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Muscle Nerve. 2016 Nov;54(5):850-855. doi: 10.1002/mus.25148. Epub 2016 Sep 21.

Correlates of health related quality of life in adult patients with spinal muscular atrophy.

Author information

1
Departments of Rehabilitation, Nursing Science and Sports, Brain Centre Rudolf Magnus, University Medical Centre Utrecht, Postbus 85500, 3508 GA, Utrecht, The Netherlands. e.t.kruitwagen@umcutrecht.nl.
2
Department of Neurology and Neurosurgery, Brain Centre Rudolf Magnus, University Medical Centre Utrecht, Utrecht, The Netherlands.
3
Departments of Rehabilitation, Nursing Science and Sports, Brain Centre Rudolf Magnus, University Medical Centre Utrecht, Postbus 85500, 3508 GA, Utrecht, The Netherlands.

Abstract

INTRODUCTION:

To improve care for patients with spinal muscular atrophy (SMA), we assessed the physical and mental quality of life (QoL) in 62 adult patients with SMA.

METHODS:

Physical component scores (PCS) and mental component scores (MCS) of the Short Form-36 Health Survey (SF-36) were obtained. Correlations with demographics, disease severity, and emotional distress were assessed. We used hierarchical multiple regression analysis to identify determinants of QoL.

RESULTS:

PCS scores were lower, and MCS scores higher than in the healthy reference population. Patients with milder SMA types reported lower scores on several MCS domains. Motor skills scores and emotional distress explained 16% of the variance in PCS. SMA type and emotional distress explained 10% and 45% of the variance of MCS, respectively.

CONCLUSIONS:

Patients with milder forms of SMA tend to have a reduced mental QoL. Psychological interventions to reduce emotional distress may improve both mental and physical QoL. Muscle Nerve 54: 850-855, 2016.

KEYWORDS:

SF-36; SMA; adults; neuromuscular diseases; perceived health; quality of life

PMID:
27074445
DOI:
10.1002/mus.25148
[Indexed for MEDLINE]

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