Analysis Of Electrophysiological Characteristics And Differences Between Amyotrophic Lateral Sclerosis And Spinal And Bulbar Muscular Atrophy

Objective:A variety of neurodegenerative diseases exist,but Amyotrophic Lateral Sclerosis(ALS)is defined by the progressive degeneration of upper and lower motor neurons.The following motor neuron involvement is a feature of the X-linked recessive genetic condition known as Spinal and Bulbar Muscular Disease(SBMA).High clinical variability and covert progression characterize ALS.The goal of this study was to aid in differential diagnosis and increase the rate of clinical diagnosis.It examined the differences between ALS and SBMA in motor nerve conduction,sensory nerve conduction,needle-level electromyography,and repetitive frequency electrical stimulation from an electrophysiological perspective.Method:Subjects: Retrospective analysis was performed on 10 cases of clinically diagnosed SBMA patients in the First Hospital of Jilin University from January 2016 to August 2022 and 26 cases of inpatients with ALS mainly with motor neurone signs from January 2021 to January 2023.In addition,10 age-matched individuals who had zero neuroelectrophysiological tests were chosen as the ALS healthy control group.As a healthy control group for SBMA,healthy male individuals with unfavorable demographic traits and electrophysiological testing with SBMA patients were chosen.Statistical analysis:The measurement results were represented by a mean and standard deviation and followed a normal distribution.For group comparison,the Kruskal-Wallis test,twosample t test,and Mann-Whitney U test were applied.The study was omitted for missing data.In terms of frequency(percentage)(n(%)),n< 40 was used to describe count data.It was statistically significant at P< 0.05.SPSS 27.0 data analysis software was used to collect all the data.Results:1.SNAP amplitude and conduction velocity of sensory nerve in SBMA group were lower than those in normal control group,and the difference was statistically significant(P < 0.05).2.The CAMP amplitude of the upper extremity motor nerve in ALS group was significantly lower than that in SBMA group,and the difference was statistically significant(P < 0.05);the distal latency was higher than that in normal control group,and the difference was statistically significant(P < 0.05).3.SNAP amplitude of sensory nerve in SBMA group was significantly decreased compared with ALS group,and the difference was statistically significant(P < 0.05).4.Extensive neurogenic lesions were observed in pole electromyography in both ALS and SBMA groups,but the incidence of fibrillation potential and positive sharp wave in ALS group was higher than that in SBMA group.5.The attenuation degree of response of trapezius muscle and abductor muscle of little finger in SBMA group to low-frequency repetitive electrical stimulation was significantly decreased compared with ALS group,and the difference was statistically significant(P < 0.05).Conclusion:1.There are obvious differences in motor nerve conduction examination between ALS patients and SBMA patients,in which the significant reduction of CMAP amplitude of median nerve,ulnar nerve,tibial nerve and common peroneal nerve is a characteristic manifestation of ALS patients,which is helpful to distinguish SBMA from the following ALS with motor neuron involvement mainly.2.For male ALS patients with the following motor neuron involvement,if SNAP amplitude of sensory nerve decreases significantly,especially if multiple sensory nerves(including median nerve,ulnar nerve,sural nerve and superficial peroneal nerve)are involved,SBMA diagnosis should be considered,and SBMA gene testing is recommended.3.Both ALS patients and SBMA patients have extensive neurogenic impairment,but both contribute to differential diagnosis in spontaneous potential elicitation rate and motor unit action potential amplitude and duration.4.Significant amplitude attenuation of repetitive frequency electrical stimulation can be recorded in both ALS and SBMA patients,suggesting that abnormalities in the structure or function of neuromuscular junctions are involved in motor neurone disease.Amplitude attenuation of repetitive frequency electrical stimulation is helpful to detect subclinical lesions of neuromuscular joints in motor neurone disease patients and promote the diagnosis and differential diagnosis of SBMA.