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1、中枢神经系统脱髓鞘疾病DemyelinatingDiseasesoftheCentralNervousSystem 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。Keypoints-DemyelinatingDiseasesofCNS Chapter 1 Intraduction 1.Concept:A group of disease characterized by demyelinating of the brain and spinal
2、cord.PATHOLOGY:Demyelination 髓 鞘 构 成CNSPNS2.PathologicFindings 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 integrity of the axis cylind
3、ers 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(acutedisseminatedencephalomyelitis,ADEM)多发性硬化症(multiplesclerosis,MS)亚型视神经脊髓炎(Devicdiseases)急性出血性白质脑病(acutehemorrhageleukoencephalitis,AHLE)多发性硬化症(MS)多发性硬化MultipleSclerosis,MS 1.Concept:Ms is a ki
4、nd 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 episodes of demyelination are separated in time a
5、nd 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.EtiologyAndPathogenesis 1)病毒感染及自身免疫反应：Since the exact cause is uncertain.Immunological mechanisms undoubtedly play a role,although the
6、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 to a low-prevalence area prior to puberty,the risk of developing MS is higher than in t
7、he 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 and then-DR3,B7 and A3.4.PathologicFindings Characteristic:Multiple d
8、emyelinated 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 stage:Astrocyte proliferition,forming of astrocytic scab.急 性
9、 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.ClinicalManifestations 1)Prodrome:The symptoms evolved more slowly,over several weeks or months.2)Acute or subacute onset(Relapsing-remitting).3)Early symptoms and signs:Weakness or numbness;(1/2 patients have paresthesia on one or more limb
10、s)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)Nystagmus and palsy of eye muscles;(internuclear ophthalmoplegia,PPRF one and a half syndrome)“一个半综合征”垂直眼震
11、Sensation disorder:Romberg s sign,(1/2)Lhermitte s sign;Ataxia(1/2),Charcot s syndrom(later stage);Impairment of PNS;Attack syndrom;Other clinical feature.6.LaboratoryandassistantTests 1)CSFTest Number of MNC 0.7(70%);oligoclonal bands(OB)(95%);MBP,PLP,MAG,MOG Abs and Ab-secreting cells;CSF-Alb/seru
12、m-Alb1.7(probability of MS)2)Evokedpotentials: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,cerebellum and spinal cord;atrophy symptom.-AbnormalMRIscansarefound
13、in96%withadefinitediagnosisofMS70%withadiagnosisofprobableMS30-50%withadiagnosisofpossibleMS-MRICriteriafordiagnosingMSAtleast3Lesionsandtwoofthefollowing:1LesionsabuttingtheLateralVentricles2Lesionswithdiametersgreaterthan5mm3LesionspresentinthePosteriorFossaSource(Offenbacher H,Fazekas F,Schmidt R
14、 et al.Assessment Of MRI Criteria For A Diagnosis Of MS*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 an
15、d CSF OB/IgG;One attack,two lesions and CSF 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
16、/IgG;Two attacks involving different part of CNS,intermission at lest one month;each attack must continue for 24hs.多发硬化的诊断标准诊断 诊断 发作次数 发作次数 临床病灶数 临床病灶数 亚临床证据 亚临床证据 CSF OB/CSF OB/IgG IgG 临床确诊 临床确诊（CDMS CDMS）2 22 22 21 1 及 及 1 1实验室支持确诊 实验室支持确诊(LSDMS LSDMS）2 21 11 11 12 21 1或 或 1 1及 及 1 1+临床可能 临床可能(CPM
17、S)CPMS)2 21 11 11 12 21 11 1+实验室支持可能 实验室支持可能(LSPMS LSPMS）2 2 两次发作均累及CNS不同部位，间隔至少一个月，每次持续24小时。+DifferentialDiagnosis 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：目前尚无一种特效疗法，治疗的主要目的是：1.1.急性活动期抑制其炎症性脱髓鞘过程，急性活动期抑制其炎症性脱髓鞘过程，遏止病情的进展。遏止病情的进展。2.2.尽量预防能促发的外因，减少复发次尽量预防能促
18、发的外因，减少复发次 数，延长缓解间歇期。数，延长缓解间歇期。3.3.预防并发症。预防并发症。4.4.对症及支持疗法。对症及支持疗法。1.Relapsing-RemittingMS：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 2.Progressiv
19、eMS：Methotrexate,MTX;Cyclosphoamide;Cyclosporine A;Plasma transplantation.3.Symptomatictreatment:Spasticity:baclofen,dantrolene,diazepam and tizanidine can be helpful.Bladder dysfunction:anticholinergic drugs urinary catheter may be required.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Examples患者，女，32岁。主诉：行走不稳
20、1年，左耳鸣、视物双影半年。走路不稳，踩棉花感 左耳鸣 复视 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 Romberg征(+)，左Hoffmann征(+)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MRI未见异常 视神经和脊髓受累较多见，病灶中的软化、坏死较多见.视神经脊髓炎又称Devic病,为多发性硬化的一个亚型.中国,日本等东方人1.Introduction2.2.EtiologyAndPathogenesis视神经脊髓炎(Neuromyelitisoptica,NMO)3.Patholgy主要侵犯视神经、视交叉、和脊髓胸颈段Acutestage
21、:infiltration of inflammatory cells.Astrocyte proliferitionClinical Manifestations-NMO 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
22、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颈髓脱髓鞘视神经炎急性播散性脑脊髓炎(acutedisseminatedencephalomylitis,ADEMADEM)1.Introduction:ADEM is a kind of acute ADEM is a kind of acute inflammatory disseminated disease involved the inflammatory disseminated disease involved the whiter matter of brain
23、 and spinal cord.whiter matter of brain and spinal cord.(感染出疹或疫苗接种)爆发型：急性出血性白质脑炎(acute necrotizing hemorrhagic encephalomyelitis,AHL)2.Etiology And Pathogenesis 病毒感染，脑组织+FAC可诱发EAE，认为ADEM是急性MS或其变异型。3.Pathology 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。ClinicalManifestations 1.Prodrome 2.Typeofencephalitis 3.Typ
24、eofmeningitis 4.Typeofmyelitis 1.Lab Teste 1)WBC,pressureofCSFornormal,Pr,IgGandOB(+);2)AbnormalofEEG;3)CTscanshowsthelesionsofmultiple diffusionsubcortexlowdesity;MRIshowsthemultiplelesionsofabnormal T1,T2inbrainandwhitermatter.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Otherdemyelinatingdiseases 1.Diffusesclerosis 2.Leukodystrophy 3.Centralpontinemyelinolysis,CPM 4.ConcentricsclerosisofBalo1.Diffusesclerosis(Schilders)2.Leukodystrophy3.Centralpontinemyelinolysis,CPM 4.ConcentricsclerosisofBalo