【作者】 黄德晖；

【导师】 吴卫平；

【作者基本信息】 军医进修学院 ， 临床医学神经病专业， 2001， 硕士

【摘要】 背景：中枢神经系统原发性炎性脱髓鞘病是以中枢神经系统白质神经纤维脱髓鞘和小血管周围炎性细胞浸润为主要病理表现的一组自身免疫性疾病。发病机制目前尚不清楚，临床以多发性硬化(MS)和急性播散性脑脊髓炎(ADEM)最为常见。亚洲被认为是MS的低发病区，然而国内尚缺乏大规模的病例研究。 目的：总结多发性硬化和肿瘤样炎性脱髓鞘病(Tumor-likedemyelinating disease)的临床、影像及病理学特点。 方法：从临床、影像学及组织病理学等多方面对226例多发性硬化病例及14例肿瘤样炎性脱髓鞘病病例进行回顾性分析。 结果：226例多发性硬化病例中，临床确诊196例，实验室支持确诊16例，临床或实验室可能14例；男性85例，女性141例；发病年龄4岁至67岁，平均32.46岁，小于15岁的早发型病例占93%；30%病例病前有诱发因素，其中779%与感染有关；首发症状以感觉异常、肢体无力及视力减退最为多见，88.1%病例呈急性或亚急性起病，复发缓解型占 49.1%；病变部位中脊髓151例，脑干74例，小脑49例，大脑半球95例，视神经120例，66.8%病例出现脊髓受累，50.4%病例曾出现过尿便障碍，其中视神经脊髓炎占 29.2%。 2 军医进修学院硕士学位论文 中文摘要 在经手术后病理证实的14 例肿瘤样炎性脱髓鞘病病例 中，男8例，女6例，脊髓病变4例，颅内病变10例，急性 或亚急性起病9例，慢性起病5例，临床确诊为多发性硬化5 例。影像学显示14 例病灶周边均有不同程度的水肿及占位 效应，急性期、亚急性期更为突出，合并有出血、坏死4例， 环形强化 4例。组织病理学显示·14例病变区 LFB染色均可 见大片髓鞘脱失，NF及Bodlan染色显示轴索相对保存完好。 急性及亚急性病变区血管周围有较密集的以淋巴细胞为主的 袖套状浸润，并有大量的单核及泡沫样巨噬细胞弥漫浸润。呈 慢性过程的病灶表现为胶质纤维增多或斑痕形成，血管周围仍 可见到淋巴细胞为主的炎性浸润，但不如急性病变显著。 结论：国内多发性硬化的发病年龄较西方国家早，起病急， 病程短，视神经及脊髓易受侵犯，小脑、脑干较少受累，临床 表现中以视神经受损及尿便障碍较为突出，致残率较高。诱发 电位及MRI检查有助于发现亚临床病变。急性、亚急性期应 一 用激素疗效显著。 肿瘤样炎性脱髓鞘病在临床及影像学等方面可以有肿瘤 样非典型性表现，极易造成误诊。早期激素试验性治疗及立体 定向脑组织活检有利于确定病变性质。组织病理学上应注意观 察血管周边有无炎性浸润以及髓鞘脱失和轴索保留情况，免疫 组化染色有助于确立诊断。更多还原

【Abstract】 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更多还原