Article

PURPOSE: To provide a comprehensive description of functioning and disability
with regard to stages of disease progression in adults with myotonic dystrophy
type 1 (DM1). Further to explore associations of measures of manual dexterity and
of walking capacity with measures of activities of daily living (ADL) and
participation in social and lifestyle activities. METHODS: Seventy persons with
DM1 underwent examinations, tests and answered questionnaires. Stages of disease
progression were based on the muscular impairment rating scale. RESULTS:
Overweight, cardiac dysfunctions, respiratory restrictions, fatigue and/or low
physical activity levels were found in approximately 40% of those with DM1. Over
75% had muscle impairments, and activity limitations in manual dexterity and
walking. Dependence in personal and instrumental ADL was found in 16% and 39%,
respectively, and participation restrictions in social and lifestyle activities
in 52%. The presence of concurrent body-function impairments, activity
limitations and participation restrictions was high. Significant differences were
found in muscle impairment, manual dexterity, mobility, ADL and social and
lifestyle activities with regard to disease progression. Cut-off values in
measures of manual dexterity and walking capacity associated to functioning are
proposed. CONCLUSION: This information can be used for developing clinical
practise and for health promotion for persons with DM1.

Langue : ANGLAIS