[Physical medical rehabilitation of patients with dystrophinopathies: dynamics of the disease's course considering clinical anthropometric indicators].

Abstract

Dystrophinopathies - a group of hereditary X-linked neuromuscular diseases characterized by worsening fibrofatty degeneration of skeletal muscles, muscular weakness, low exercise tolerance, as well as orthopedic, cardiovascular and respiratory complications. Study of the effectiveness of physical medical rehabilitation in patients with neuromuscular diseases, evaluation of the influence of external and internal factors on functional capabilities and effectiveness of the conducted rehabilitation are highly relevant.

Objective: To evaluate the effectiveness of physical medical rehabilitation of patients with dystrophinopathies at the outpatient stages of the disease's course and the influence of anthropometric characteristics and functional status of patients' motor capabilities.

Material and methods: The study included 47 patients with genetically confirmed Duchenne muscular dystrophy (DMD) (the 1st group - 32 patients at an early outpatient stage, mean age 7.0±0.2 years; the 2nd group - 15 patients at a late outpatient stage, mean age 9.0±0.2 years) and 17 patients with Becker muscular dystrophy (BMD) (the 3rd group, mean age 12.0±1.2 years). All patients performed aerobic exercises 3 times a week, the duration of rehabilitation course was 4 months. The distance of 6-minute walk test (6MWT) with fatigue assessment on the visual analogue scale, the speed of rising from the floor with assessment of the functional class on a 6-point scale, the functional capacities on the Motor Function Measure (MFM) scale, dimension D1 (standing and transfers) were evaluated at baseline and after 2 and 4 months.

Results: Positive dynamics has been revealed in the form of elongating 6MWT distance, increasing speed of tests for time performance with increase of test's functional class, improvement of motor functions on the MFM scale at the end of case follow-up in the 1st and 3rd groups (p<0.01). A high correlation of the patients' motor capabilities with reduction of strength and speed indicators, moderate relationship with age and anthropometric indicators have been found (p<0.01).

Conclusions: Performance of aerobic exercises without weight combined with exercises on an ergocycle allows to improve motor capabilities in patients with DMD at the early stages and in patients with BMD. Exercising at the late stages of DMD can slow down the disease's progression. The dynamics of motor capabilities' indicators in regular performance of dynamic aerobic exercises are more influenced by the initial functional status, less - by age and concomitant overweight and high body mass index.