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.
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
90
Demographic data registration, medical history taking, physical examination. Electrocardiogram. Pulmonary function tests.
depending on the diseases enrolled, ALS and KD chose ALS-FRS functional scores and CMT chose CMTNS2 functional scores
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.
Peking University Third Hospital
Beijing, China
RECRUITINGThe 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).
Time frame: 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).
Time frame: 1 year
This platform is for informational purposes only and does not constitute medical advice. Always consult a qualified healthcare professional.