Application of Motor Unit Estimation Index in Amyotrophic Lateral Sclerosis and Related Diseases

• Conditions: Amyotrophic Lateral Sclerosis
• Interventions: Other: Basic information; Other: Functional scores; Device: MINUX

• Registration Number: NCT04956822
• Lead Sponsor: Peking University Third Hospital

Brief Summary

This was a cross-sectional study in which patients were divided into 20 patients with amyotrophic lateral sclerosis, 20 patients with peroneal muscular dystrophy, 20 patients with Kennedy's disease and 30 healthy controls, in which patients with amyotrophic lateral sclerosis continued to be followed up for 1 year and the results of 4 cross-sectional examinations were taken.

Detailed Description

Background Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease that usually starts in middle age and manifests mainly as progressive atrophy and weakness of skeletal muscles throughout the body, with death from respiratory muscle involvement after 3 to 5 years. There are no biomarkers for early diagnosis and no effective treatments. Because of the rapid progression of amyotrophic lateral sclerosis, it is important to find indicators that can objectively reflect early changes in the disease. The motor unit number index (MUNIX) is a non-invasive, rapid and objective method to assess the number of motor units, which reflects the loss of motor neurons and has its theoretical basis in monitoring early disease progression.

Objective To explore the diagnostic value of MUNIX in motor neuron disease and other related disorders.

To investigate the role of the 1-year rate of change of MUNIX in monitoring the disease progression in patients with amyotrophic lateral sclerosis.

To investigate the role of MUNIX in predicting survival analysis of ALS patients

\[Design\] This was a cross-sectional study in which patients were divided into 20 patients with amyotrophic lateral sclerosis, 20 patients with peroneal muscular dystrophy, 20 patients with Kennedy's disease and 30 healthy controls, in which patients with amyotrophic lateral sclerosis continued to be followed up for 1 year and the results of 4 cross-sectional examinations were taken.

Study Timeline

• Study Start: 2018-06-01
• Status Verified: 2021-06
• Study First Submitted: 2021-06-27
• Study First Submitted QC: 2021-07-04
• Last Update Submitted: 2021-07-04
• Study First Posted: 2021-07-09
• Last Update Posted: 2021-07-09
• Primary Completion: 2022-12-01
• Study Completion: 2022-12-01

Recruitment & Eligibility

• Status: UNKNOWN
• Sex: All
• Target Recruitment: 90

Inclusion Criteria

• ALS patient group: 20 patients with confirmed or proposed ALS meeting the 1998 revised El Escorial diagnostic criteria for limb onset ALS.

• CMT group: 20 patients with peroneal muscular dystrophy meeting the genetically confirmed diagnosis, who signed an informed consent form.

• KD group: 20 patients with genetically confirmed Kennedy's disease consistent with genetic diagnosis, signed informed consent.

• healthy controls:

  1. age-matched healthy adults who volunteered to participate;
  2. definite exclusion of tremor, tonicity, and prior brain disease;
  3. exclusion of common disorders affecting peripheral nerves such as entrapment peripheral neuropathy, diabetic peripheral neuropathy, and alcoholic peripheral neuropathy;
  4. signed informed consent.

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Exclusion Criteria

1. signs of sensory impairment;
2. significant sphincter dysfunction;
3. visual and oculomotor impairment;
4. autonomic dysfunction;
5. signs of extravertebral symptoms;
6. severe cortical dysfunction;
7. ALS-like syndrome.

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Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
Control	Basic information	Healthy control group
KD	Functional scores	Kennedy's disease group
KD	MINUX	Kennedy's disease group
CMT	Basic information	peroneal muscular dystrophy group
CMT	Functional scores	peroneal muscular dystrophy group
Control	Functional scores	Healthy control group
ALS	Basic information	Amyotrophic lateral sclerosis group
ALS	MINUX	Amyotrophic lateral sclerosis group
KD	Basic information	Kennedy's disease group
Control	MINUX	Healthy control group
ALS	Functional scores	Amyotrophic lateral sclerosis group
CMT	MINUX	peroneal muscular dystrophy group

Primary Outcome Measures

Name	Time	Method
The size of motor unit	1 year	Motor unit estimation index (MUNIX) is a non-invasive electrophysiological technique that uses compound muscle action potential (CMAP) and surface electromyographic interference pattern (SIP) methods to evaluate the number and size of motor units (Mus) (motor unit number size, MUSIX).
The number of motor unit	1 year	Motor unit estimation index (MUNIX) is a non-invasive electrophysiological technique that uses compound muscle action potential (CMAP) and surface electromyographic interference pattern (SIP) methods to evaluate the number of motor units (Mus) (motor unit number size, MUSIX).

Trial Locations

Locations (1)

Peking University Third Hospital; 🇨🇳 Beijing, China