Myotonia and Muscle Stiffness in NMD

Completed

• Conditions: Neuromuscular Disorders
• Interventions: Device: MyotonPro; Diagnostic Test: Six-minute walk test; Diagnostic Test: Semiquantitative muscle ultrasound; Diagnostic Test: Hand-held Dynamometry of muscle strength

• Registration Number: NCT04411732
• Lead Sponsor: LMU Klinikum

Brief Summary

The primary objective of this study is to assess stiffness, muscle tone, relaxation periods and elasticity of various muscles in patients with dystrophic or non-dystrophic myotonia. The secondary objectives are (1) to provide reference values for stiffness, muscle tone, relaxation periods and elasticity of various muscles in patients with dystrophic or non-dystrophic myotonia; to provide reference values for stiffness, muscle tone, relaxation periods and elasticity of various muscles in patients with non-myotonic neuromuscular disorders, (3) assess correlations between to compare result values for stiffness, muscle tone, relaxation periods and elasticity with clinical muscle function tests, measured by clinical evaluation (MRC-scale) and the 6-minute walk test; (4) assess correlations between subcutaneous fat and muscle thickness and echogenicity, measured by muscle ultrasound and result values for stiffness, muscle tone, relaxation periods and elasticity.

Detailed Description

Not available

Study Timeline

• Study Start: 2019-10-01
• Study First Submitted: 2020-05-25
• Study First Submitted QC: 2020-05-29
• Study First Posted: 2020-06-02
• Primary Completion: 2023-07-31
• Study Completion: 2023-09-30
• Status Verified: 2023-10
• Last Update Submitted: 2023-10-09
• Last Update Posted: 2023-10-11

Recruitment & Eligibility

• Status: COMPLETED
• Sex: All
• Target Recruitment: 133

Inclusion Criteria

For Patients:

• ≥18 years of age
• Confirmed neuromuscular disease
• written consent
• able and willing to perform study prodcedures

For healthy volunteers:

• age ≥18 years of age
• written informed consent
• no clinical sign for neuromuscular disorders

Exclusion Criteria

• Severe comorbidities
• Patient is participating in another clinical study using investigational treatment
• Patient cannot perform required muscle function tests
• the patient, in the opinion of the investigator, is unable to adhere to the requirements of the study

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
healthy controls	MyotonPro	cohort of healthy controls
patients with neuromuscular disorder	MyotonPro	cohort of patients with neuromuscular disorder
patients with neuromuscular disorder	Semiquantitative muscle ultrasound	cohort of patients with neuromuscular disorder
patients with neuromuscular disorder	Hand-held Dynamometry of muscle strength	cohort of patients with neuromuscular disorder
patients with neuromuscular disorder	Six-minute walk test	cohort of patients with neuromuscular disorder
healthy controls	Six-minute walk test	cohort of healthy controls
healthy controls	Semiquantitative muscle ultrasound	cohort of healthy controls
healthy controls	Hand-held Dynamometry of muscle strength	cohort of healthy controls

Primary Outcome Measures

Name	Time	Method
MyotonPro device in patients with dystrophic and non-dystrophic myotonia	1 day	The primary objective of this study is the comparison of viscoelastic stiffness of the underlying tissue measured by stiffness (S), elasticity (D) and relaxation (R) between patients with dystrophic and non-dystrophic myotonia.

Secondary Outcome Measures

Name	Time	Method
MyotonPro device - reference values in patients with non-myotonic neuromuscular disorders	1 day	The secondary objective of this study is to provide reference values for stiffness, muscle tone, relaxation periods and elasticity of various muscles in patients with non-myotonic neuromuscular disorders.
MyotonPro device - correlations between stiffness, muscle tone, relaxation periods and elasticity and the 6-minute-walk test	1 day	The secondary objective of this study is to compare stiffness, muscle tone, relaxation periods and elasticity with clinical muscle endurance function tests, measured by the 6-minute walk test.
MyotonPro device - correlations between stiffness, muscle tone, relaxation periods and elasticity with subcutaneous fat and muscle thickness and echogenicity	1 day	The secondary objective of this study is to assess correlations between subcutaneous fat and muscle thickness and echogenicity, measured by muscle ultrasound and result values for stiffness, muscle tone, relaxation periods and elasticity.
MyotonPro device - reference values in patients with dystrophic and non-dystrophic myotonia	1 day	The secondary objective of this study is to provide reference values for stiffness, muscle tone, relaxation periods and elasticity of various muscles in patients with dystrophic or non-dystrophic myotonia.
MyotonPro device - correlations between stiffness, muscle tone, relaxation periods and elasticity with clinical muscle function tests	1 day	The secondary objective of this study is to compare stiffness, muscle tone, relaxation periods and elasticity with clinical muscle function tests, measured by clinical evaluation (MRC-scale).

Trial Locations

Locations (1)

Friedrich-Baur-Institute, Dep. of Neurology Klinikum der Universitaet Muenchen; 🇩🇪 Munich, Bavaria, Germany