Validation of the Japanese Version of the Muscular Dystrophy Spine Questionnaire in Patients with Flaccid Neuromuscular Scoliosis in Japan

Abstract

Introduction: Progressive spinal deformity is frequently observed in children with flaccid-type neuromuscular disease. Reports evaluating activities of daily living (ADL) or quality of life (QOL) for those with flaccid-type neuromuscular disease with spine deformity (fNMS) are limited. In Japan, there is no standard patient-based questionnaire in Japanese to evaluate ADL or QOL for fNMS. We have translated the Muscular Dystrophy Spine Questionnaire (MDSQ) from English into Japanese and evaluated the reliability and validity of the Japanese version of the MDSQ.

Methods: We explained the Japanese MDSQ to patients at our regular outpatient clinic. The patients completed the questionnaire at home and mailed it to us. The questionnaire was administered twice, separated by two-week intervals, to establish reliability. We calculated the total score of the Japanese MDSQ and analyzed its correlation with whole spine X-ray parameters. We also analyzed the correlations between the 36-Item Short-Form Survey for QOL and the Japanese MDSQ scores.

Results: We analyzed results from 26 patients (average age, 13 years). Those with Duchenne muscular dystrophy (11), spinal muscular atrophy (9), and other congenital diseases were included. The averaged scoliosis Cobb angle was 67.2°, pelvic obliquity (PO) was 19°, the maximum kyphosis Cobb angle was 62.3°, and spinal pelvic obliquity (SPO) was 27.4°. The total Japanese MDSQ scores at the first and second responses were 74.9±21.3 and 75.9±20.9, respectively. The reliability of the Japanese MDSQ was excellent, with an intraclass correlation coefficient of 0.90. Total MDSQ scores correlated with scoliosis Cobb angle, PO, and SPO.

Conclusions: The Japanese MDSQ demonstrates excellent reliability and reflects the radiographic parameters of spinal and pelvic deformities in those with fNMS. It appears to be a sufficiently robust instrument for evaluating ADL in children with fNMS with spinopelvic deformity in Japan.