中枢神经系统脱髓鞘疾病课件(1)

中枢神经系统脱髓鞘疾病Demyelinating Diseases of the CentralNervous System Chapter 1 Intraduction1. ConceptA 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 Sclerosis1. ConceptMultiple 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 Pathogenesis1)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. Epidemiologyn Incidence of MS associated with latitude.n Contacting with some kind of environment may play an important role in the incidence of MS.n Heredity may be an important factor. n MS associated with the HLA-DR locus on the sixth chromosome, HLA-DR2 express strongly and then -DR3 , B7 and A3 .4. Pathologic FindingsCharacteristic: 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 Manifestations1) 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; Ataxia: Charcots syndrom Sensation disorder: Rombergs sign, Lhermittes sign; Attack syndrom Other clinical feature6.Laboratory and other assistant Tests1. CSF Test1) Number of MNC 0.7;oligoclonal bands(OB) positive;3) MBP, PLP, MAG, MOG Abs and Ab-secreting cells4) 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 criteria1. 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 Diagnosis1. 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，治疗的主要目的目前尚无一种特效疗法，治疗的主要目的是：是：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. 尽量预防能促发的外因，减少复发次尽量预防能促发的外因，减少复发次数，延长缓解间歇期。数，延长缓解间歇期。3. 预防并发症。预防并发症。4. 对症及支持疗法。对症及支持疗法。1. Relapsing-Remitting MS： ACTH and Steoids： methylprenisolone,prednison, dexamethasone; IFN- ; Azathioprine； 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。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 Pathogenesi