Functioning and disability in adults with myotonic dystrophy type 1.
OBJECTIVE
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.


CONCLUSIONS
This information can be used for developing clinical practise and for health promotion for persons with DM1.
Division of Physiotherapy, Department of Neurobiology, Care Sciences and Society, Karolinska Institutet, Stockholm, Sweden. marie.kierkegaard@karolinska.se
Marie
M
Kierkegaard
Karin
K
Harms-Ringdahl
Lotta Widén
LW
Holmqvist
Anna
A
Tollbäck
D000203:Activities of Daily Living; D000368:Aged; D000369:Aged, 80 and over; D003430:Cross-Sectional Studies; D004185:Disability Evaluation; D006233:Persons with Disabilities / Q000534:rehabilitation / Q000706:statistics & numerical data; D018450:Disease Progression; D005260:Female; D006801:Humans; D008019:Life Style; D008297:Male; D008875:Middle Aged; D009132:Muscles / Q000503:physiopathology*; D009223:Myotonic Dystrophy / Q000145:classification / Q000503:physiopathology* / Q000534:rehabilitation*; D011788:Quality of Life; D012720:Severity of Illness Index; D058992:Social Participation; D011795:Surveys and Questionnaires; D013548:Sweden; D016138:Walking / Q000502:physiology