An exercise regimen (PRIME: Proximal Resistance In-House Movement Exercise) has been designed for patients with myotonic dystrophy type 2 (DM2). The hypothesis is that this patient-friendly physical therapist (PT)-guided exercise program associates with improved functional capacity and muscle composition in DM2 in this two-period two-sequence cross-over study. Thus, participant will be randomized to one of the three possible groups. Participants in GROUP A will perform exercise routine in clinic under the direct supervision of a physical therapist twice a week for the first three months, then they will continue with same exercise routine at home for the last 3 months on their own. Participants in GROUP B will perform exercise routine virtually under the direct supervision of a physical therapist twice a week for the first three months, then they will continue with same exercise routine at home for the last 3 months on their own. Participants in GROUP C will perform exercise routine on their own during the first 3 months, then they will perform exercise routine virtually under the direct supervision of a physical therapist. Each group will include around 8 participants. Duration of the study is 6 months. In addition to exercise sessions, participants will have evaluation of their strength, motor function and muscle composition at three time points: initiation, 3 months and completion of the study at 6 months. Muscle composition will be assessed by electrical impedance myography which is a portable, non-invasive, painless and non-radiation tool that applies a weak high multifrequency electrical current to the examined muscle and allows to obtain information about its composition.
Study Type
INTERVENTIONAL
Allocation
RANDOMIZED
Purpose
TREATMENT
Masking
NONE
Enrollment
24
A patient-friendly exercise routine has been designed for patients with myotonic dystrophy type 2. Benefits of this exercise regimen in different settings (at home versus clinic, self-directed versus physical therapist-directed) will be investigated. In person exercise supervised by physical therapist and then self-directed exercise in Group A
Exercise virtually supervised by physical therapist and then self-directed for group B
Self-directed and virtually supervised exercise by physical therapist for Group C
Neuromuscular Diagnostic Center. Massachusetts General Hospital
Boston, Massachusetts, United States
RECRUITINGManual Muscle Test (MMT)
Score of manual muscle strength test scale. This is a strength exam at bedside of muscle groups
Time frame: From enrollment to the end of treatment at 6 months
Six-minute walking test
Distance in meters that subject can walk in six minutes
Time frame: From enrollment to the end of treatment at 6 months
Quantitative myometry
Muscle dynamometry of different muscle groups measured in kilograms
Time frame: From enrollment to the end of treatment at 6 months
Muscle resistance/reactance measures in ohms
using electrical impedance myography, resistance and reactance measures in ohms will be obtained simultaneously from each examined muscle
Time frame: From enrollment to the end of treatment at 6 months
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