Abstract
The aim of this study was to investigate and compare the performance exposure due to different muscle weakness involvement in proximally and distally affected neuromuscular diseases (NMD's). A total of 40 pediatric patients with different NMD's who were grouped based on proximal (Group 1) and distal (Group 2) muscle weakness involvement were included in the study. The children between Grade 1 and 3 according to Brooke Lower Extremity Functional Classification (BLEFC) were taken under assessments. Lower extremity regional and total muscular strengths were assessed with manual muscle testing. 10m walking time (s) and 6 Minute Walk Test (6MWT) (m) were used to evaluate performance level. The relationship between muscle weakness and performance levels was analyzed. 20 patients with proximal (Duchenne Muscular Dystrophy-DMD) and 20 with distal muscle weakness (Hereditary Sensory Motor Neuropathy) who were respectively 9±3.1 and 12.9±3.3years old were included in the study. Lower extremity total muscle strengths were statistically different with 81.5±18.7 in group 1 and 98.7±16.5 in group 2 ( z =−2.764; p z =−0.122) and 6MWT distances ( z =−0.609) between two groups ( p >0.05). There were positive correlations between lower extremity muscles strength scores except for ankle girdle and 6MWT distance ( p p p >0.05). Muscle weakness distribution is an important performance determinative in NMD's. Performance is more effected in proximal muscle weakness involvement than distal at early stages of the disease.
Published Version
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