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Sleep disorders in spinal muscular atrophy
Maria C Pera1, Domenico M Romeo2, Alessandra Graziano1
1Department of Paediatric Neurology, Catholic University, Rome, Italy; Nemo Roma Center for Neuromuscular Disorders, Rome, Italy.
Sleep Medicine
|February 21, 2017
Summary
Sleep disorders are common in young individuals with spinal muscular atrophy (SMA). This study found that over 30% of children with SMA experienced sleep disturbances, impacting their quality of life.
Area of Science:
- Neurology
- Pediatrics
- Sleep Medicine
Background:
- Spinal muscular atrophy (SMA) is a rare genetic disorder affecting motor neurons.
- Sleep disturbances are frequently reported in pediatric populations but understudied in SMA.
Purpose of the Study:
- To determine the prevalence of sleep disorders in young patients with type 2 and type 3 SMA.
- To investigate correlations between sleep issues and factors like motor function, age, and assistive device use.
Main Methods:
- Eighty-five SMA patients (ages 6-25) were evaluated.
- Utilized the Sleep Disturbance Scale for Children (SDSC) and Hammersmith Functional Motor Scale Expanded (HFMSE).
Main Results:
- 16.4% of SMA patients had abnormal total sleep scores; 16.7% had abnormal scores on specific sleep factors.
- No overall correlation between sleep disturbances and general motor function (HFMSE total score).
- Specific motor abilities, like rolling over, were significantly associated with sleep disturbance severity.
Conclusions:
- Sleep disorders are a prevalent issue in children diagnosed with SMA.
- Targeted assessment of specific sleep factors may be more informative than general scores.
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