Clinical, Pathological And Animal Models Research On MND

Etiology and mechanisms of motor neuron disease(MND), a nervous system degeneration disease; are unknow at present. Many evidences suggested that MND, probably resulting from multigeneous or heterogenic elements, were involved in hereditary, environment, virus infection and immune abnormality. Auto-immune theory data of MND were increased in last few years with technological development of immunology and molecular biology.Based on clinical data analysis and clinical pathological investigation of MND, studies on experimental diagnostic method were done, three animal models mimicking human clinical diseases of immune-mediated MND were induced; the changes in CNTF-positive expression and apoptotic phenomena of immune-mediated animal model of EAGMD were investigated. Main results of the study are summarized as follows:1. Clinical inverstigation of MND 1.1 Clinical data analysis of MNDClinical features of 80 cases of MND were summarized retrospectively, it shown that Chinese suffering from the disease were 10—20 years earlier than overseas patients and ratio of male to female is twice higher. Lesion sites: ALS involved cervical and lumbar spinal cord, oblongatal medullar. PSMA mainly cervical spinal cord, PLS mainly lumbar, all of other types mixed. Duration: Patients with faster development and shorter duration( <6 months) are mainly ALS, the duration of majority of ALS patients is 7-24 months. Cases with duration longer than 40 months are mainly PSMA, secondly ALS-PUMNS, ALS. Therefore we consider that pyramidal sings is related to duration, and the duration of patients without pyramidal signs is...