The aim of this study was to evaluate hand function and self-rated occupational performance before and after specific hand training in five participants with myotonic dystrophy (MD) type 1. MD is the commonest of the muscular dystrophies with adult onset and is associated with muscle weakness, wasting and myotonia. Compensatory techniques and strategies are mostly offered as the only intervention therapy. In the present study, the participants were their own controls using pre- and post-test measures. Each participant was tested at intervals a total of nine times before and nine times after hand training. A hand-held myometer (Microfet2), Grippit, Purdue Pegboard and self-rated myotonia were used as outcome measures. Occupational performance was assessed pre- and post-test using the Canadian Occupational Performance Measurement (COPM) in which the participants rated their own performance and satisfaction in five self-chosen occupational performance areas. The participants performed hand training for a total of three months, three times per week using a specific resistance training programme including Theraputty, a silicone-based putty. Hand function was significantly increased and a positive change in self-rated occupational performance was noted after the training period. This study indicates that hand training has a positive effect on hand function, i.e. motor function and occupational performance. The present results need to be replicated in a controlled study including a larger number of patients and over a longer period of time.

Aims Welander distal myopathy (WDM) and myotonic dystrophy type 1 (DM1) are conditions characterized by gradually impaired hand function. Measurement of hand function is therefore important in therapy for patients. However, to date reliability of common hand function test instruments for these patients has not been evaluated. The aim of the study was to test intra-rater reliability (test-retest) of the hand function testing instruments: the Grippit®, Grip Ability Test (GAT), hand-held myometer (Microfet2TM) and Purdue Pegboard in patients with WDM and DM1. In addition, inter-rater reliability of these instruments was tested in DM1 patients. Methods For test-retest, the two patient groups (16 patients in each group) were tested on 2 consecutive weeks, on the same day of the week and at the same time of day. During the second week, the DM1 patients were randomly tested either before or after retest for inter-rater reliability. Results Mainly good–very good (intra-class correlation coefficient≥0.61) intra-rater and inter-rater reliability were found in three out of four instruments tested: the Purdue Pegboard, Grippit and the hand-held myometer for both WDM and DM1 patients. Conclusions The instruments are thus considered reliable for evaluating hand function in patients with WDM and DM1. However, the GAT and pinch grip measured with the Grippit® need to be interpreted with caution, as the reliability of these instruments varied from fair to good.

Myotonic dystrophy type 1 (DM1) is a slowly progressive neuromuscular disease characterised by myotonia and muscle weakness and wasting. People with DM1 are often concerned about their ability to carry out ADL and to participate in, e.g. work, sports and hobbies when they gradually become weaker. This pilot study showed that a hand-training programme improved wrist flexor force and self-perception and satisfaction of occupational performance. Resistance training of hand muscles with a silicon-based putty can be a therapy option for people with DM1 in clinical practise.