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1、中枢神经系统脱髓鞘疾病 Demyelinating Diseases of the Central Nervous System,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,Chapter 1 Intraduction,1. Concept: A group of disease characterized by demyelinating of the b
2、rain and spinal cord.PATHOLOGY: Demyelination,髓 鞘 构 成,CNS,PNS,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 ;, Infiltration of inflammatory cells in a perivenous distribution; A relative
3、 integrity of the axis cylinders in the lesions and a lack of wallerian,the secondary degeneration of fiber tracts.,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),多发性硬化症(
4、MS),多发性硬化 Multiple 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
5、 CNS.The episodes of demyelination are separated in time and place,and classically the disease runs a relapsing-remitting course. (brain and spinal cord),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,2. Etiology And Pathogenesis,1) 病毒感染及自身免疫反应：Since the exact cause is
6、 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),3. Epidemiology,Incidence of MS associated with latitude. On moving from a high-prevalence area t
7、o 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.,Heredity may be an important factor. MS associated with the HLA-DR locus on the sixth chromosome, HLA
8、-DR2 express strongly and then -DR3 , B7 and A3 .,4. Pathologic Findings,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 inflamma
9、tory cells distributed in perivenous. Recovery stage : Astrocyte proliferition, forming of astrocytic scab.,急 性 期: 充血、水肿、炎性脱髓鞘、血管周围Lc浸润。 恢 复 期: 星状细胞增生、胶质斑痕形成。 肉眼观：CNS内脱髓鞘斑块,5. Clinical Manifestations,1) Prodrome: The symptoms evolved more slowly, over several weeks or months. 2) Acute or subacute on
10、set (Relapsing-remitting).,3) Early symptoms 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 di
11、sorders, relapsing-remitting) Nystagmus and palsy of eye muscles; (internuclear ophthalmoplegia, PPRFone and a half syndrome),“一个半综合征”,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 featu
12、re.,6. 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),2) Evoked potentials: 50%-90% abnormal. visual evoked potentials(VEP); brain stem auditory evoked potentials (
13、BAEP) ; somatosensory evoked potentials(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
14、 MRI Criteria for diagnosing MSAt 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
15、*Neurology 1993; 43:905-909),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 C
16、SF OB/IgG ; One attack , one 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 CNS, intermission at lest one month ; each attack must continue for 24hs.,
