Joint range of motion limitations in children and young adults with spinal muscular atrophy

Abstract

Abstract Wang HY, Ju YH, Chen SM, Lo SK, Jong YJ. Joint range of motion limitations in children and young adults with spinal muscular atrophy. Objectives To elicit descriptive data about limited joint range of motion (ROM) in subjects with type II or III spinal muscular atrophy (SMA) and to examine the relation between the number of motions with limited range and both age and functional ability. Design Descriptive cross-sectional study. Setting Neurologic pediatric outpatient clinic at a hospital in Taiwan. Participants Twenty-seven subjects with SMA type II (mean age, 9.8±6.5y) and 17 with SMA type III (mean age, 12.2±8.7y). Intervention Measurement with transparent goniometers of joint ROM bilaterally of the shoulder, elbow, wrist, hip, knee, and ankle. Main outcome measures The proportion of participants with each ROM limitation compared with all participants with the same SMA type, age distribution of the participants with each ROM limitation, mean range loss of each motion limitation, and the contracture index (risk index of joint contracture). Results Eighty-nine percent of the participants with SMA type II experienced knee extension limitation. Approximately 50% of the participants with both types of SMA had ankle dorsiflexion limitation. The motions of knee and hip extension and ankle dorsiflexion also had a relatively high contracture index. The number of motions with limited range positively correlated ( P<.001) with age and upper-extremity functional grade (the higher the functional grade, the poorer the functional ability) for SMA type II. Conclusions We found varying degrees of joint ROM limitation. Certain motions were noted to be high risks for the development of contractures. This risk was higher mostly in younger children.