Abstract
Recent studies on Myotonic dystrophy type 1 (DM1) have shown profound impairments in gait, leading to falls. We analyzed functional ambulation profile (FAP) score that reflects the temporal and spatial gait characteristics and investigated correlations with the lower limb muscle magnetic resonance imaging (MRI) and 6 min walk test (6MWT). Twenty patients with DM1 and 20 controls participated in this study. The 6MWT and gait analysis including FAP scores via GAITRite were performed in all patients and controls. DM1 patients displayed slower gait, shorter stride length, shorter stance length, and lower FAP score. Among lower extremity muscles, the gastrocnemius, soleus and tibialis anterior showed the most severe fat infiltration and these crural muscles significantly correlated with FAP and 6MWT. Among crural muscles, tibialis anterior was the most important muscle affecting gait speed, whereas the gastrocnemius contributed substantially to gait instability. FAP score correlated with the muscle imaging and 6MWT in DM1. Therefore, FAP score maybe used as an non-invasive marker that reflects deterioration of gait and a possible surrogate biomarker in DM1.
Highlights
Myotonic dystrophy type I (DM1; Steinert disease; OMIM160900) is the most common form of adult onset muscular dystrophy
Among the parameters in GAITRite, we focused on the functional ambulation profile (FAP) score that represent the quantitative spatiotemporal gait parameters that encompasses gait speed and distance that evaluates fall risks and gait balance as well
We investigated the potential relationship between gait parameters with emphasis on FAP score with these two biomarkers
Summary
Myotonic dystrophy type I (DM1; Steinert disease; OMIM160900) is the most common form of adult onset muscular dystrophy. DM1 is characterized by myotonia and pronounced distal weakness in both upper and lower limbs [1]. A limited number of studies have described gait impairments in patients with DM1, and different technical approaches were used by each study in small samples of patients [5,6,7]. These studies using 3D motion analysis with electromyography and infrared-emitting diodes have indicated an important contribution of distal lower limb muscle weakness to gait impairments in DM1. Muscle strength was not quantitatively assessed and it did not show weakness until moderate muscle degeneration. Recent studies have shown the usefulness of muscle magnetic resonance imaging in identifying patterns of muscle involvement
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