S.P.16 Egen classification scale (Ek) for Duchenne Muscular Dystrophy and Spinal Muscular Atrophy: Validity and reliability study of the Turkish version

Abstract

<h2>Abstract</h2> EK scale was developed to assess detailed functional ability of non-ambulatory Duchenne Muscular Dystrophy (DMD) patients who have severe physical impairments by Steffensen et al. in 2002. EK total score was found to distinguish the different functional levels and show the prognosis of the nonambulatory DMD and Spinal Muscular Atrophy (SMA) patients. The aim of this study was to make the cultural adaptation and investigate the validity and intra/inter-tester reliability of Turkish Version of EK scale on non-ambulatory DMD and SMA patients The original English version of EK scale was translated into Turkish and necessary arrangements were made after the consensus by the researchers. After the arrangements and cultural adaptation; the pre-test study was made on five patients, inter-tester and intra-tester reliability studies were made on totally 34 patients. Brooke Functional Classification Scale for Upper and Lower Extremities (BFC), Barthel's Index for Activities of Daily Living and Pulmonary Dysfunction Test (PDT) were used as gold standards to investigate the validity of the scale in Turkish DMD and SMA population. 11 SMA and 23 DMD patients (Mean age: 13.71±4.25) with non-ambulatory functional status were included in the study. Intra-tester reliability of the EK scale was 0.979 (ICC) with % 95 Confidence Interval [0.930; 0.994] and inter-tester was 0.916 (ICC) with % 95 Confidence Interval [0.407; 0.990]. There were correlations between Turkish version of EK scale and Barthel Index (<i>r</i>=−0.716, it p<0.01) and PDT (it r=0.541, it p<0.05) and BFC for Upper Extremities (it r=0.576, it p<0.05). The Turkish version of the EK scale has excellent inter and intra-tester reliability and validity on Turkish DMM and SMA patients. EK scale is an adequate and useful instrument to assess functional status and predict the prognosis of non-ambulatory DMD and SMA patients in Turkish population.