Study Purpose:
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 Status:
Recruiting
Disease:
Amyotrophic Lateral Sclerosis
Study Type:
Observational
Type of Intervention:
N/A
Intervention Name:
N/A
Placebo:
N/A
Phase:
N/A
Study Chair(s)/Principal Investigator(s):
Xiaoxuan Liu, Peking University Third Hospital
Clinicaltrials.gov ID:
Neals Affiliated?
No
Coordinating Center Contact Information
Xiaoxuan Liu / email hidden; JavaScript is required / 13910982101
Full Study Summary:
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.
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 Sponsor:
Peking University Third Hospital
Estimated Enrollment:
90
Estimated Study Start Date:
06 / 01 / 2018
Estimated Study Completion Date:
12 / 01 / 2022
Posting Last Modified Date:
07 / 09 / 2021
Date Study Added to neals.org:
07 / 09 / 2021
Minimum Age:
N/A
Maximum Age:
N/A
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.
Peking University Third Hospital | Recruiting
Xiaoxuan Liu / 13910982101
Beijing
China