Evaluation of grip strength and range of motion of the upper limbs in patients with mucopolysaccharidosis VI

Abstract

Objective: The present study aims to evaluate the grip strength and range of motion of the upper limbs of patients with Mucopolysaccharidosis VI in order to observe how these factors can affect and correlate with the activities of daily living. Methods: The sample consisted of 13 patients: 8 males and 5 females, with a mean age of 17.76 years, mean weight of 31.30Kg, and a mean height of 1.17m, who agreed to participate and who met the inclusion criteria. The study was conducted in the state of Pernambuco at the Inborn Errors of Metabolism Treatment Center (Centro de Tratamento de Erros Inatos do Metabolismo), located at the Integrative Medicine Institute (Instituto de Medicina Integral) Prof. Fernando Figueira (IMIP). Range of motion (ROM) measurements of their upper limbs and manual grip strength were collected, and the subjects answered a structured questionnaire. Results: In this study the flexion of the shoulder joint that had the greater impairment mostly did not follow this pattern, since the average ROM for the left arm was 90.38 and 93.38 for the right. The ROM found to be the farthest below the predicted average in the study was for wrist extension in both the left and right limbs. In grip strength assessment, only one individual measured above the predicted average, 9 (69.21%) showed a degree of strength between 0 and 2 pounds (lb) in the right hand and 8 (61.52%) in the left. Conclusion: This study hopes to serve as a means of monitoring the evolution of MPS VI , which can support new studies, evaluation protocols, and motor rehabilitation.