The Study Of Demyelinating Disease Of Central Nervous System On Clinic,Imaging And Pathology

Background: Primary demyelinating disease of central nervous system is a series auto-immune diseases, which the pathology shows demyelination of never fiber and infiltrative inflammation of perivascular in the white mater of central nervous system The pathogenesis of the disease is not clear Multiple sclerosis ( MS ) and acute disseminated encephalomyelitis ( ADEM ) are very common in the clinic The incidence rate of MS in Asia is lower than in western cquntry But we have not large case studies in China Objective: To summarize the clinical, imaging and pathological characteristics of MS and tumor-like demyelinating disease - Methods: We have retrospectively studied the characteristics of 226 MS and 14 Tumor-like demyelinating disease cases on clinic, imaging and histopathology Result: Among 226 MS cases, there are 196 clinically definite MS, 16 laboratory supported definite MS and 14 clinically or laboratory supported probable MS 85 in male and 141 in female The first onset age from 4 to 67 years old~ The children type (<15 years old) of MS holds 9 3% Before illness, 30 1% cases have evoke factors in which 77 9 relevant to 4 infections Paresthesia, weakness and visual deterioration are the most common initial symptoms 88 1% have acute and subacute courses and 49 1% have relapses The location of the lesions have 151 cases in spinal cord, 74 cases in brain stem, 45 cases in cerebellum, 95 cases in cerebral hemisphere and 120 cases in optic nerve 66 8% have spinal cord symptom, 50 4% have urinary or bowel dysfunction 29 2% were diagnosed Devic disease Among the 14 cases of tumor-like demyelinating disease which confirmed by pathology after the operation, there are 8 in male and 6 in female, 4 lesions located in the spinal cord and 10 lesions located in the brain, 9 cases have acute and subacute courses and 5 cases have chronic courses, 5 cases have been diagnosed clinically defi-nite MS In the imaging study, all the cases have variant degrees of edema, mass effect, which is prominent in the acute and subacute lesions 4 cases show hemorrhage and necrosis 8 cases show enhancement, in which 4 cases have a ring-like enhancement In histopathology, all the cases show demyelination by luex fast blue stain and relatively axon preservated by NF and Bodian stain In the acute and subacute lesions, there are prominent inflammatory infiltrate, especially a mixture of lymphocytes in the perivascular area and plus the massive monocytes and foam macrophages In the 5 chron1c leslons, gelatlnous flbers are lncreased and form wlthplaques We also can see per1vascular lnflammatory lnflltratlon,but do not promlnent as the acute leslonsConcluslon Compared to the western country, the onsetage of MS ln Ch1na 1s younger and the eplsode 1s more urgent,the course ls shorter Optlc nerve and splnal cord are easy to belnvaslved, Cerebellum and braln stem are seldom 1nvolved Inthe cllnlcal s1tuatlon, optlc nerve damage and urlnary or boweldysfunct1on are promlnent whlch cause the hlgh mutllatlon rateEvoked potentlals can flnd the paraclln1cal or s1lent leslons-- Treatment to the acute or subacute MS wlth cortlcosterolds lse ffe...