Myotonic Dystrophy, PrOteiN and Diet study. Part 1.

Recruitment & Eligibility

Status: Completed

Sex: Not specified

Target Recruitment: 30

Inclusion Criteria

DM1 affected subjects:
- Age 18 years or older.
- Legally competent adult.
- Defined DM1 of the adult subtype.
- Participants must be able to walk and to cycle (in order to perform exercise
tests).
- Participants must give informed consent by signing and dating an informed
consent form. Healthy control group:
- Age 18 years or older.
- Legally competent adult.

Exclusion Criteria

- Implantation of pacemaker or ICD device, a implantable insulin device, a
neurostimulator, internal hearing aid or artificial heart valve.
- Implantation of orthopaedic prostheses, screws or plates.
- Metal shreds or splinters inside the body.
- Vascular clips in the body.
- Big tattoo*s and/or permanent make-up.
- Claustrophobia.
- Use of medication interacting with muscle metabolism (such as steroids and
statins).
- Diabetes mellitus.
- Weight loss of more than 3 kg in the last three months.
- Pregnant or lactating women.
- Use of protein supplements.
- Participation in an exercise program.
- Patients who are not able to perform basic activities of daily living such as
walking or patients who are suffering from other disabling comorbidity that
seriously hamper physical exercise (e.g. heart failure, coronary artery
disease, chronic obstructive pulmonary disease (COPD), orthopedic conditions).
- Body mass index (BMI) <18 or >35.
- Use of oral anticoagulants.
- In case of DM1 affected subjects: muscular impairment rating scale (MIRS)
score of 5 (which represents severe proximal muscle weakness).
- In case of the healthy control group: presence of a neuromuscular disorder or
an abnormal neurological examination (specifically if muscle weakness is
present), or other condition possibly interfering with muscle strength or
muscle mass.

Study & Design

Study Type: Observational invasive

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
<p>The main study endpoints are total energy expenditure (TEE), resting metabolic<br /><br>rate (RMR) and substrate selection at rest.</p><br>

Secondary Outcome Measures

Name	Time	Method
<p>Secondary endpoints include physical activity level (PAL) measured by<br /><br>accelerometer, whole-body skeletal muscle mass measured by MRI, quadriceps<br /><br>muscle cross-sectional area measured by CT, maximal grip strength measured by<br /><br>dynamometer, body composition expressed as leg and whole-body lean tissue mass<br /><br>measured by DEXA, muscle tissue morphology, muscle fibre differentiation,<br /><br>muscle fibre type specific cross-sectional area and vascularization, muscle<br /><br>fiber biochemical analysis, maximal oxygen uptake capacity (VO2 max) and<br /><br>aerobic and anaerobic ventilatory thresholds determined by cycling exercise<br /><br>test and muscle strength measured by1-RM (only applicable for DM1 affected<br /><br>subjects taking part in the training program pilot). </p><br>