Low-frequency Repetitive Nerve Stimulation in Myotonic Dystrophy Type 1
Myotonic Dystrophy 1
About this trial
This is an interventional basic science trial for Myotonic Dystrophy 1
Eligibility Criteria
Inclusion Criteria: * genetically confirmed DM1 Exclusion Criteria: minor age auto-immune diseases medical conditions involving the neuromuscular junction: (myasthenia gravis, Lambert-Eaton myasthenic syndrome, congenital myasthenia syndromes).
Sites / Locations
- UZ Brussel
Arms of the Study
Arm 1
Arm 2
Arm 3
Arm 4
Active Comparator
Active Comparator
Active Comparator
Active Comparator
Questionnaires
grip strength via dynamometer
short exercise test
needle EMG
Patients with DM1 were asked to complete a questionnaire to rate a 25-item activity scale (DM1-Activ) and the Myasthenia Gravis Activity of Daily Life scale (MG-ADL) to rate their level of functional burden. For the DM1-Activ a score of 40 alludes no impairment and a score of 0 indicates the highest functional burden of physical activity. This scale has proven to be practical, reliable and valid. For the MG-ADL the total score ranges from 0 to 24, a score of 0 denotes no and 24 the highest functional burden. It should be noted that this scale is not adjusted for DM1. The rationale was to gain information about muscle fatigue and consequently the neuromuscular junction.
The isometric grip strength was tested by using a dynamometer. The subject will be asked to perform an increasing force against the dynamometer over a period of several seconds.
The subject was asked to contract the ADM muscle as hard as possible in isometric conditions for 10 seconds. CMAP's was recorded 2 seconds after the end of the exercise and then every 10 seconds for 50 seconds.
The electrical myotonia of each examined muscle was scored according to the Streiss and Sun scale.