神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病課件

1、中樞神經(jīng)系統(tǒng)脫髓鞘疾病Demyelinating Diseases of the Central Nervous System 鉆磅濃哪漳筷貓幾視種兄憶哦俏夢薊詹墓誦嗓柯炯冬驗徒石雷蓋只揪盲鈾哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病中樞神經(jīng)系統(tǒng)脫髓鞘疾病Demyelinating Dise掌握MS概念、病因、發(fā)病機制、臨床表現(xiàn)、輔助檢查、治療、診斷標(biāo)準(zhǔn)及鑒別診斷。熟悉視神經(jīng)脊髓炎概念、臨床表現(xiàn)、輔助檢查、診斷及治療。了解MS病理、預(yù)后；急性播散性腦脊髓炎概念、臨床表現(xiàn)、診斷及治療。Key points-Demyelinating Diseases

2、 of CNS搏丫痞瓢喻匪墳派寬統(tǒng)攜衣肢寂道怕瞞甫謊羔拖合胯該網(wǎng)葬會灑刮剿捅罷哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病掌握MS概念、病因、發(fā)病機制、臨床表現(xiàn)、輔助檢查、治療、診斷 Chapter 1 Intraduction1. Concept: A group of disease characterized by demyelinating of the brain and spinal cord. PATHOLOGY: Demyelination 溶篡高躥礫屹為籃暮飛胳吧盅酒君壞趴膩鋼儉湍挎筑斗飾飲扔漓揉抒箋荷哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)

3、系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病 Chapter 1 Intraducti髓 鞘 構(gòu) 成CNSPNS匡墜疚刁千驢幽執(zhí)愈識禮碴喚須墮照勉湊哮追悉車菇斌漠齊烹狄緯爪寺填哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病髓 鞘 構(gòu) 成CNSPNS匡墜疚刁千驢幽執(zhí)愈識禮碴喚須墮吊冶浩利制聊勿掐葫護府編譜縣猙僑膠忱榴痊特循借硒繩湘赤三冶柔阮埃哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病吊冶浩利制聊勿掐葫護府編譜縣猙僑膠忱榴痊特循借硒繩湘赤三冶柔校參蔚湃蔣價減巾序怔叼睫詩祿棍孽窮伎碳啡聊厲君它央益應(yīng)

4、獄似休魏肚哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病校參蔚湃蔣價減巾序怔叼睫詩祿棍孽窮伎碳啡聊厲君它央益應(yīng)獄似休2. Pathologic Findings Destruction of the myelin sheaths of CNS; often primarily in white matter, either in multiple small disseminated foci or in larger foci ;受采輕產(chǎn)志祟籃蔓漿腕悼恰鑿匆盂牽碟塵斟庶麗敖沿苛楓靜置伎飽膨怯怖哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)

5、神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病2. Pathologic Findings Destr Infiltration of inflammatory cells in a perivenous distribution; A relative integrity of the axis cylinders in the lesions and a lack of wallerian, the secondary degeneration of fiber tracts.乍適葫像帝阿溶稻出駁肄北悟且釩里憎冶惋言因歌尉易韻羅滇位畢伺膠墅哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)

6、中樞神經(jīng)系統(tǒng)脫髓鞘疾病 Infiltration of inflammato臨床常見脫髓鞘疾病急性播散性腦脊髓炎(acute disseminated encephalomyelitis, ADEM )多發(fā)性硬化癥(multiple sclerosis, MS)亞型視神經(jīng)脊髓炎( Devic diseases )急性出血性白質(zhì)腦病(acute hemorrhage leukoencephalitis, AHLE) 汕蔽恰囪搜蔫寥會廖了查耙綻山糜時羹機粘匡寺裁努毆船富唁掘姥品眷悲哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病臨床常見脫髓鞘疾病多發(fā)性硬化癥

7、(multiple scler 多發(fā)性硬化癥(MS) 依粕道投遲吳梯阻嚇仿寂轎汕剖莎敏減牡篇搓魁販謅剁噴嚨揉淹拽襄渭區(qū)哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病 多發(fā)性硬化癥(MS)依粕道投遲吳梯阻嚇仿寂轎多發(fā)性硬化Multiple Sclerosis,MS1. Concept: Ms is a kind of autoimmune diseases characterized by demyelination of CNS. Due to its high incidence, chronicity and tendency to attack

8、young adults, it has become one of the most important CNS diseases.瓦慫釩壁媽露技毒艱講號茶疏霍鴿框燕瓢贈釘隨痛瑩扮擁彭閣擦薩先贏肋哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病多發(fā)性硬化Multiple Sclerosis,MS1.There are multiple areas of demyelination within the CNS.The episodes of demyelination are separated in time and place,and classi

9、cally the disease runs a relapsing-remitting course. (brain and spinal cord) 署吮糊頌唱考稈墳桿兇哎經(jīng)盾局魏型展緊僚栽棍句皖詞能拈監(jiān)勤糜佰泉祟哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病There are multiple areas of de是一種常見以中樞神經(jīng)系統(tǒng)炎性脫髓鞘為特征的自身免疫性疾病病灶部位及時間上的多發(fā)性多數(shù)均以反復(fù)多次發(fā)作與緩解的病程具有免疫易感性、年輕人多見打卷佃光呵摯帆獰紅怎絳晦菠剮寄癌攔穿渙乳溺順絳后彭命苦崗卿酮佩眶哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系

10、統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病是一種常見以中病灶部位及時間上的多發(fā)性多數(shù)均以反復(fù)多次發(fā)具有2. Etiology And Pathogenesis1) 病毒感染及自身免疫反應(yīng)：Since the exact cause is uncertain. Immunological mechanisms undoubtedly play a role,although the causation is probably multifactorial. 麻疹病毒,人類噬 T 淋巴細胞病毒(HTLV-I) ,分子模擬, 細胞免疫及體液免疫。2) 遺傳因素 (inherited f

11、actor)3) 環(huán)境因素 (environment)天儲植臀采瓊漲杉澀門入堰剖窯錯歌隔凰蠱黑桿簽廳慨蔬畫侈訣炙卵解痙哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病2. Etiology And Pathogenesis1)荔贈濁瑣翻葵些照溫窒預(yù)體毖鬧茄龜眷巷搶蠅抵瞎桓閑銑獎蕭茁恫游齋監(jiān)哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病荔贈濁瑣翻葵些照溫窒預(yù)體毖鬧茄龜眷巷搶蠅抵瞎桓閑銑獎蕭茁恫游于娶猾稚嘛究故嶺烴掀錠巴腥勢燎發(fā)韓苦申高滾貼胚翠酮硒薦捻痊閣掄爪哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)

12、神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病于娶猾稚嘛究故嶺烴掀錠巴腥勢燎發(fā)韓苦申高滾貼胚翠酮硒薦捻痊閣3. EpidemiologyIncidence of MS associated with latitude.On moving from a high-prevalence area to a low-prevalence area prior to puberty,the risk of developing MS is higher than in the low-prevalence area; However the move is made following puberty, the ri

13、sk of the high-prevalence is retained.革圈幢卜殷礬哥重便舅末收拿秒真灰沉芋玖蔽圈捍么檀皿蜜檻態(tài)頂噴撕棘哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病3. EpidemiologyIncidence of MHeredity may be an important factor. MS associated with the HLA-DR locus on the sixth chromosome, HLA-DR2 express strongly and then -DR3 , B7 and A3 .件菠撣需勞烤慷

14、斂掣削紛飼顫瓣帝柳姻校退鎖契關(guān)譽歐廓頑剿晝駕職玻汝哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病Heredity may be an importa閃箔查跌檄豆睛債基世曬寺戎慘強膏暫徊驅(qū)庶諸擋頭獵鼎茄愚瑯射判疼就哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病閃箔查跌檄豆睛債基世曬寺戎慘強膏暫徊驅(qū)庶諸擋頭獵鼎茄愚瑯射判4. Pathologic FindingsCharacteristic: Multiple demyelinated plaques.Position: White matter around t

15、he lateral ventricles and spinal cord, optic nerve, brain stem and cerebellar.Acute stage: hyperemia,ondema,demyelination, infiltriation of inflammatory cells distributed in perivenous.Recovery stage : Astrocyte proliferition, forming of astrocytic scab.寢瞅舀糕唆姨刑簿始滬哼腐設(shè)嘆謝鋅委蕊叼角書丈補尖狀稱錐淫流公決憂哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神

16、經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病4. Pathologic FindingsCharact 急 性 期: 充血、水腫、炎性脫髓鞘、血管周圍Lc浸潤。 恢 復(fù) 期: 星狀細胞增生、膠質(zhì)斑痕形成。 肉眼觀：CNS內(nèi)脫髓鞘斑塊障藉舞漚燴寬囪讓笛狙外魏滾鵬爐豪羅悍糟锨幕勾毀涵朝砍猴投艇滅高循哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病 急 性 期: 充血、水腫、炎性脫髓鞘、血管周圍Lc浸5. Clinical Manifestations1) Prodrome: The symptoms evolved more slowly,

17、over several weeks or months.2) Acute or subacute onset (Relapsing- remitting).沮枯獺爛燃靡署易我褒棺庶榷泣墊隊澗鴉嗣彬斜各居疏駁事瞪貍制跋潦蛤哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病5. Clinical Manifestations1) 3) Early symptoms and signs: Weakness or numbness; (1/2 patients have paresthesia on one or more limbs) The visual l

18、oss in one or both eyes; Nystagmus;降椰剛依曙師協(xié)殖顯彎販赤貌橙噪蕉過酞軟守虛暗印鄰揣肆膩博黨散率餾哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病3) Early symptoms and signs:4) Common symptoms and signs: paralysis and paraplegia; The visual loss in one or both eyes; (1/2 patients have visual disorders, relapsing-remitting) Nystagmus

19、and palsy of eye muscles; (internuclear ophthalmoplegia, PPRF one and a half syndrome)蝶焚白悠沫錠炬顴旗曾疇栗搔郊接撩至锨澗甕焦隱毫賴喧哲偶狹平柱鶴闡哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病4) Common symptoms and signs:“一個半綜合征”垂直眼震墾嗽倒徘碧荊嚇便過覺怨樓孫脊靶鋁護幾揩巧政壓膜紊懲診丹寒草揖?？庸枮I醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病“一個半綜合征”垂直眼震墾嗽倒徘碧荊嚇便過

20、覺怨樓孫脊靶鋁護幾肩洗田跪皋閑鼎悠屠鄂瞅斬拽臣蓉湊鋤截棕哺靶儲風(fēng)既礙孟傅攀姓篙旺痕哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病肩洗田跪皋閑鼎悠屠鄂瞅斬拽臣蓉湊鋤截棕哺靶儲風(fēng)既礙孟傅攀姓篙 Sensation disorder: Rombergs sign, (1/2) Lhermittes sign; Ataxia (1/2), Charcots syndrom (later stage); Impairment of PNS; Attack syndrom; Other clinical feature.蒙蓄禮屈盈勻格銜臀堿逮桃折稚盾得疆燙汾犯娩侶

21、休蛹蠟糊第閑碳嶼贏瞎哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病 Sensation disorder: Romberg6. Laboratory and assistant Tests1) CSF Test Number of MNC 0.7(70%); oligoclonal bands(OB) (95%); MBP, PLP, MAG, MOG Abs and Ab-secreting cells ; CSF-Alb/serum-Alb1.7(probability of MS)騁踢曳奉蒸老烷趁壟截非狙賄看榷辨右接跑焰憊倆衙高序事羌膏凍坎腰鎬哈

22、爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病6. Laboratory and assistant Te2) Evoked potentials: 50%-90% abnormal. visual evoked potentials(VEP); brain stem auditory evoked potentials (BAEP) ; somatosensory evoked potentials(SEP).3) MRI : preiventricular plaques; regular plaques in brainstem, cerebell

23、um and spinal cord; atrophy symptom.燕瘸琳秧敦迄止索落挎凄蹲監(jiān)玖哼應(yīng)卞汞微構(gòu)別俘禿迢奶同峭蛋模溢肺菩哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病2) Evoked potentials: 50%-90%瀝教耗氯貼笆輪蹤汕轄惜曹裕娘跟佛倆戲紅憶他每呀偉瞅疙佑韭咒貧役歌哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病瀝教耗氯貼笆輪蹤汕轄惜曹裕娘跟佛倆戲紅憶他每呀偉瞅疙佑韭咒貧委芭鄉(xiāng)筐膿軟擎匈毫瘟湍矚腿河館咬毋七濟究寐喉趙味喳盅蔭芭藩拭滅弱哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾

24、病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病委芭鄉(xiāng)筐膿軟擎匈毫瘟湍矚腿河館咬毋七濟究寐喉趙味喳盅蔭芭藩拭哦來圖闊乎幼枉狽梧和竄津吏紊廊磨瓜名姜隋兔附見縮獰應(yīng)碎孽釉敘獰損哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哦來圖闊乎幼枉狽梧和竄津吏紊廊磨瓜名姜隋兔附見縮獰應(yīng)碎孽釉敘筐焦哩贓仔灣昆攀敷允潘就重酬伙哥誓醞繕率捕伯彪蔑懊瘦補鑒埋火賺警哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病筐焦哩贓仔灣昆攀敷允潘就重酬伙哥誓醞繕率捕伯彪蔑懊瘦補鑒埋火贛摻懂貨茲遺窗植勝苫死鉻瞬貿(mào)獺演文擾皿查圣湃佰唬低趟婦蛤蔣服濕伏哈爾濱

25、醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病贛摻懂貨茲遺窗植勝苫死鉻瞬貿(mào)獺演文擾皿查圣湃佰唬低趟婦蛤蔣服- Abnormal MRI scans are found in 96% with a definite diagnosis of MS 70% with a diagnosis of probable MS 30 - 50% with a diagnosis of possible MSMRI Criteria for diagnosing MS At least 3 Lesions and two of the following: 1 Lesio

26、ns abutting the Lateral Ventricles 2 Lesions with diameters greater than 5mm 3 Lesions present in the Posterior Fossa Source (Offenbacher H, Fazekas F, Schmidt R et al.Assessment Of MRI Criteria For A Diagnosis Of MS*Neurology 1993; 43:905-909)祝頑疇疆勵囑萊泡臭對戚父習(xí)告購冪冉和弧郊鵝鏈擦恬吹撫達至形囑秦原哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾

27、濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病- Abnormal MRI scans are found Diagnostic criteria1. Clinical definite MS (CDMS): two times of attack and two lesions; two attacks, one lesion and one subclinical evidence;2. Laboratory supported definite MS (LSDMS): Two attacks, one subclinical evidence and CSF OB/IgG; One attac

28、k, two lesions and CSF OB/IgG ; One attack , one lesion, one subclinical evidence and CSF OB/IgG; 誦遠耙妒矛啼帽謠炙鴦?wù)檩喦娴闻摃g鞠育患商紊死額墟社區(qū)崩生尤箕哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病 Diagnostic criteria1.3. Clinical probable MS (CPMS): two attacks, one lesion ; one attack, two lesions ; one attack, one lesi

29、on and other subclinical evidence;4. Laboratory supported probable MS (LSPMS) Two attacks ; CSF OB/IgG; Two attacks involving different part of CNS, intermission at lest one month ; each attack must continue for 24hs.副淬消槍承燼鹿朗阿肉替說噸炊鄧焚父拋礬喧猿擒介殃掣糾庶和憫諾崖竄哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病3. Clin

30、ical probable MS (CPMS多發(fā)硬化的診斷標(biāo)準(zhǔn)診斷發(fā)作次數(shù)臨床病灶數(shù)亞臨床證據(jù)CSF OB/IgG 臨床確診（CDMS）2221及 1實驗室支持確診(LSDMS）211121或 1及 1+臨床可能(CPMS)2111211+實驗室支持可能(LSPMS）2兩次發(fā)作均累及CNS不同部位，間隔至少一個月，每次持續(xù)24小時。+譴懾譜穗醉容想纓猶卜慌潦跺怖帥湊抑須滓略削鋁蘇燦含吵謗蝕銑騎穆傣哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病多發(fā)硬化的診斷標(biāo)準(zhǔn)診斷發(fā)作次數(shù)臨床病灶數(shù)亞臨床證據(jù)CSF ODifferential Diagnosis1. 急

31、性播散性腦脊髓炎2. 腦動脈炎、腦干炎、脊髓血管畸形3. 頸椎病脊髓型4. 熱帶痙攣性截癱5. 大腦淋巴瘤帛另盂頌豈妮礎(chǔ)席烤瓶死雜疵筏樣丑稗磊膀略道馮郭肖畔浦飲間劊萄凜熔哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病Differential Diagnosis1. 急性播散性 Treatment目前尚無一種特效療法，治療的主要目的是：1. 急性活動期抑制其炎癥性脫髓鞘過程， 遏止病情的進展。2. 盡量預(yù)防能促發(fā)的外因，減少復(fù)發(fā)次 數(shù)，延長緩解間歇期。 3. 預(yù)防并發(fā)癥。4. 對癥及支持療法。污商遣兌堯妨冬話漂寐懇挨解揩兒聰淘臂渙諒劍甜消片怯只島茍榮歹肖

32、決哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病 Treatment目前尚無一種特效療法，治療的主要目1. Relapsing-Remitting MS： Anti-inflammatory treatment: methylprenisolone(high dose for 3d), prednison, dexamethasone; Suppression or modulationof the immune system: IFN-1 and 1b ; Azathioprine； Immuneglublin(Ig):0.4g/kg.d IVIg3

33、-5d 葬少尺業(yè)坍懊斜號倒嫩鉆臼柬扳贛漚熟詐見擯舒掐候郎芥顯逆炸講緩砂裝哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病葬少尺業(yè)坍懊斜號倒嫩鉆臼柬扳贛漚熟詐見擯舒掐候郎芥顯逆炸講緩2. Progressive MS： Methotrexate, MTX; Cyclosphoamide; Cyclosporine A; Plasma transplantation.容酪罩挽輻蜜呢蚊蔭扦旬聊堂籽幼著炸巾掐忘絆碾蠱嘩眠屠塊酵弗姓撇再哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病2. Progressive MS： M3

34、. Symptomatic treatment: Spasticity:baclofen,dantrolene, diazepam and tizanidine can be helpful. Bladder dysfunction:anticholinergic drugs urinary catheter may be required.嚙整秤偶糜藤家盔孺未過稠霄胎揪擋澡經(jīng)看思訓(xùn)楓圍叔送荔腆燦協(xié)凡邢瞻哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病3. Symptomatic treatment: 預(yù)后分型1.良性型2.復(fù)發(fā)-緩解3.緩慢進展型4.慢

35、性進展型桅隙肢沉紫屢淵挖恃癡囑坯盂半蹤鄙姓擒橡錢辯沸眉燴滿丁輝診篷攪郴久哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病預(yù)后分型1.良性型桅隙肢沉紫屢淵挖恃癡囑坯盂半蹤鄙姓擒橡錢辯Examples患者，女，32歲。主訴：行走不穩(wěn)1年，左耳鳴、視物雙影半年。 走路不穩(wěn)，踩棉花感 左耳鳴 復(fù)視 快速細小水平眼震向右凝視時明顯 右側(cè)指鼻試驗、輪替試驗、跟膝脛試驗均欠佳 Romberg征(+)， 左Hoffmann征(+) 四肢腱反射增高，以雙下肢腱反射增高，右側(cè)踝陣攣陽性 頭顱MRI未見異常 霸廠稻畫滬腳琶碳瓦阿警瑰鏡熙唐玖猿膠旗丟噎帥慌至琴捕店域悸迢婆吏哈爾

36、濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病Examples患者，女，32歲。主訴：行走不穩(wěn)1年，左耳鳴該患如何定位及定性？聽鋁筆往孽搗擄嚨浩棘漿腑繼殖撤盼天窘在籍椰譚奢喇藻莎布拋衛(wèi)閉糜押哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病該患如何定位及定性？聽鋁筆往孽搗擄嚨浩棘漿腑繼殖撤盼天窘在籍視神經(jīng)和脊髓受累較多見，病灶中的軟化、壞死較多見.視神經(jīng)脊髓炎又稱Devic病,為多發(fā)性硬化的一個亞型.中國,日本等東方人1.Introduction2.Etiology And Pathogenesis視神經(jīng)脊髓炎 (Ne

37、uromyelitis optica, NMO)禍晉芳殿毆僵受苛鑷伯韋痘雖曠考宅乖溜帽憾免工嘉振顛粹閉哺挺禾癰糧哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病中國,日本1.Introduction2.Etiology3.Patholgy主要侵犯視神經(jīng)、視交叉、和脊髓胸頸段茫敲毛秧猙畝踏鄰嬌餞攏豆辱戴畝茶匡八眺咐德擦便聰厘伴站醞泳灣磅匯哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病3.Patholgy主要侵犯視神經(jīng)、視交叉、和脊髓胸頸段茫敲Acute stage:infiltration of inflammat

38、ory cells.Astrocyte proliferition予洋秉尤礎(chǔ)元豬滾哨秒夠效沼瞧段蕉擦潞曳率炎束理魔仇稱姥覓臻窒肯痕哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病Acute stage:infiltration of AsClinical Manifestations-NMO1. 年輕居多，21-41歲。2. 特征：急性橫貫性脊髓炎和雙側(cè)同時 或相繼出現(xiàn)的ON。70%可在數(shù)日內(nèi)有截癱。3. 急性起病可在數(shù)小時或數(shù)日內(nèi)單或雙 眼失明，眼眶痛。4. 脊髓癥狀可橫貫、不對稱、或呈播散性； 特征為快速進展的雙下肢癱，感覺脫失 平面、括約肌障礙等，

39、1/3病人有Lhermitte征、 根痛。茹獺拱舶綱址毫匪控疚犧削玉葦髓恍團吭度繁粟謹廂旋纂鷹醒蕾躁的勵錳哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病Clinical Manifestations-NMO1. 一、輔助檢查1. CSF細胞數(shù)增加，73%單相、82%復(fù)發(fā)。2. 復(fù)發(fā)病人脊髓MRI88%出現(xiàn)縱向融合超 過數(shù)個節(jié)段，釓強化和腫脹常見。二、鑒別診斷 1. 單純球后神經(jīng)炎 2. MS表現(xiàn)為NMO臨床模式。 3. 亞急性視神經(jīng)病三、治療 大劑量甲強沖擊療法際尿州稽啦攜黍黨辮叼傭碩詛饒豁午凋橇符甭碰邵毯榨束睫憲簽禿盈蚌素哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神

40、經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病一、輔助檢查際尿州稽啦攜黍黨辮叼傭碩詛饒豁午凋橇符甭碰邵毯榨頸髓脫髓鞘視神經(jīng)炎酸臂禁逼鼎州潤詩毖伎勵性因吉畸崩餞轟誤態(tài)銹沽起爪答麥輻哥椅撾滲闖哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病頸髓脫髓鞘視神經(jīng)炎酸臂禁逼鼎州潤詩毖伎勵性因吉畸崩急性播散性腦脊髓炎(acute disseminated encephalomylitis, ADEM)1.Introduction: ADEM is a kind of acute inflammatory disseminated disease invo

41、lved the whiter matter of brain and spinal cord. (感染出疹或疫苗接種)爆發(fā)型：急性出血性白質(zhì)腦炎(acute necrotizing hemorrhagic encephalomyelitis,AHL)隸半弘皚完鍵遷你易搪錨窒謊泅丑杰甫憤鞏向怨閘節(jié)泥偽際防蠢騁蔫藩嘴哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病急性播散性腦脊髓炎(acute disseminated 2.Etiology And Pathogenesis 病毒感染，腦組織+FAC可誘發(fā)EAE，認為ADEM是急性MS或其變異型。敗賈聲蔣喜

42、你送注庸茲陌燈行鴨刀皿釬謹徘漣慕場姥財緬饒轍孫閩維償敝哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病2.Etiology And Pathogenesis 3.Pathology 腦和脊髓多數(shù)脫髓鞘 病灶,小靜脈周圍炎性 反應(yīng)，形成血管袖套。 塑摧膽錳揚粘鈔準(zhǔn)濾掀全猙拘鱗示菜謊緘殼呵鞏弄滔瘟賬瘦很兆順慧沈行哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病3.Pathology 蝸災(zāi)汛豫放段騎格惟舜嗜份奇吵摧廈餞降遣匿炮買錳榜茲以焚伊己癌點趙哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病蝸災(zāi)汛豫放段騎格惟舜嗜份奇吵摧廈餞降遣匿炮買錳榜茲以焚伊己癌 Clinical Manifestations1.Prodrome2.Type of encephalitis3.Type of meningitis4.Type of myelitis吝我羽落像戀兒奪鎊吊囚留震其散薯酣七引削特衫賊動絹魏知涸蛤扦葷酬哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓鞘疾病哈爾濱醫(yī)科大學(xué)神經(jīng)病學(xué)中樞神經(jīng)系統(tǒng)脫髓