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

NCT ID: NCT04956822

Last Updated: 2021-07-09

Basic Information

• Recruitment Status: UNKNOWN
• Total Enrollment: 90 participants
• Study Classification: OBSERVATIONAL
• Study Start Date: 2018-06-01
• Study Completion Date: 2022-12-01

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.

Conditions

Amyotrophic Lateral Sclerosis

Study Design

• Observational Model Type: CASE_CONTROL
• Study Time Perspective: CROSS_SECTIONAL

Study Groups

ALS

Amyotrophic lateral sclerosis group

Basic information

Intervention Type: OTHER

Demographic data registration, medical history taking, physical examination. Electrocardiogram. Pulmonary function tests.

Functional scores

Intervention Type: OTHER

depending on the diseases enrolled, ALS and KD chose ALS-FRS functional scores and CMT chose CMTNS2 functional scores

MINUX

Intervention Type: DEVICE

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

Basic information

Intervention Type: OTHER

Demographic data registration, medical history taking, physical examination. Electrocardiogram. Pulmonary function tests.

Functional scores

Intervention Type: OTHER

depending on the diseases enrolled, ALS and KD chose ALS-FRS functional scores and CMT chose CMTNS2 functional scores

MINUX

Intervention Type: DEVICE

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

Basic information

Intervention Type: OTHER

Demographic data registration, medical history taking, physical examination. Electrocardiogram. Pulmonary function tests.

Functional scores

Intervention Type: OTHER

depending on the diseases enrolled, ALS and KD chose ALS-FRS functional scores and CMT chose CMTNS2 functional scores

MINUX

Intervention Type: DEVICE

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

Basic information

Intervention Type: OTHER

Demographic data registration, medical history taking, physical examination. Electrocardiogram. Pulmonary function tests.

Functional scores

Intervention Type: OTHER

depending on the diseases enrolled, ALS and KD chose ALS-FRS functional scores and CMT chose CMTNS2 functional scores

MINUX

Intervention Type: DEVICE

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.

Interventions

Basic information

Demographic data registration, medical history taking, physical examination. Electrocardiogram. Pulmonary function tests.

Intervention Type: OTHER

Functional scores

depending on the diseases enrolled, ALS and KD chose ALS-FRS functional scores and CMT chose CMTNS2 functional scores

Intervention Type: OTHER

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.

Intervention Type: DEVICE

Eligibility Criteria

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.

• Eligible Sex: ALL
• Accepts Healthy Volunteers: Yes

Sponsors

Peking University Third Hospital

OTHER

Sponsor Role: lead

Responsible Party

Responsibility Role: SPONSOR

Principal Investigators

Xiaoxuan Liu

Role: PRINCIPAL_INVESTIGATOR

Peking University Third Hospital

Locations

Peking University Third Hospital

Beijing, , China

Site Status: RECRUITING

Countries

China

Central Contacts

Xiaoxuan Liu

Role: CONTACT

zhangys0317@126.com

13910982101

Xiaoxuan Liu

Role: CONTACT

zhangys0317@126.com

Facility Contacts

Xiaoxuan Liu

Role: primary

13910982101

Other Identifiers

M2018223

Identifier Type: -

Identifier Source: org_study_id