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

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ClinicalTrials.gov Identifier: NCT04956822
Recruitment Status : Recruiting
First Posted : July 9, 2021
Last Update Posted : July 9, 2021
Sponsor:
Information provided by (Responsible Party):
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.

Condition or disease Intervention/treatment
Amyotrophic Lateral Sclerosis 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.

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Study Type : Observational
Estimated Enrollment : 90 participants
Observational Model: Case-Control
Time Perspective: Cross-Sectional
Official Title: Application of Motor Unit Estimation Index in Amyotrophic Lateral Sclerosis and Related Diseases
Actual Study Start Date : June 1, 2018
Estimated Primary Completion Date : December 1, 2022
Estimated Study Completion Date : December 1, 2022


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.




Primary Outcome Measures :
  1. The number of motor unit [ Time Frame: 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).

  2. The size of motor unit [ Time Frame: 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).



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Ages Eligible for Study:   Child, Adult, Older Adult
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   Yes
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.
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.

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT04956822


Contacts
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Contact: Xiaoxuan Liu 13910982101 zhangys0317@126.com
Contact: Xiaoxuan Liu zhangys0317@126.com

Locations
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China
Peking University Third Hospital Recruiting
Beijing, China
Contact: Xiaoxuan Liu    13910982101      
Sponsors and Collaborators
Peking University Third Hospital
Investigators
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Principal Investigator: Xiaoxuan Liu Peking University Third Hospital
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Responsible Party: Peking University Third Hospital
ClinicalTrials.gov Identifier: NCT04956822    
Other Study ID Numbers: M2018223
First Posted: July 9, 2021    Key Record Dates
Last Update Posted: July 9, 2021
Last Verified: June 2021
Individual Participant Data (IPD) Sharing Statement:
Plan to Share IPD: No

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Studies a U.S. FDA-regulated Drug Product: No
Studies a U.S. FDA-regulated Device Product: No
Additional relevant MeSH terms:
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Motor Neuron Disease
Amyotrophic Lateral Sclerosis
Sclerosis
Pathologic Processes
Neurodegenerative Diseases
Nervous System Diseases
Neuromuscular Diseases
Spinal Cord Diseases
Central Nervous System Diseases
TDP-43 Proteinopathies
Proteostasis Deficiencies
Metabolic Diseases