Prion-like Mechanism Of ALS-Research On Clinical Manifestation And EMG

Objective:From the clinical manifestation and EMG perspective to analyze the ALS protein-like progression mechanism.Methods:103 patients with sporadic ALS were retrospectively analyzed.It was divided into continuous and non-continuous types according to the clinical manifestation and the affected site of EMG to study the prion-like mechanism.And comparing the differences among continuous and non-continuous pattern at the aspect of age,onset site,gender,course of disease and FRST.Results:In this study,103 patients were finally recruited.At the aspect of distribution of clinical affected region,continuous,47(48.4%)cases,non-continuous,48(51.6%)cases,At the aspect of distribution of EMG affect region,90(87.4%)cases were continuous pattern and 13(12.6%)cases were non-continuous pattern.47 clinical non-continuous pattern cases were due to lack of thoracic involvement.There was no statistically relationship among continuous and non-continuous pattern at the aspect of gender,age,course of disease and FRST.Among the 60 male patients,37 cases with upper limb onset,10 cases with lower limb onset,7 cases with bulbar onset,male were more often suffered from limb onset.In 43 cases of female patients,13 cases with bulbar onset,14 cases with upper limb onset,7 cases lower limb onset,female were more often suffered from bulbar and upper limb onset.The site of the onset was statistically related to gender(chi-square test P=0.013).Patients older than 60 years share majority among the patients with bulbar onset;The rest of patients share majority in the age of 40 to 60 years old,theonset site were statistically related to age(chi-square test,P=0.030).Conclusion:Analysis on clinical and EMG affected region,the distribution of the affected region was always starts from one region and then spread to adjacent region,which was consistent with the prion-like mechanism of ALS.