医科大学精品课件:锥体外系疾病(2-运动障碍性疾病)-7.pptx
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1、运动障碍疾病运动障碍疾病 EXTRAPYRAMIDAL DISEASES ( MOVEMENT DISORDERS) 神经病学教研室神经病学教研室 DEPARTMENT OF NEUROLOGY ZHIJIAN ZHANG 运动障碍(锥体外系)疾病, 主要表现为随 意运动迟缓,不自主运动、肌张力障碍、姿 势步态障碍等运动症状。肌力、 Extrapyramidal diseases mainly impair the regulation of voluntary motor activity(sluggish), involuntary movement,myodystonia, withou
2、t 感觉及小脑功能不直接受影响。运 动障碍疾病源于基底节功能紊乱。 directly affecting strength, sensation, or cerebellar function. Movement disorders result from dysfunction of deep subcortical gray matter structures termed the basal ganglia. 解剖:解剖: AnatomyAnatomy: 基底节:尾状核、豆状核(壳核、 苍白球)、屏状核和杏仁复合体 (丘脑底核与黑质为相关结构)。 Basal ganglia: cauda
3、te nucleus, lentiform nucleus ( putamen, globus pallidus), claustrum and amygdaloid complex. 新纹状体:壳核、尾状核。 Neostriatum: putamen, caudate. 旧纹状体:苍白球 Paleostiratum: globus pallidus 古纹状体:杏仁复合体 Archistriatum: amygdaloid complex 相关结构:红核、黑质、丘脑底核、 中脑间质核、大脑皮质的4、6区。 Dependency structure: red nucleus ( corpus r
4、ubrum), substantia nigra, mid brain interstitial nucleus, 4 and 6 regions of cerebral cortex 神经环路神经环路: Neuronal circuitryNeuronal circuitry: 1.皮质-皮质环路:大脑-皮质-尾 状核、壳核-内侧苍白球-丘脑-大 脑皮质(直接通路,间接通路) 1. Corticortical loop: cerebral cortex-caudate, putamen-the internal segment of the globus pallidus-thalamus-
5、 cerebral cortex (direct path, indirect path ) 2 .黑质-纹状体环路:黑质与尾状 核、壳核之间的往返联系纤维 2. Nigrostriatal loop: connecting the substantia nigra, caudate and putamen 3. 纹状体-苍白球环路:尾状核、 壳核-外侧苍白球-丘脑底核-内侧 苍白球 3. Striaropallidal loop: projects from the caudate and putamen to the external segment of the globus palli
6、dus, then to the subthalamic nucleus, and finally to the internal segment of globus pallidus. 书本简约: 大脑皮质-基底核-丘脑-大脑皮质 神经递质神经递质:乙酰胆碱(Ach)、多 巴胺 (DA)、去甲肾上腺素 (NE)、 5-羟色胺 (5-HT)、-氨基丁酸 (GABA) 、谷氨酸等。 Neurotransmitter(NT): acetylcholine, dopamine, noradrenaline, 5- hydroxytryptamine(serotonin), gamma-aminobu
7、tyric acid, aminoglutaminic acid, etc. 椎体外系症状 肌张力异常:高或低 运动迟缓 异常不自主运动:震颤、舞蹈、投掷、 徐动。 临床分类:临床分类: Clinical classification: 1运动过多:震颤、肌张力障碍、舞蹈 症、手足徐动症、抽搐 1. hypercinesia: tremor, dysmyotonia, chorea, posthemiplegic chorea, tic 2运动减少:帕金森病和帕金森综合征。 2. hypokinesia: Parkinsons disease and Parkinsons syndrome 3
8、. 其他疾病:共济失调、肝豆状核变性。 3. Other diseases: ataxia, Kinnier- Wilson syndrome 治疗原则:治疗原则: Therapeutic principleTherapeutic principle: 病因治疗、药物治疗、神经外科治疗、个 体化原则。 Etilogical treatment,drug treatment, neurosurgical therapy, individualized treatment 帕金森病帕金森病 ( agitans paralysis; paralysis agitans; Parkinsons dis
9、ease; PD) 帕金森病又名震颤麻痹,是一 种常见的中老人神经系统变性疾病 Parkinsons disease, also named Paralysis agitans, is a kind of frequent nervous system degenerative disease . 65岁以上人群(北京、西安、上海)患 病率1.7%, 男性居多。 The morbidity rate is 1.7% in the population of above 65 years old. 以黑质多巴胺能神经元变性缺失和以黑质多巴胺能神经元变性缺失和 路易小体形成为特征。临床上表现路易小
10、体形成为特征。临床上表现 静止性震颤、运动迟缓、肌强直和静止性震颤、运动迟缓、肌强直和 姿势步态异常。姿势步态异常。 Characteristic loss and Characteristic loss and degeneration of dopamine degeneration of dopamine neurons in the neurons in the substantiasubstantia nigranigra. . The The lewylewy bodies forming. bodies forming. Present symptoms are static P
11、resent symptoms are static tremor, tremor, bradykinesiabradykinesia, , myotoniamyotonia , , posture and walking disordersposture and walking disorders . . 病因病因: etiological factor : 病因不明。发病可能与以下因素有关: etiology unknown. The factors bellow possibly relates the onset: 1. 年龄老化:主要发病为中老年人, 但只是促发因素(多巴胺含量减少
12、80% )。 Ageing: it mainly attacks aged people, but the ageing is only the precipitating factor. 2. 环境因素:MPTP使ATP生成减少, 自由基生成,氧化应激反应,导致多巴 胺能神经元变性死亡。 Environmental factor: The MPTP reduces the generation of ATP, preciptitates the generation of free radical and increases the oxidative stress, which resul
13、ts in the regeneration and death of dopamine neurons. 3. 遗传因素:约10%-15%病人有阳性家 族史,多呈常染色体显性遗传。 Hereditary factor: about 10%-15% percent patients have the family history, and most of them belong to autosomal dominant inheritance. 4.多因素: 病机病机: Pathogenesis: 1.氧化应激氧化应激 氧自由基、羟自由基、兴奋性氨基酸。目前氧自由基、羟自由基、兴奋性氨基酸。
14、目前 认为是多因素作用的结果。认为是多因素作用的结果。 1. Oxidation stress:1. Oxidation stress: free radical, free radical, hydroxyhydroxy radical, radical, excitatory amino excitatory amino acids.Recentlyacids.Recently the the occurenceoccurence of this disease is considered of this disease is considered as the effective res
15、ult of multiple as the effective result of multiple factorsfactors 2.腺粒体功能缺陷腺粒体功能缺陷 减少减少ATPATP合成合成, ,增加氧自由基的生成增加氧自由基的生成. . 2. 2. Mitochondrial Mitochondrial disfunctiondisfunction The ATP synthesis reduce, The ATP synthesis reduce, oxidatedoxidated free radical increase.free radical increase. 3.泛素泛素
16、- -蛋白酶体功能异常蛋白酶体功能异常 - -突触核蛋白过度表达突触核蛋白过度表达, , 出现出现lewylewy小体小体. . 泛素、蛋白酶体亚单位、泛素羧基水解酶泛素、蛋白酶体亚单位、泛素羧基水解酶L1L1、 ParkinParkin等等蛋白过度表达和聚集。蛋白过度表达和聚集。 3. 3. DisfunctionDisfunction of of ubiquitinubiquitin- -proteasomeproteasome systemsystem The over expression ofThe over expression of- - synucleinsynuclein i
17、nduce the formation of induce the formation of lewylewy body.body. 病理与生化病理病理与生化病理: Pathology and biochemical pathology: 1. 病理: 含色素的神经元变性、缺失, 尤以黑质致密部DA能神经元为著。出现路 易小体,苍白小体。 1. pathology: The loss and degeneration of pigmentosus neurons, especial the dopamine neurons in the substantia nigra. The lewy b
18、odies and pale bodies can be seen also. 该二小体尚可在老年痴呆、共济失调、 毛细血管扩张症和哈-斯二氏病见到。 These two bodies also can be seen in senile dementia, ataxia, telangiectasia and Hallervoeden- spatz disease. Braak 延髓背核、前嗅核、脑桥、中脑、新皮质 2.生化病理:多巴胺与乙酰胆碱作为生化病理:多巴胺与乙酰胆碱作为 纹状体中两种重要的神经递质系统,功纹状体中两种重要的神经递质系统,功 能互相拮抗,它们的平衡在该病的发病能互相拮
19、抗,它们的平衡在该病的发病 起重要作用。起重要作用。 2. Biochemical pathology: As two 2. Biochemical pathology: As two important neurotransmitters in important neurotransmitters in striatum, DA and Ach functionally striatum, DA and Ach functionally antagonize each other, playing antagonize each other, playing very important
20、roles in very important roles in Parkinsons Disease.Parkinsons Disease. PD 患者黑质-纹状体通路变性,纹状 体DA含量显著降低(减少70%以上), Because the degeneration of substantia nigra- striatum pathway, the content of DA in striatum remarkably decrease (over 80 percent), 造成Ach系统功能相对亢进,导致基底 节输出过多,丘脑-皮质反馈活动受到 过度抑制, the acetylcho
21、line system exerts a excitatory effect, which results in the over output of basal ganglia, the inhibition of the feed backing activity of thalamus opticus-cortex, 其对皮质的易化作用受到削弱,因此产 生肌张力增高、动作减少等运动症状。 and the impairment of facilitation effect to cortex, so the movement disorder symptoms appear, such a
22、s hypermyotonia, and reduction of movement 临床表现临床表现: Clinical findings:Clinical findings: 1. 40岁少,大部分60岁以后发病。65 岁1%-2%。 1. symptoms occur mostly in the age of 50, or older. 2. 起病隐袭,缓慢发展,逐渐加剧。 2. insidious onset, slow development, gradual aggravation 3. 主要症状为静止性震颤、肌张力 增高、运动迟缓等。首发症状:60-70% 震颤,12%步行障碍,
23、10%肌强直,10% 运动迟缓。 3. present symptoms are static tremor, hypermyotonia, bradykinesia. First symptoms: tremor 60-70%, walking disorder 12%, myotonia 10%, bradykinesia10%. 65-70%从一侧上肢开始,渐波及同侧下肢,对侧 上肢与下肢。 25-30%从一侧下肢开始。 (1)震颤: 静止性,紧张加剧,搓丸样动作,每秒 4-6次 These symptoms 65-70% start from one side upper extrem
24、ity, gradually to same side lower extremity, then the opposite side extremities. (1)Tremor: static, it increases with emotional stress, the fingers appear the pill-rolling- like action.4-6 times per sec. (2)肌强直: 铅管样,齿轮样强直。路标现象。 (2)Rigidity: lead pipe-like, cogwheel rigidity. A road sign phenomenon.
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