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

1、中樞神經(jīng)系統(tǒng)脫髓鞘疾病demyelinating diseases of the central nervous system 掌握ms概念、病因、發(fā)病機(jī)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 of cns chapter 1 intraduction 1. concept: a group of disease characterized by demyelinating of the bra

2、in and spinal cord. pathology: demyelination 髓 鞘 構(gòu) 成cnspns2. pathologic findings destruction of the myelin sheaths of cns; often primarily in white matter, either in multiple small disseminated foci or in larger foci ; infiltration of inflammatory cells in a perivenous distribution; a relative integ

3、rity of the axis cylinders in the lesions and a lack of wallerian, the secondary degeneration of fiber tracts.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) 多發(fā)性硬化癥(ms) 多發(fā)性硬化mul

4、tiple sclerosis,ms 1. concept: ms is a kind of autoimmune diseases characterized by demyelination of cns. due to its high incidence, chronicity and tendency to attack young adults, it has become one of the most important cns diseases. there are multiple areas of demyelination within the cns.the epis

5、odes of demyelination are separated in time and place,and classically the disease runs a relapsing-remitting course. (brain and spinal cord) 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(shí)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2. etiology and pathogenesis 1) 病毒感染及自身免疫反應(yīng)：since the exact cause is uncertain. immunologi

6、cal mechanisms undoubtedly play a role,although the causation is probably multifactorial. 麻疹病毒,人類噬 t 淋巴細(xì)胞病毒(htlv-i) ,分子模擬, 細(xì)胞免疫及體液免疫。 2) 遺傳因素 (inherited factor) 3) 環(huán)境因素 (environment)3. epidemiology incidence of ms associated with latitude. on moving from a high-prevalence area to a low-prevalence ar

7、ea prior to puberty,the risk of developing ms is higher than in the low-prevalence area; however the move is made following puberty, the risk of the high-prevalence is retained. heredity may be an important factor. ms associated with the hla-dr locus on the sixth chromosome, hla-dr2 express strongly

8、 and then -dr3 , b7 and a3 .characteristic: multiple demyelinated plaques. position: white matter around the lateral ventricles and spinal cord, optic nerve, brain stem and cerebellar.acute stage: hyperemia,ondema,demyelination, infiltriation of inflammatory cells distributed in perivenous. recovery

9、 stage : astrocyte proliferition, forming of astrocytic scab. 急急 性性 期期: : 充血、水腫、炎性脫髓鞘、血管周圍lc浸潤。 恢恢 復(fù)復(fù) 期期: : 星狀細(xì)胞增生、膠質(zhì)斑痕形成。 肉眼觀：cns內(nèi)脫髓鞘斑塊5. clinical manifestations 1) prodrome: the symptoms evolved more slowly, over several weeks or months. 2) acute or subacute onset (relapsing- remitting).3) early s

10、ymptoms and signs: weakness or numbness; (1/2 patients have paresthesia on one or more limbs) the visual loss in one or both eyes; nystagmus;4) common symptoms and signs: paralysis and paraplegia; the visual loss in one or both eyes; (1/2 patients have visual disorders, relapsing-remitting) nystagmu

11、s and palsy of eye muscles; (internuclear ophthalmoplegia, pprf one and a half syndrome)“一個(gè)半綜合征一個(gè)半綜合征”垂直眼震垂直眼震 sensation disorder: rombergs sign, (1/2) lhermittes sign; ataxia (1/2), charcots syndrom (later stage); impairment of pns; attack syndrom; other clinical feature.6. laboratory and assistant

12、 tests 1) 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) 2) evoked potentials: 50%-90% abnormal. visual evoked potentials(vep); brain stem auditory evoked potentials (baep) ; somatosensory evoked p

13、otentials(sep). 3) mri : preiventricular plaques; regular plaques in brainstem, cerebellum and spinal cord; atrophy symptom.- 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 ms- mri criteria for diagnosing

14、ms at least 3 lesions and two of the following: 1 lesions 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)

15、diagnostic criteria 1. 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 attack, two lesions and csf ob/igg ; one attack , one

16、lesion, one subclinical evidence and csf ob/igg; 3. clinical probable ms (cpms): two attacks, one lesion ; one attack, two lesions ; one attack, one lesion and other subclinical evidence; 4. laboratory supported probable ms (lspms) two attacks ; csf ob/igg; two attacks involving different part of cn

17、s, intermission at lest one month ; each attack must continue for 24hs.多發(fā)硬化的診斷標(biāo)準(zhǔn)多發(fā)硬化的診斷標(biāo)準(zhǔn)兩次發(fā)作均累及兩次發(fā)作均累及cns不同部位，不同部位，間隔至少一個(gè)月，間隔至少一個(gè)月，每次持續(xù)每次持續(xù)24小時(shí)。小時(shí)。differential diagnosis 1. 急性播散性腦脊髓炎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 treatment anti-inflammatory treatment: methylprenisolone(high dose for

18、 3d), prednison, dexamethasone; suppression or modulationof the immune system: ifn-1 and 1b ; azathioprine； immuneglublin(ig):0.4g/kg.d ivig3-5d 2. progressive ms： methotrexate, mtx; cyclosphoamide; cyclosporine a; plasma transplantation.3. symptomatic treatment: spasticity:baclofen,dantrolene, diaz

19、epam and tizanidine can be helpful. bladder dysfunction:anticholinergic drugs urinary catheter may be required.預(yù)后分型 1.良性型 2.復(fù)發(fā)-緩解 3.緩慢進(jìn)展型 4.慢性進(jìn)展型examples患者，女，患者，女，32歲。主訴：行走不穩(wěn)歲。主訴：行走不穩(wěn)1年，左耳鳴、視物雙影半年。年，左耳鳴、視物雙影半年。 走路不穩(wěn)，踩棉花感走路不穩(wěn)，踩棉花感 左耳鳴左耳鳴 復(fù)視復(fù)視 快速細(xì)小水平眼震向右凝視時(shí)明顯快速細(xì)小水平眼震向右凝視時(shí)明顯 右側(cè)指鼻試驗(yàn)、輪替試驗(yàn)、跟膝脛試驗(yàn)均欠佳右側(cè)指鼻試驗(yàn)、

20、輪替試驗(yàn)、跟膝脛試驗(yàn)均欠佳 romberg征征(+)， 左左hoffmann征征(+) 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 頭顱頭顱mri未見未見異常異常 視神經(jīng)和脊髓受累較多見，病灶中的軟化、視神經(jīng)和脊髓受累較多見，病灶中的軟化、壞死較多見壞死較多見. .視神經(jīng)脊髓炎又稱視神經(jīng)脊髓炎又稱devicdevic病病, ,為為多發(fā)性硬化的一個(gè)亞型多發(fā)性硬化的一個(gè)亞型. .中國中國, ,日本日本等東方人等東方人1.introductionetiology and pathogenesis視神經(jīng)脊髓炎 (neuromyelitis op

21、tica, nmo)3.patholgy主要侵犯視神經(jīng)、視交叉、和脊髓胸頸段主要侵犯視神經(jīng)、視交叉、和脊髓胸頸段acute stage:infiltration of inflammatory cells.astrocyte proliferitionclinical manifestations-nmo 1. 年輕居多，年輕居多，21-41歲。歲。 2. 特征：急性橫貫性脊髓炎和雙側(cè)同時(shí)特征：急性橫貫性脊髓炎和雙側(cè)同時(shí) 或相繼出現(xiàn)的或相繼出現(xiàn)的on。70%可在數(shù)日內(nèi)有截癱?？稍跀?shù)日內(nèi)有截癱。 3. 急性起病可在數(shù)小時(shí)或數(shù)日內(nèi)單或雙急性起病可在數(shù)小時(shí)或數(shù)日內(nèi)單或雙 眼失明，眼眶痛。眼失明，眼眶

22、痛。 4. 脊髓癥狀可橫貫、不對稱、或呈播散性；脊髓癥狀可橫貫、不對稱、或呈播散性； 特征為快速進(jìn)展的雙下肢癱，感覺脫失特征為快速進(jìn)展的雙下肢癱，感覺脫失 平面、括約肌障礙等，平面、括約肌障礙等，1/3病人有病人有l(wèi)hermitte征、征、 根痛。根痛。 一、一、 1. 1. csfcsf細(xì)胞數(shù)增加，細(xì)胞數(shù)增加，73%73%單相、單相、82%82%復(fù)發(fā)。復(fù)發(fā)。 2. 2. 復(fù)發(fā)病人脊髓復(fù)發(fā)病人脊髓mri88%mri88%出現(xiàn)縱向融合超出現(xiàn)縱向融合超 過數(shù)個(gè)節(jié)段，釓強(qiáng)化和腫脹常見。過數(shù)個(gè)節(jié)段，釓強(qiáng)化和腫脹常見。 1. 1. 單純球后神經(jīng)炎單純球后神經(jīng)炎 2. 2. msms表現(xiàn)為表現(xiàn)為nmonmo臨床模式。臨床模式。 3. 3. 亞急性視神經(jīng)病亞急性視神經(jīng)病 大劑量甲強(qiáng)沖擊療法大劑量甲強(qiáng)沖擊療法頸髓脫髓鞘視神經(jīng)炎感染出疹或疫苗接種感染出疹或疫苗接種) ) 爆發(fā)型：急性出血性白質(zhì)腦炎爆發(fā)型：急性出血性白質(zhì)腦炎 ( (acute necrotizing hemorrhagic encephalomyelitis,ahl)etiolog