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Pilot Study for the Development of an Activity and Quality of Life Questionnaire for the Follow-up of Patients With Non-dystrophic Myotonia

Not Applicable
Not yet recruiting
Conditions
Non-Dystrophic Myotonia
Interventions
Other: Questionnaire for Non-dystrophic myotonias
Registration Number
NCT06136416
Lead Sponsor
Centre Hospitalier Universitaire de Nice
Brief Summary

Non-dystrophic myotonias (MND) are rare neuromuscular diseases caused by mutations in the voltage-dependent channels of skeletal muscles, resulting in delayed muscle relaxation after voluntary contraction. They include various conditions such as congenital myotonia, congenital paramyotonia and sodium channel myotonia. The main characteristic is myotonia, muscle stiffness accompanied by pain, fatigue and weakness. Symptoms vary in intensity, and fluctuation complicates clinical assessment.

Until now, no validated scale to assess the severity of myotonia is the subject of a consensus among neurologists. It therefore seems necessary to establish a scale to simply and quickly assess the severity of myotonia to fill this need.

The areas of this future scale were identified by the study coordinator based on existing questionnaires and scales. These areas have been validated by a scientific committee composed of expert neurologists.

The main objective of the study is to validate the adequacy and formulation of the scale questions by involving 10 patients who will complete the questionnaire twice to assess its fidelity. At the end of the study, the committee will exclude inappropriate questions. The goal is to create a reliable scale to assess the severity of myotonia.

Detailed Description

Not available

Recruitment & Eligibility

Status
NOT_YET_RECRUITING
Sex
All
Target Recruitment
10
Inclusion Criteria

Not provided

Exclusion Criteria

Not provided

Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Arm && Interventions
GroupInterventionDescription
Patients with Non-dystrophic myotoniasQuestionnaire for Non-dystrophic myotonias-
Primary Outcome Measures
NameTimeMethod
Evaluate the relevance of the MNDActiveLive questionnaireThe day of inclusion

The relevance of the MNDActiveLive questionnaire will be measured using patient responses to the relevance and formulation assessment grid. Indeed, it is about evaluating the percentage of relevant questions (that is, relatively relevant, perfectly relevant and relevant). The question will be considered relevant if the percentage is higher than 80%.

Secondary Outcome Measures
NameTimeMethod
Assess reproducible issuesThe day of inclusion and 7 days after

The reproducibility of each question will be assessed by the percentage of patients with change in response between the response provided at inclusion and that provided 7 days later

Select questions to keepThe day of inclusion and 7 days after

Questions to be retained will be identified using the percentage of questions that are reproducible, well formulated and relevant

Identify questions to rephraseThe day of inclusion

Questions to be reformulated will be identified using the percentage of questions that are poorly formulated and relevant (i.e., poorly formulated or unclear, slightly ambiguous or that may be confusing/relatively relevant, perfectly relevant and relevant). The question will be reformulated if the percentage is greater than 80%

Evaluate the formulation of the MNDActiveLive questionnaireThe day of inclusion

The formulation of the MNDActiveLife questionnaire will be evaluated using the percentage of well-formulated questions (i.e., satisfactorily formulated, well-formulated, perfectly formulated) and the percentage of poorly-formulated questions (i.e. poorly worded or unclear, slightly ambiguous or confusing). The question will be considered well formulated if the percentage is greater than 80%.

Identify questions to be excludedThe day of inclusion

Questions to be excluded will be identified using the percentage of questions poorly formulated and irrelevant (poorly formulated or unclear, slightly ambiguous or may be confusing/not at all relevant, not relevant). The question will be excluded if the percentage of poorly formulated and irrelevant questions is greater than 80%.

Trial Locations

Locations (1)

Nice University Hospital

🇫🇷

Nice, Alpes Maritimes, France

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