Application of Motor Unit Estimation Index in Amyotrophic Lateral Sclerosis and Related Diseases
试验速览
- 阶段
- 不适用
- 入组人数
- 90
- 试验地点
- 1
- 主要终点
- The size of motor unit
研究概览
简要总结
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.
详细描述
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.
研究设计
- 研究类型
- Observational
- 观察模型
- Case Control
- 时间视角
- Cross Sectional
入排标准
- 性别
- All
- 接受健康志愿者
- 是
入选标准
- •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:
- •age-matched healthy adults who volunteered to participate;
- •definite exclusion of tremor, tonicity, and prior brain disease;
- •exclusion of common disorders affecting peripheral nerves such as entrapment peripheral neuropathy, diabetic peripheral neuropathy, and alcoholic peripheral neuropathy;
- •signed informed consent.
排除标准
- •signs of sensory impairment;
- •significant sphincter dysfunction;
- •visual and oculomotor impairment;
- •autonomic dysfunction;
- •signs of extravertebral symptoms;
- •severe cortical dysfunction;
- •ALS-like syndrome.
结局指标
主要结局
The size of motor unit
时间窗: 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
时间窗: 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).
次要结局
未报告次要终点