The Effect of Trunk Brace Use on Motor and Pulmonary Functions in a Patient with LAMA2-Related Congenital Muscular...

LAMA2-related congenital muscular dystrophy (CMD), the most common form of CMD, is characterized by very early-onset muscle weakness, trunk instability, neuromuscular scoliosis, and progressive respiratory failure. The effectiveness of trunk bracing in neuromuscular scoliosis is controversial. The aim of this study was to investigate the effects of trunk bracing on spinal alignment, motor function, and pulmonary parameters in a patient diagnosed with LAMA2-related CMD. A 6-year-old female patient with a homozygous LAMA2 deletion and 37° left thoracolumbar scoliosis was recommended to use a thoracolumbar-sacral orthosis (TLSO) while sitting. Assessments were performed at baseline, on day 20, and at week 6. Spinal asymmetry during sitting, Motor Function Measure (MFM), Expanded Hammersmith Functional Motor Scale (HFMSE), Trunk Control Measurement Scale (TCMS), and spirometry-based respiratory function tests (Forced Expiratory Volume in 1 second (FEV1), Forced Vital Capacity (FVC) were evaluated. The patient continued with a standard physical therapy program twice a week. The average daily corset usage time was 4 hours. The MFM score increased from 27 to 42 points, and the HFMSE score increased from 5 to 9 points. An increase was observed in pulmonary parameters (FEV1: 39-54%; FVC: 40-64%). TCMS scores remained stable (7-7-7 points). No side effects were reported. The use of a trunk brace may have positive effects on sitting posture, motor performance, and pulmonary function in LAMA2-related congenital muscular dystrophy.