Proksimal ve Distal Tutulumlu Nöromusküler Hastalıklarda Fonksiyonel Düzey, Fonksiyonel Kapasite ve Yaşam Kalitesinin Karşılaştırılması

Abstract

The purpose of this study is comparing functional capacities of children having a neuromusclar disease with early functional levels of proximal and distal muscle weaknesses. 40 children between the ages of 6 and 18 were included in this study where 20 of them were diagnosed with Duchenne Musclar Dysthropy (DMD) and 20 of them were diagnosed with Neuropathy in stage 1, 2, and 3 according to Brooke lower extremity functional scala. The demographic characteristics of the children were recorded. They were evaluated with respect to various characteristics including range of motion, muscle strength, muscle shortness, timed performance, balance, daily activity, life quality and physiological cost. And a 6 minutes walking test was performed. We observed that among the children with early functional levels, the ones in the distal group have high scores in terms of age, height, and body weight and their muscles were stronger excluding the distal areas (p<0.05). On the other hand, we could not observe a significant difference between the groups in terms of motion limitation range, muscle shortness, and timed performance tests. In the evaluation of balance, there were significant differences among the groups on Timed Up and Go Test results and Pediatric Functional Reach Test total score and, anterior and right reaching in sitting position parameters of this test (p<0.05). Besides, such differences have been observed on anterior and posterior postural balance measurements of stabilometric platform, and stride lengths which is one of the parameters of 6 minute walking test (p<0.05). In terms of life quality, a difference on the mother/child health score, child psycho-social health score, and school functionality score have been observed in favour of the distal group (p<0.05). Furthermore, the spatial distribution of the weakness on NMH was observed to be effective on functional capacity.