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

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.