Participation and the Role of Neuropsychological Functioning in Myotonic Dystrophy Type 1.

Background: Myotonic Dystrophy type 1 (DM1) is primarily a neuromuscular disease but can also have neuropsychological consequences (i.e.cognitive, emotional and behavioural) which can influence daily living and societal participation. Not much is known about the level of participation of DM1 patients and their relatives and the factors influencing participation. This information can guide the development of rehabilitation programs and identify those at risk of long-term participation problems.

Objective: To investigate the level of participation of DM1 patients and their partners and the determinants.

Methods: Cross-sectional study using the Utrecht Scale for Evaluation of Rehabilitation-Participation. Determinants were demographic, disease related, and neuropsychological characteristics. Spearman correlations and backward multiple regression analyses were performed.

Results: Of the 66 DM1 participants, 54% reported severe fatigue and 47% apathy. Experienced participation restrictions were most prevalent in housekeeping (74%), sports (68%) and outdoor activities (55%). Participants were relatively satisfied with their level of participation, but dissatisfaction occurred mostly in housekeeping (58%), outdoor activities (55%), and sports (54%). Age and apathy were significant predictors of frequency of (R2 = 10.4) and restrictions in participation in the multivariate model (R2 = 14.4). Emotional functioning was the only significant predictor of satisfaction with participation (R2 = 23.8).

Conclusions: A considerable number of DM1 patients have Central Nervous System-related problems such as fatigue and apathy in addition to neuromuscular restrictions. Risk factors for lower participation are age, apathy, and emotional problems. Physical as well as neuropsychological rehabilitation programs are recommended.