哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病课件

1、哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病1中枢神经系统脱髓鞘疾病demyelinating diseases of the central nervous system 哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病2 掌握ms概念、病因、发病机制、临床表现、辅助检查、治疗、诊断标准及鉴别诊断。 熟悉视神经脊髓炎概念、临床表现、辅助检查、诊断及治疗。 了解ms病理、预后；急性播散性脑脊髓炎概念、临床表现、诊断及治疗。key points-demyelinating diseases of cns哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病3 chapter 1 intraduction 1.

2、 concept: a group of disease characterized by demyelinating of the brain and spinal cord. pathology: demyelination 哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病4髓 鞘 构 成cnspns哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病5哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病6哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病72. pathologic findings destruction of the myelin sheaths of cns; often primarily

3、 in white matter, either in multiple small disseminated foci or in larger foci ;哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病8 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.哈尔滨医科

4、大学神经病学中枢神经系统脱髓鞘疾病9临床常见脱髓鞘疾病急性播散性脑脊髓炎(acute disseminated encephalomyelitis, adem )多发性硬化症(multiple sclerosis, ms)亚型视神经脊髓炎( devic diseases )急性出血性白质脑病(acute hemorrhage leukoencephalitis, ahle) 哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病10 多发性硬化症(ms) 哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病11多发性硬化multiple sclerosis,ms 1. concept: ms is a kind

5、 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.哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病12 there are multiple areas of demyelination within the cns.the episodes of demyelinati

6、on are separated in time and place,and classically the disease runs a relapsing-remitting course. (brain and spinal cord) 哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病13是一种常见以中枢神经系统炎性脱髓鞘为特征的自身免疫性疾病病灶部位及时间上的多发性多数均以反复多次发作与缓解的病程具有免疫易感性、年轻人多见哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病142. etiology and pathogenesis 1) 病毒感染及自身免疫反应：since the exact c

7、ause is uncertain. immunological mechanisms undoubtedly play a role,although the causation is probably multifactorial. 麻疹病毒,人类噬 t 淋巴细胞病毒(htlv-i) ,分子模拟, 细胞免疫及体液免疫。 2) 遗传因素 (inherited factor) 3) 环境因素 (environment)哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病15哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病16哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病173. epidemiology i

8、ncidence 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 risk of the high-prevalence is retained.哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病

9、18 heredity 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 .哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病19哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病20characteristic: multiple demyelinated plaques. position: white matter around the lateral ventricles

10、 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. 哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病21 急急 性性 期期: : 充血、水肿、炎性脱髓鞘、血管周围lc浸润。 恢恢 复复

11、期期: : 星状细胞增生、胶质斑痕形成。 肉眼观：cns内脱髓鞘斑块哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病225. clinical manifestations 1) prodrome: the symptoms evolved more slowly, over several weeks or months. 2) acute or subacute onset (relapsing- remitting).哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病233) early symptoms and signs: weakness or numbness; (1/2 patients

12、 have paresthesia on one or more limbs) the visual loss in one or both eyes; nystagmus;哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病244) common symptoms and signs: paralysis and paraplegia; the visual loss in one or both eyes; (1/2 patients have visual disorders, relapsing-remitting) nystagmus and palsy of eye muscles; (i

13、nternuclear ophthalmoplegia, pprf one and a half syndrome)哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病25“一个半综合征一个半综合征”垂直眼震垂直眼震哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病26哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病27 sensation disorder: rombergs sign, (1/2) lhermittes sign; ataxia (1/2), charcots syndrom (later stage); impairment of pns; attack syndrom; other cl

14、inical feature.哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病286. laboratory and assistant 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)哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病29 2) evoked potentials: 50%-90% abnormal. visual evo

15、ked potentials(vep); brain stem auditory evoked potentials (baep) ; somatosensory evoked potentials(sep). 3) mri : preiventricular plaques; regular plaques in brainstem, cerebellum and spinal cord; atrophy symptom.哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病30哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病31哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病32哈尔滨医科大学神经病学中枢神

16、经系统脱髓鞘疾病33哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病34哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病35- 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 ms at least 3 lesions and two of the following: 1 lesions abutting

17、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)哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病36 diagnostic criteria 1. clinical definite ms

18、 (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 lesion, one subclinical evidence and csf ob

19、/igg; 哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病37 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 cns, intermission at

20、 lest one month ; each attack must continue for 24hs.哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病38多发硬化的诊断标准多发硬化的诊断标准两次发作均累及两次发作均累及cns不同部位，不同部位，间隔至少一个月，间隔至少一个月，每次持续每次持续24小时。小时。哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病39differential diagnosis 1. 急性播散性脑脊髓炎2. 脑动脉炎、脑干炎、脊髓血管畸形 3. 颈椎病脊髓型4. 热带痉挛性截瘫5. 大脑淋巴瘤哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病40 treatment哈尔滨医科大学

21、神经病学中枢神经系统脱髓鞘疾病41 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 ivig3-5d 哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病422. progressive ms： methotrexate, mtx; cyclosphoamide;

22、 cyclosporine a; plasma transplantation.哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病433. symptomatic treatment: spasticity:baclofen,dantrolene, diazepam and tizanidine can be helpful. bladder dysfunction:anticholinergic drugs urinary catheter may be required.哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病44预后分型 1.良性型 2.复发-缓解 3.缓慢进展型 4.慢性进展型哈尔滨医科

23、大学神经病学中枢神经系统脱髓鞘疾病45examples患者，女，患者，女，32岁。主诉：行走不稳岁。主诉：行走不稳1年，左耳鸣、视物双影半年。年，左耳鸣、视物双影半年。 走路不稳，踩棉花感走路不稳，踩棉花感 左耳鸣左耳鸣 复视复视 快速细小水平眼震向右凝视时明显快速细小水平眼震向右凝视时明显 右侧指鼻试验、轮替试验、跟膝胫试验均欠佳右侧指鼻试验、轮替试验、跟膝胫试验均欠佳 romberg征征(+)， 左左hoffmann征征(+) 四肢腱反射增高，以双下肢腱反射增高，右侧踝阵挛阳性四肢腱反射增高，以双下肢腱反射增高，右侧踝阵挛阳性 头颅头颅mri未见未见异常异常 哈尔滨医科大学神经病学中枢神经

24、系统脱髓鞘疾病46该患如何定位及定性？哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病47视神经和脊髓受累较多见，病灶中的软化、视神经和脊髓受累较多见，病灶中的软化、坏死较多见坏死较多见. .视神经脊髓炎又称视神经脊髓炎又称devicdevic病病, ,为为多发性硬化的一个亚型多发性硬化的一个亚型. .中国中国, ,日本日本等东方人等东方人1.introductionetiology and pathogenesis视神经脊髓炎 (neuromyelitis optica, nmo)哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病483.patholgy主要侵犯视神经、视交叉、和脊髓胸颈段主要侵犯视神

25、经、视交叉、和脊髓胸颈段哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病49acute stage:infiltration of inflammatory cells.astrocyte proliferition哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病50clinical manifestations-nmo 1. 年轻居多，年轻居多，21-41岁。岁。 2. 特征：急性横贯性脊髓炎和双侧同时特征：急性横贯性脊髓炎和双侧同时 或相继出现的或相继出现的on。70%可在数日内有截瘫。可在数日内有截瘫。 3. 急性起病可在数小时或数日内单或双急性起病可在数小时或数日内单或双 眼失明，眼眶痛。眼失

26、明，眼眶痛。 4. 脊髓症状可横贯、不对称、或呈播散性；脊髓症状可横贯、不对称、或呈播散性； 特征为快速进展的双下肢瘫，感觉脱失特征为快速进展的双下肢瘫，感觉脱失 平面、括约肌障碍等，平面、括约肌障碍等，1/3病人有病人有lhermitte征、征、 根痛。根痛。哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病51 一、一、 1. 1. csfcsf细胞数增加，细胞数增加，73%73%单相、单相、82%82%复发。复发。 2. 2. 复发病人脊髓复发病人脊髓mri88%mri88%出现纵向融合超出现纵向融合超 过数个节段，钆强化和肿胀常见。过数个节段，钆强化和肿胀常见。 1. 1. 单纯球后神经炎单

27、纯球后神经炎 2. 2. msms表现为表现为nmonmo临床模式。临床模式。 3. 3. 亚急性视神经病亚急性视神经病 大剂量甲强冲击疗法大剂量甲强冲击疗法哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病52颈髓脱髓鞘视神经炎哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病53感染出疹或疫苗接种感染出疹或疫苗接种) ) 爆发型：急性出血性白质脑炎爆发型：急性出血性白质脑炎 ( (acute necrotizing hemorrhagic encephalomyelitis,ahl)哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾病54etiology and pathogenesis 病毒感染，脑组织病毒感染，脑组织+ +facfac可诱发可诱发eaeeae，认为认为ademadem是急性是急性msms或其变异型。或其变异型。哈尔滨医科大学神经病学中枢神经系统脱髓鞘疾