and 43 age- and sex-matched healthy controls (HC). Subjective sleep quality and daytime sleepiness were assessed using the Pittsburgh Sleep Quality Index (PSQI) and the Epworth Sleepiness Scale (ESS). RLS was diagnosed using the International RLS Study Group diagnostic criteria. Revised Upper Limb Module (RULM), Hammersmith Functional Motor Scale Expanded (HFMSE) and respiratory function, as well as, 36-Item Short Form Health Survey (SF-36), Beck Depression Inventory (BDI) and Fatigue Severity Scale (FSS) were recorded.ResultsSMA patients had significantly worse scores in sleep efficiency domain of the PSQI (0.7 ± 1.0 vs. 0.3 ± 0.6, p = 0.04) as well as in global PSQI score compared to HC (5.5 ± 3.5 vs. 4.0 ± 2.6, p = 0.029). Patients classified as poor sleepers (PSQI > 5) had a higher body mass index (BMI), greater BDI, and ESS scores. No association between global PSQI score and HFMSE or RULM score was observed. Patients classified as poor sleepers had lower total SF-36 score in comparison to good sleepers (53.2 ± 15.4 vs. 69.6 ± 12.3, p < 0.001). RLS was present in five SMA patients (11.6%).ConclusionPoor sleep quality is common in adults with SMA. It contributes to a lower QoL and should be addressed as a part of the standard of care in adults with SMA.