Transcultural adaptation and inter-examiner reliability of upper extremity assessment scale - pul for children and adolescents with Duchenne Muscular Dystrophy and Charcot-Marie-Tooth Disease

• Grant number: 20/13380-0
• Support Opportunities: Scholarships in Brazil - Scientific Initiation
• Start date: July 01, 2021
• End date: June 30, 2022
• Field of knowledge: Health Sciences - Physiotherapy and Occupational Therapy
• Principal Investigator: Ana Cláudia Mattiello-Sverzut
• Grantee: Giovanna Constantin Silva
• Host Institution: Faculdade de Medicina de Ribeirão Preto (FMRP). Universidade de São Paulo (USP). Ribeirão Preto , SP, Brazil

Abstract

Neuromuscular diseases, such as Duchenne Muscular Dystrophy (DMD) and Charcot Marie-Tooth disease (CMT), can impair the functionality of upper limbs. This fact compromises the patient’s functionality and independence in activities of daily living, reducing the quality of life. Specific tools that evaluate the functionality of the upper limbs of this group, allows health professionals to monitor patients in all stages of the disease and assist in the therapeutic strategies to maintain the functionality and autonomy as much as possible. As there are no translated tools and reliable data available in Brazilian Portuguese to assess the functionality of the upper limbs of these patients, this study aims to perform the cross-cultural adaptation to Brazilian Portuguese of Performance of Upper Limbs (PUL) scale for children and adolescents with DMD, verify their applicability in patients with CMT and evaluate the construct validity and inter-examiner reliability of PUL in patients with DMD and CMT. The cross-cultural adaptation will follow the steps: initial translation into Brazilian Portuguese, translation synthesis, back-translation, back-translation synthesis, expert committee and pre-final version test. The pre-final version of the PUL will be applied in 24 patients (DMD = 12 and CMT = 12), followed up at the Centro de Reabilitação do Hospital das Clínicas da Faculdade de Medicina de Ribeirão Preto Universidade de São Paulo (CER-HC-FMRPUSP). For assessment of construct validity and reliability, the final version of the PUL will be applied in 40 patients (DMD = 20 and CMT = 20). Descriptive analysis (mean-SD) and inferential data. Spearman's correlation coefficient will be used to ascertain the validity of the PUL construct and the Intraclass Correlation Coefficient will assess the reliability of the scale.