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Completed
NCT05662150
Low-frequency Repetitive Nerve Stimulation in Myotonic Dystrophy Type 1
Conditions: Myotonic Dystrophy 1
Sex: All
Phase: NA
Enrollment: 9
Sponsor: Vrije Universiteit Brussel
Location: Belgium
Summary
The study design is a prospective cohort study.
It aims to evaluate the neuromuscular junction in dystrophic myotonia 1 (DM 1) using low-frequency repetitive nerve stimulation (RNS) on several nerve-muscle pairs of the one side including proximal and distal muscles of upper and lower extremities.
First, it will be investigated whether a decrement with 3 Hz stimulation, as described in literature, is reproducible in our patient population.
If this is the case, it will be examined whether it is the consequence of a dysfunction of the neuromuscular junction or rather linked to a hypo-excitability of some muscle fibers due to myotonia.
For this purpose, additional tests including short exercise test (to observe any decrement resulting from an inexcitability in myotonic muscle fibers) and needle EMG (for mapping myotonic discharges in the muscles tested with repetitive nerve stimulation) will be performed.
Single fiber-EMG will not be provided in this study as an abnormal result does not necessarily indicate a dysfunction of the neuromuscular junction but could just as well be due to the muscular dystrophy in the context of DM1.
Finally, it will be investigated if there is a correlation between the decrementwith 3 Hz stimulation and clinical signs as fixed muscle weakness (via Medical Research Counsil (MRC) scale, DM-activ scale [30]) and fatigue (via MG-ADL scale).
Eligibility Criteria
Inclusion Criteria:* genetically confirmed DM1Exclusion Criteria:minor ageauto-immune diseasesmedical conditions involving the neuromuscular junction: (myasthenia gravis, Lambert-Eaton myasthenic syndrome, congenital myasthenia syndromes).
Source: ClinicalTrials.gov (NCT05662150). StuddyBuddy aggregates publicly available trial information.
