《神经病学》（英文）课件7.Demyelinating Diseases

,单击此处编辑母版标题样式,*,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,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 cells,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,tracts.,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.,Epidemiology,Incidence of MS associated with latitude.,Contacting with some kind of environment,may play an important role in the,incidence of MS.,Heredity may be an important factor.,MS associated with the HLA-DR locus on the sixth chromosome, HLA-DR,2,express strongly and then,-,DR,3,B7 and A,3,.,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:,hyperemia,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,Relapsing-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 other brain nerves;, A,taxia:,Charcots,syndrom,Sensation disorder: Rombergs sign,Lhermittes,sign;,Attack,syndrom,Other clinical feature,6.,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,(probability 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 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.,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.,Differential 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-, ;,A,zathioprine,；,Immuneglublin（Ig,).,2.,Progressive 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.,Introduction,：,Acute or,subacute,demyelinating,disease involved optic N and spinal cord at the,same time.,2.,Etiology And Pathogenesis,：,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.,Pathology：,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,