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

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 Overview

• Status: Recruiting
• Conditions: Amyotrophic Lateral Sclerosis
• Intervention / Treatment: Other: Basic information; Other: Functional scores; Device: MINUX

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 Type: Observational
• Enrollment (Anticipated): 90

Contacts and Locations

• Study Contact: Name: Xiaoxuan Liu; Phone Number: 13910982101; Email: zhangys0317@126.com
• Study Contact Backup: Name: Xiaoxuan Liu; Email: zhangys0317@126.com

Study Locations

• China
  • Beijing, China
    • Recruiting
    • Peking University Third Hospital

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Child; Adult; Older Adult
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Probability Sample

Study Population

ALS is a degenerative disease of the nervous system, with an incidence of 2-3 in 100,000. It usually begins in middle age and mainly presents with progressive atrophy and weakness of skeletal muscles throughout the body. After 3 to 5 years, it dies of respiratory muscle involvement. At present, there are no biomarkers for early diagnosis and no effective treatment.

Description

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.

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.

Study Plan

How is the study designed?

Design Details

• Observational Models: Case-Control
• Time Perspectives: Cross-Sectional

Number of groups / cohorts

4

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
ALS; Amyotrophic lateral sclerosis group	Other: Basic information; Demographic data registration, medical history taking, physical examination. Electrocardiogram. Pulmonary function tests.; Other: Functional scores; depending on the diseases enrolled, ALS and KD chose ALS-FRS functional scores and CMT chose CMTNS2 functional scores; Device: MINUX; MUNIX examination was performed on all subjects. A total of eight bilaterally symmetrical muscles were selected, bilateral abductor little finger or abductor pollicis brevis, bilateral biceps or deltoid muscles, bilateral tibialis anterior muscles, and bilateral femurs. For quadriceps, choose a muscle with less muscle atrophy.
CMT; peroneal muscular dystrophy group	Other: Basic information; Demographic data registration, medical history taking, physical examination. Electrocardiogram. Pulmonary function tests.; Other: Functional scores; depending on the diseases enrolled, ALS and KD chose ALS-FRS functional scores and CMT chose CMTNS2 functional scores; Device: MINUX; MUNIX examination was performed on all subjects. A total of eight bilaterally symmetrical muscles were selected, bilateral abductor little finger or abductor pollicis brevis, bilateral biceps or deltoid muscles, bilateral tibialis anterior muscles, and bilateral femurs. For quadriceps, choose a muscle with less muscle atrophy.
KD; Kennedy's disease group	Other: Basic information; Demographic data registration, medical history taking, physical examination. Electrocardiogram. Pulmonary function tests.; Other: Functional scores; depending on the diseases enrolled, ALS and KD chose ALS-FRS functional scores and CMT chose CMTNS2 functional scores; Device: MINUX; MUNIX examination was performed on all subjects. A total of eight bilaterally symmetrical muscles were selected, bilateral abductor little finger or abductor pollicis brevis, bilateral biceps or deltoid muscles, bilateral tibialis anterior muscles, and bilateral femurs. For quadriceps, choose a muscle with less muscle atrophy.
Control; Healthy control group	Other: Basic information; Demographic data registration, medical history taking, physical examination. Electrocardiogram. Pulmonary function tests.; Other: Functional scores; depending on the diseases enrolled, ALS and KD chose ALS-FRS functional scores and CMT chose CMTNS2 functional scores; Device: MINUX; MUNIX examination was performed on all subjects. A total of eight bilaterally symmetrical muscles were selected, bilateral abductor little finger or abductor pollicis brevis, bilateral biceps or deltoid muscles, bilateral tibialis anterior muscles, and bilateral femurs. For quadriceps, choose a muscle with less muscle atrophy.

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
The number of motor unit	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).	1 year
The size of motor unit	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).	1 year

Collaborators and Investigators

• Sponsor: Peking University Third Hospital
• Investigators: Principal Investigator: Xiaoxuan Liu, Peking University Third Hospital

Study record dates

Study Major Dates

• Study Start (Actual): June 1, 2018
• Primary Completion (Anticipated): December 1, 2022
• Study Completion (Anticipated): December 1, 2022

Study Registration Dates

• First Submitted: June 27, 2021
• First Submitted That Met QC Criteria: July 4, 2021
• First Posted (Actual): July 9, 2021

Study Record Updates

• Last Update Posted (Actual): July 9, 2021
• Last Update Submitted That Met QC Criteria: July 4, 2021
• Last Verified: June 1, 2021

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Pathologic Processes; Metabolic Diseases; Central Nervous System Diseases; Nervous System Diseases; Neuromuscular Diseases; Neurodegenerative Diseases; Spinal Cord Diseases; TDP-43 Proteinopathies; Proteostasis Deficiencies; Sclerosis; Motor Neuron Disease; Amyotrophic Lateral Sclerosis
• Other Study ID Numbers: M2018223

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: No

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No