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Low-frequency Repetitive Nerve Stimulation in Myotonic Dystrophy Type 1

NCT05662150 · Status: COMPLETED · Phase: NA · Type: INTERVENTIONAL · Enrollment: 9

Last updated 2022-12-22

No results posted yet for this study

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 decrement

with 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).

Conditions

  • Myotonic Dystrophy 1

Interventions

DIAGNOSTIC_TEST

Low frequency repetitive stimulation

Neuromuscular transmission was tested by using short-lasting low frequency RNS (10 stimuli at 3 Hz). The test was applied on the abductor digiti minimi (ADM), anterior tibial, orbiculis oculi, trapezius, anconeus and EDB muscles of one side, in this particular order, by supramaximal stimulation of the corresponding nerve.

Sponsors & Collaborators

  • Vrije Universiteit Brussel

    lead OTHER

Principal Investigators

  • Jella De Ville, MD · Universitair Ziekenhuis Brussel

Study Design

Allocation
RANDOMIZED
Purpose
BASIC_SCIENCE
Masking
NONE
Model
SINGLE_GROUP

Eligibility

Sex
ALL
Healthy Volunteers
No

Timeline & Regulatory

Start
2021-09-01
Primary Completion
2022-06-11
Completion
2022-06-20

Countries

  • Belgium

Study Locations

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Read the full study record

This page highlights key information. For complete eligibility criteria, study locations, investigator contacts, and the full protocol, visit the original record on ClinicalTrials.gov.

View NCT05662150 on ClinicalTrials.gov