Decreased and Improved Movement Abilities in a Case of Myotonic Dystrophy Type 1: Examining Longitudinal Characteristics Based on Repeated Evaluations

TL;DR

This case study examines how muscle strength and movement abilities change over time in a DM1 patient through 44 repeated evaluations over two years.

Contribution

The study provides detailed longitudinal insights into DM1 progression using frequent, repeated assessments in a single patient.

Findings

Right knee extensor muscle strength decreased significantly after a fall, indicating disuse syndrome.

Walking speed decreased, but timed up-and-go test scores improved, showing compensatory movement strategies.

Walking endurance was maintained despite muscle weakness, highlighting functional compensation.

Abstract

Several large longitudinal studies on myotonic dystrophy type 1 (DM1) patients have revealed that proximal muscles show more gradual muscle weakness than distal muscles and that the progression of muscle weakness might differ between the sexes. However, these longitudinal studies were based on two follow-up time points. The present report aimed to verify the longitudinal characteristics of muscle strength and various movement abilities in a case of DM1 by examining the results of 44 repeated evaluations for approximately two years. A 40-year-old male patient with DM1 could walk independently without any aid. We recorded the longitudinal changes in his muscle strength and movement ability during outpatient rehabilitation. During follow-up, he had a fall and was diagnosed with a right ankle sprain. To evaluate the effects of the fall, we examined his recorded data. He had a significant decrease in right knee extensor muscle strength after the fall, suggesting muscle weakness due to disuse syndrome. Although his right knee extensor muscle strength and walking speed decreased, the timed up-and-go test score was improved, and walking endurance in the 2-minute walk test was maintained. In the present case, there were some motor tasks in which the movement ability was maintained or improved, likely due to the use of compensation by residual function, even when muscle weakness was present. Regular and repeated evaluations of patients with DM1 lead to reveal longitudinal characteristics of their dysfunction and movement ability.

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