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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 relativ
3、e 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
4、症(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 t
5、he 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
6、is 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 ar
7、ea to 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,
8、 HLA-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 infl
9、ammatory 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 subacut
10、e onset (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 vi
11、sual 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, Sensation disorder: Rombergs sign, (1/2) Lhermittes sign; Ataxia (1/2), Charcots syndrom (later stage); Impairment of PNS; Attack syndrom; Other clinic
12、al feature.,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 pot
13、entials (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 p
14、ossible MS MRI Criteria for diagnosing MS At 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 Diag
15、nosis 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 and CSF OB/IgG; One attack, two le
16、sions 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; Tw
17、o 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,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
18、. 预防并发症。 4. 对症及支持疗法。,1. Relapsing-Remitting MS： 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. Progressive MS：, Methotrexate, MTX; Cyclosph
19、oamide; Cyclosporine A; Plasma transplantation.,3. Symptomatic treatment:, 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1年，左耳鸣、视物双影半年。 走路
20、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,该患如何定位及定性？,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.Introduction,2.Etiology And Pathogenesis,视神经脊髓炎 (Neuromyelitis optica, NMO),3.Patholgy,主要侵犯视神经、视交叉、和脊髓胸颈段,
21、Acute stage:infiltration of inflammatory cells.,Astrocyte proliferition,Clinical 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
22、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,颈髓脱髓鞘,视神经炎,急性播散性脑脊髓炎 (acute disseminated encephalomylitis, ADEM),1.Introduction: ADEM is a kind of acute inflammatory disseminated disease involved the whiter matter of brain and spinal cord. (感染出疹或疫苗接种) 爆发型：急
23、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。,Clinical Manifestations,1.Prodrome 2.Type of encephalitis 3.Type of meningitis 4.Type of myelitis,1.Lab Teste 1) WBC , pressure
24、 of CSF or normal, Pr , IgG and OB (+); 2) Abnormal of EEG; 3) CT scan shows the lesions of multiple diffusion subcortex low desity; 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. Leukodystrophy 3. Central pontine myelinolysis, CPM 4. Concentric sclerosis of Balo,1. Diffuse sclerosis(Schilders),2. Leukodystrophy,3. Central pontine myelinolysis, CPM,4. Concentric sclerosis of Balo,谢谢,