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中枢神经系统感染 INFECTIONS OF THE CENTRAL NERVOUS SYSTEM Neurology Department The Second Hospital of Kunming Medical University
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第一节 概述 Term : Infections of CNS All kinds of pathogen (bacteia,viruses,spirochete,parasite s,fungi,rickettsia and prion prottein) invade cerebral parenchyma, meninges and blood vessel lead to acute and subacute infections.
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第一节 概述 分类 : 感染的部位 ;发病情况及病程 ; 特异性致病因子 CNS 感染途径 (pathway of infection) ① hematogenous spread ; ② direct infection ; ③ Peripheral nerves pathway CNS 病毒感染性疾病新的认识
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第二节 病毒感染性疾病 Viral infections of CNS 单纯疱疹病毒性脑炎 Herpes Simplex Encephalitis Creutzfeldt-Jakob Disease
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Herpes Simplex Encephalitis 病因及发病机制 ( Causes and Mechanisms) 病理( pathology) 临床表现 (Clinical features) 辅助检查 (laboratory finding) 诊断及鉴别诊断 (Diagnosis and Differential Diagnosis) 治疗( treatment)
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HSE -- Causes and Mechanisms HSV- 嗜神经 (neurotropic )DNA 病毒DNA 病毒 90% 的人类 HSE 是由 I 型引起 70%HSE 起因于内源性病毒的活化 ( 复发性疱疹感染 ) 25% 的病例是原发感染 ( 口腔和呼吸道 ) 6%~15% 系由 II 型所致 ( 产妇生殖道 HSV-II 原发感染 ) 绝大多数新生儿的 HSE 系 HSV-II 引 起
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HSE -- Pathology 颞叶、额叶等部位出血性坏死 | 、渗 出出血性坏死 渗 出 急性期后可见小胶质细胞增生 Intense hemorrhagic necrosis of the inferior and medial parts of the temporal lobes and the medial- orbital parts of the frontal lobes.
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HSE-Pathology Cowdry A 型包涵体 (A type inclusion body) 存在于病灶边缘的部 分神经细胞核内 及星型细胞和少突 胶质细胞核内。
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HSE -- Clinical feature 1 .任何年龄、季节均可发病原发感染的潜伏 期为 2~21 天,平均 6 天;前驱期症状 2 .病程 多急性起病 ,口唇疱疹史 ( 1/4 ), 高热,首发症状 :头痛、轻微的意识和人格 改变 或全身性或部分性运动性发作 ;病情缓 慢进展精神症状表现突出智能障碍也较明显 3 .神经症状 局灶性脑损害 ;脑膜刺激征 ; 意识障碍 ;全身性或部分性癫痫 。重症脑疝 形成而死亡 (死亡率高达 40%~70% )。
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HSE-Clinical feature 1.It occurs sporadically throughout the year and in patients of all ages. During prodromal stage:fever,headache,muscular ache etc. 2.The onset is acute,patients may had herpes labialis(1/4) and fever.The early manifestations:headach,personality change,slight concious disorder and seizures.Psychotic behavior, memory loss become evident later.
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HSE-Clinical feature 3.Neurologic symptom and sign hemiparesis,aphasia,meningeal rritatioin,disorder of conciousness,focal or generalized seizures.It may result in coma or death in some cases.
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HSE -- Laboratory Diagnosis 1 .脑电图 弥漫性高波幅慢波 2 .头颅 CT 可正常,也可见一侧或双侧颞叶、 海马及边缘系统局灶性低密度区 3 .脑脊液检查压力及细胞数正常或轻度增 高,重症者可明显增高。 4 .脑脊液病原学检查对诊断颇有意义 ①检 测 HSV 抗原 ②检测 HSV 特异性 lgM 、 lgG 抗 体 ③ CSF 中 HSV-DNA ( PCR 快速诊断) 脑组织病理学及病原学检查
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HSE--Laboratory Diagnosis EEG:lateralized high-voltage slow- waves. CT scans show hypodensity of the affected areas. MRI shows signal changes in almost all. 图 1 图 2 图 3 图 1 图 2 图 3 CSF: increased pressure, lymphocytic pleocytosis, mild protein elevation and normal glucose.
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HSE--Laboratory Diagnosis Tests for the detection of HSV antigen in the CSF by the application of PCR are useful. The absolute way for dianosis:fluorecent antibody study and viral cultrue of cerebral tissue obtained by brain biopsy.
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HSE --diagnosis 1 .临床诊断依据: ①口唇或生殖道疱疹史; ②高热、脑炎、精神症状三主征及局灶性神 经系统损害体征;③脑脊液红、白细胞数增 多,糖和氯化物正常;④脑电图以颞、额区 损害为主的脑弥漫性异常;⑤头颅 CT 或 MRI 发现颞叶局灶性出血性脑软化灶;⑥ 特异性抗病毒药物治疗有效。 2. 实验室检查 : CSF 病原体检查及病理检查
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HSE —Differential diagnosis 急性播散性脑脊髓炎:多在感染或疫苗接种 后急性发病 结核性脑膜脑炎:结核病病史或接触史,慢 性过程,脑膜刺激征是 TBM 早期表现 ,可 有脑神经损害, CSF 检查提示诊断 。 肠道病毒性脑炎:也是病毒性脑炎的常见 病因之一,多见于夏秋季,可为流行性或散 发性 带状疱疹病毒性脑炎
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HSE -treatment Antiviral agents There was no specific treatment for HVE untill the late 1970s acyclovir was introduced. Acyclovir and ganciclovir are most effective drugs.They significantly reduce both the mortality and morbidity.
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HSE -- treatment 1 .抗病毒化学药物治疗 (1) 无环鸟苷 ( 阿昔洛韦, acyclovir) (2) 更昔洛韦 (ganciclovir) 2 .免疫治疗 ①干扰素及其诱生剂 ②转移因子 ③肾上腺 皮质激素 3 .全身支持治疗 4 .对症治疗
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Creutzfeldt-Jakob Disease Creutzfeldt-Jakob 病 (CJD) 是最常见的人类朊蛋 白病 (具传染性的朊蛋白所致的散发性中枢神 经系统变性疾病) CJD is also called Subacute Spongiform Encephalopathy. (SSE) It belongs to the category called the transmissible spongiform encephalopathies (prion diseases)
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PrP and prion Protein dease 朊蛋白 (prion protein, PrP) 一种既具有传染 性又缺乏核酸的非病毒性致病因子 Prion is neither a virus nor a viroid(nucleic acid alone,without a capsid structure)but the conversion of a normal cellular protein.
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PrP and prion Protein dease 人类朊蛋白病还有 Kuru 病、 Gerstmann-Straussler 综合征 (GSS) 、 致死性家族性失眠症 (FFI) 、缺乏特 征性病理改变的朊蛋白痴呆和伴痉 挛性截瘫的朊蛋白痴呆 。
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What is CJD? Refer to a distinctive cerebral disease in which a rapidly progressive and profound dementia associated with cerebellar ataxia,diffuse myoclonic jerks and a variety of other visual and neurologic abnormalities. The outstanding features of the neuropathologic changes are widespread neuronal loss and gliosis accompanied by a striking vacuolation or spongy state of the affected regions
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CJD- Pathogenesis and Type 1 型和 2 型存在于散发性 CJD( sporadicula CJD ) 3 型为医源性 CJD- 通过角膜、硬脑膜移植, 脑源性生物制品和埋藏未充分消毒的脑电极 而传播 Type-3:iatrogenic CJD by transplantation of corneas and implantation of infected depth electrdes etc.
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CJD- Pathogenesis 4 型是新变异型 - 与疯牛病 (MCD) 具有相 似的种系特异性 PrP 基因突变形成遗传性家族型 CJD
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CJD- Pathology 大体 -- 脑呈海绵状改变,皮质、基 底节和脊髓萎缩变性。 Spongy appearance shows in cerebral and cerebellar cortex.
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CJD -Pathology 显微镜下 -- 神经元丢失、星形细胞增 生、细胞胞浆中空泡形成,可发现 感染组织内异常 PrP 淀粉样斑块。 Widespread neuronal loss and gliosis accompanied by a striking vacuolation and PrPsc in the affected regions.
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CJD- 临床表现 1 .发病年龄 25~78 岁,平均 58 岁,男女均可 罹患,新变异型平均 26 岁 2 .隐袭起病,缓慢进行性发展 ①初期:表现颇似神经症,可有头痛、眩晕、 共济失调及视觉障 碍等 ②中期:进行性痴呆为主要表现,伴人格改 变 ,有失语、偏瘫、锥体束征 或肌肉萎缩 及 2/3 病人出现肌阵挛,最具特征性 ③晚期:出现尿失禁、无动性缄默、昏迷 等 3 .变异型 CJD 临床表现共济失调和行为改 变
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CJD-clinical feature 1. It occurs mostly in the late middle age, although can occur in young adult. 2. Progressive development. The early stage: Atypical The mid stage: gradual dementia with personality change. Myoclonus occurs in 2/3 of patients. The late stage : Coma, akinetic mutism. 3. Variant CJD
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CJD- Laboratory Finding 1 .免疫荧光检测 CSF 中 14-3-3 蛋白可呈 阳性 -- 可疑 CJD 病人重要指标。血清 S100 蛋白(随病情进展呈持续性增高)。 2 .脑电图:疾病中晚期可出现间隔 0.5~2 秒周期性棘 - 慢复合波。 3 .晚期 CT 和 MRI :可见脑萎缩; MRI 显示双侧尾状核、壳核 T2 呈对称性均质 高信号 , T1 可完全正常 。
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CJD-Laboratory Finding Test of CSF by immunoassay, the finding of 14-3-3 protein is very useful in separating SSE. Also is the serum P-100. EEG :High voltage slow and sharp-wave complexes(0.5-2Hz). MRI subtle hyperintensity of the lenticular nuclei on T2 weighted images when the disease is fully established.
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CJD- Diagnosis( 诊断标准 ) 很可能 (probable) CJD ①在 2 年内发生的进行性痴呆; ②肌阵挛、视力障碍、小脑症状、无动性缄 默等四项中具有其中两项; ③脑电图周期性同步放电的特征性改变。 如病人脑活检发现海绵状态和 PrP SC 者, 则为确诊的 CJD 。 可用脑蛋白检测代替脑电图特异性改变。
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CJD-Diagnosis Probable SSE 1)Progressive dementia in 2 years 2)Two of myoclonus, visual disterbance, ataxia and akinetic mutism. 3)EEG: synchronous discharge. Definite diagnosis Spongy or PRPsc is found by brain tissue biopsy.
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CJD- 鉴别诊断 Alzheimer 病 进行性核上性麻痹 橄榄脑桥小脑萎缩 脑囊虫病 肌阵挛性癫等鉴别
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CJD- 治疗及预后 尚无有效治疗 对症治疗 巴氯芬 (baclofen) 治疗痉挛性张力增高, 氯硝西泮治疗肌阵挛, 痴呆可用三乐喜、哌醋甲酯 ( 利他林 ) 和尼 麦角林 ( 脑通 ) 等。 应用反义寡核苷酸或基因治疗可能达到 治疗目的 90% 病例于病后 1 年内死亡
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脑囊虫病 Cerebral Cysticercosis
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由猪带绦虫蚴虫 ( 囊尾蚴 ) 寄生脑组 织形成包囊所致。 Cysticercosis is the larval stage (cysticercus) of infection with the pork tapeworm.
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Cerebral Cysticercosis 是一种最常见的 CNS 寄生虫感染, 也是我国北方症状性癫常见的病 因之一。 Cysticercosis is a leading cause of epilepsy and other neurologic disturbances.
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脑囊虫病 - 病因及发病机制 最常见的传播途径是摄入带有虫卵污染 的食物 少见原因为肛门 - 口腔转移而形成的自身 感染或者是绦虫的节片逆行入胃 虫卵进入十二指肠内孵化逸出六钩蚴, 蚴虫经血液循环分布全身并发育成囊尾 蚴,有不少囊尾蚴寄生在脑内。
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脑囊虫病 - Pathology 典型的包囊大小为 5~10mm ,可有 薄壁包膜,或呈多个囊腔 The cysts may be 5-10mm.The lesions are most often multiple but may be solitary.
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Cysticercosis -Pathology 脑实质中包囊内存活的蚴虫很少引起 炎症,通常在感染后数年蚴虫死亡后 才出现明显的炎症反应 Only when the cyst degenerated many months or years after the initial infestation, an inflammatory and granulomatous reaction is elicited and focal symptoms arise.
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脑囊虫病 - Clinical Feature 1 .脑实质型 临床症状与包囊的位置有 关 。 2 .蛛网膜型 头痛、脑积水和虚性脑膜 炎等。 3 .脑室型 阻塞性脑积水;布龙 (Brun) 征发作(移动的包囊,可突然阻塞第四 脑室正中孔,导致脑压突然增高,引起 眩晕、呕吐、意识障碍和跌倒 ) 。 4 .脊髓型 非常罕见
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Clinical Feature The cerebral manifestations of cysticercosis are diverse, related to the encystment and subsequent calcification of the larvae in cerebral parenchyma, subarachnoid space and ventricle. The flow of CSF may be obstructed by large subarachnoid or intraventricular cyst and leads to obstructive hydrocephalus.
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脑囊虫病 - Laboratory Diagnosis 1 .血常规检查嗜酸性粒细胞增多。 2 .用 ELISA 和 Western 印迹法检测血清 囊虫抗体常为阳性。 3 .头颅 CT 和 MRI 可发现脑积水及被阻 塞的部位, CT 可见单个或多个钙化点, CT 平扫见包囊为小的透亮区,增强扫描 为弥散性或环形增强影。
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Laboratory Diagnosis CT or MRI may shows obstructive hydrocephalus. Enhancing cysts are easier found on MRI. Calcified cysts can be shown clearly on CT. Serum test for immunoreaction of cysticercus is very useful.
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脑囊虫病 - Diagnosis 曾居住在流行病区,并有癫痫、脑 膜炎或颅内压升高表现的患者,应 疑及本病。 Have ever been to the epidemic(en- demic) areas, with epilepsy, meningitis and the sign of high cranial pressure may be possible cysticecosis.
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脑囊虫病 - Diagnosis 2. 血清囊虫抗体试验、皮下结节的囊 虫活检和头部 CT 、 MRI 检查可帮助 诊断。 三脑室囊虫 外侧裂囊虫 CT MRI 三脑室囊虫 外侧裂囊虫 Antibody test in serum and CSF and biopsy of subcuticular tubercle of cysticercus; together with CT and MRI are all very helpful. 囊虫病巨大囊肿
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脑囊虫 -Differential Diagnosis 脑肿瘤 结核性脑膜炎 其他病因所致的癫鉴别。
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脑囊虫病 - 治疗 常用药物 (antihelminthic) ①吡喹酮 (praziquantel) :是广谱抗寄生 虫药,成人总剂量为 300mg/kg 。 ②阿苯哒唑 (albendazole, 丙硫咪唑 ) :广 谱抗寄生虫药,成人总剂量亦为 300mg/kg 。 用药过程中必须严密监测,必要时应给予 皮质类固醇或脱水剂治疗。 单个病灶 ( 尤其是脑室内者 ) 可手术摘除 。
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