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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.

|February 21, 2017

Abstract

Objective:

To estimate the frequency of sleep disorders in young persons with type 2 and type 3 spinal muscular atrophy (SMA), and to evaluate the relationship between sleep disorders and different variables such as motor impairment, age, use of ventilation, and use of night orthoses.

Methods:

A total of 85 young persons (6-25 years of age) with type 2 and type 3 SMA were assessed using the Sleep Disturbance Scale for Children (SDSC), a scale assessing different sleep factors, and the Hammersmith Functional Motor Scale Expanded (HFMSE), a scale evaluating motor impairment.

Results:

An abnormal total sleep score was found in 16.4% of children with SMA; an additional 16.7% had an abnormal score on at least one of the sleep factors assessed by the SDSC. No specific correlation was observed between sleep disturbances and functional level as expressed by the SDSC and total HFMSE scores, but the relationship with individual items on the scale was different. The SDSC total score was significantly associated with the ability to half roll on both sides and to roll from prone to supine on the HMFSE.

Conclusion:

Our results demonstrate that sleep disorders are common in children with SMA.

Keywords:

HFMSE Motor function SDSC Sleep Spinal muscular atrophy

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