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1、 中枢神经系统脱髓鞘疾病 Demyelinating Diseases of the Central Nervous System 哈医大二院神经科 Chapter 1 Intraduction 1.Concept A group of diseases of the brain and spinal cord in which demyelination is a prominent feature.2.Pathologic Findings Destruction of the myelin sheaths of nerves;Infiltration of inflammatory ce
2、lls in a perivascular distribution;A particular distribution of lesion,often perivenous and primarily in white matter,either in multiple small disseminated foci or in larger foci;A relative integrity of the axis cylinders in the lesions and a lack of wallerian,the secondary degeneration of fiber tra
3、cts.多发性硬化Multiple Sclerosis 1.Concept Multiple Sclerosis 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 diseases of CNS.2.Etiology And Pathogenesis 1)病毒感染及自身免疫反应：麻
4、疹病毒、人类噬 T 淋巴细胞病毒(HTLV-I)分子模拟、细胞免疫、体液免疫(1,2,3,4)2)遗传因素(inherited factor)3)环境因素(environment)(4)3.EpidemiologynIncidence of MS associated with latitude.nContacting with some kind of environment may play an important role in the incidence of MS.nHeredity may be an important factor.nMS associated with th
5、e HLA-DR locus on the sixth chromosome,HLA-DR2 express strongly and then-DR3,B7 and A3.4.Pathologic Findings Characteristic:Multiple demyelinated plaques in white matter of CNS.Position:White matter around the lateral ventricles and spinal cord,optic nerve,brain stem and cerebellar.Acute stage:hyper
6、emia,ondema,demyelination,infiltration of inflammatory cells in perivascular distribution.Recovery stage:Astrocyte proliferition,forming of astrocytic scab.小脑及桥脑脱髓鞘病灶桥脑脱髓鞘病灶5.Clinical Manifestations 1)Prodrome:The symptoms evolved more slowly,over several weeks or months.2)Acute or subacute onset Re
7、lapsing-remitting.3)Early symptoms and signs:Weakness or numbness,sometimes both,in one or more limbs is the initial symptom in about half of the patients;The visual loss in one or both eyes;Nystagmus(fig1)and palsy of eye muscles(internuclear ophthalmoplegia,one and a half syndrome)Impairment of ot
8、her brain nerves;Ataxia:Charcots syndrom Sensation disorder:Rombergs sign,Lhermittes sign;Attack syndrom Other clinical feature6.Laboratory and other assistant Tests 1.CSF Test 1)Number of MNC 0.7;oligoclonal bands(OB)positive;3)MBP,PLP,MAG,MOG Abs and Ab-secreting cells 4)CSF-Alb/serum-Alb1.7(proba
9、bility of MS)2.Evoked potentials:visual evoked 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.(1,2,3,4,5,6)Diagnostic criteria 1.Clinical definite
10、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 lesion,one subclinical evidence and CSF OB/IgG;3.Clinica
11、l 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.Differe
12、ntial 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 Treatment 目前尚无一种特效疗法，治疗的主要目的是：1.急性活动期抑制其炎症性脱髓鞘过程，遏止病情的进展。2.尽量预防能促发的外因，减少复发次 数，延长缓解间歇期。3.预防并发症。4.对症及支持疗法。1.Relapsing-Remitting MS：ACTH and Steoids：methylprenisolone,prednison,dexamethasone;IFN-;Azathioprine；Immuneglublin（Ig).2.Prog
13、ressive MS：Methotrexate,MTX;Cyclosphoamide;Cyclosporine A;Plasma transplantation.3.对症治疗及预防感染避免疲劳：如过度 劳累、紧张、疫苗接种、妊娠、分娩等。视神经脊髓炎 (Neuromyelitis optica,NMO)1.1.IntroductionIntroduction：Acute or subacute demyelinating disease involved optic N and spinal cord at the same time.2.2.Etiology And Pathogenesis
14、 ：与遗传素质和种族差异有关。西方-脑干；东方-视神经和脊髓；25%MS突发球后视神经炎；多方研究证实白种人对MS易感；非白种人对NMO易感。3.3.PathologyPathology：Demyelination,plaque of sclerosis,infiltriation of inflammatory cells in perivascular distribution.视神经、视交叉及胸颈段易受累。颈髓脱髓鞘视神经炎Clinical Manifestations 1、年轻居多，21-41岁。2、特征：急性横贯性脊髓炎和双侧同时 或相继出现的ON。70%可在数日内有截瘫。3、急性起
15、病可在数小时或数日内单或双 眼失明，眼眶痛。4、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 平面、括约肌障碍等，1/3病人有 Lhermitte征、根痛。一、辅助检查 1、CSF细胞数增加，73%单相、82%复发。2、复发病人脊髓MRI88%出现纵向融合超 过数个节段，钆强化和肿胀常见。二、鉴别诊断 1、单纯球后神经炎 2、MS表现为NMO临床模式。3、亚急性视神经病 三、治疗 大剂量甲强冲击疗法急性播散性脑脊髓炎（ADEM）1、概述：是一种广泛累及脑和脊髓白质的急性炎症性疾病(感染出疹或疫苗接种)爆发型：急性出血性白质脑炎(AHL)2、病因及发病机制：病毒感染，脑
16、组织+FAC可诱发EAE，认为ADEM是急性MS，或其变异型。3、病理：脑和脊髓多数脱髓鞘病灶，小静脉周围炎性反应，形成血管袖套。Clinical Manifestations 1.Prodrome 2.Type of encephalitis 3.Type of meningitis 4.Type of myelitis 1.Lab Teste 1)WBC ,pressure of CSF or normal,Pr,IgG and OB positive;2)Abnormal of EEG;3)CT scan shows the lesions of multiple diffusion subcortex low density;MRI shows the multiple lesions of abnormal T1,T2 in brain and whiter matter.2.Diagnosis and differential diagnosis 乙脑及单疱病脑 3.Treatment 大量皮质类固醇冲击疗法Other demyelinating diseases 1.Diffuse sclerosis 2.Concentric sclerosis of Balo 3.Central pontine myelinolysis,CPM