Rehabilitation technology in assessment and treatment of arm and hand function in myotonic dystrophy type 1: A single subject experimental design study

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Background: Myotonic Dystrophy type 1 (DM1) is a slowly progressive hereditary neuromuscular disorder affecting several organ systems including the musculoskeletal system. Objective: To examine the effects of rehabilitation technology on arm and hand function in the assessment and treatment of adults with DM1 with moderate to severe muscle impairment. Methods: A single subject experimental design study (SSED) with 6 participants. The assessments were done weekly through remote study assessments using the video-conference system Whereby®. Participants performed the nine-hole peg test (NHPT), active range of motion (AROM) of the shoulder and patient reported outcome measures (PROMS). Three participants also measured grip and pinch strength and performed the Nut and Bolt task. The intervention was a three-week in-patient rehabilitation stay with the use of rehabilitation technology for exercising arm and hand function. Exercises were performed in AMADEO and the ArmeoSenso. The participants exercised the upper extremities for 30 min 5 times a week. Results: Improvement on the NHPT test on the dominant hand were found for five of the six participants. Three of six improved on the non-dominant hand and in AROM. Two of the three participants that measured pinch and grip strength improved, and all three improved on the Nut and Bolt task. Self- reported function, myotonia and fatigue remained stable. All participants were able to participate in video assessments. Conclusions: Impairment in arm- and hand function affects independence in people with DM1.Our findings suggest that exercising arm- and hand using rehabilitation technology may have a positive effect on function measured by dexterity, strength, and movement in DM1. There is a need to study the effect of exercise on arm- and hand function in DM1 in larger studies. The use of video-consultations can be a supplement in assessment of people with neuromuscular conditions in research and clinical practise.