锥体外系疾病2-运动障碍性.ppt

锥体外系疾病（运动障碍性疾病）Extrapyramidal diseases ( movement disorders)神经病学教研室 Department of Neurology Zhijian Zhang 锥体外系疾病, 主要表现为随意运动调节功能障碍，肌力、Extrapyramidal diseases mainly impair the regulation of voluntary motor activity without 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：Anatomy:基底节：尾状核、豆状核（壳核、苍白球）、屏状核和杏仁复合体。Basal ganglia: caudate 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 rubrum), substantia nigra, mid brain interstitial nucleus, 4 and 6 regions of cerebral cortex 神经环路：Neuronal loops: 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1.? Corticortical loop: cerebral cortex-caudate, putamen-the internal segment of the globus pallidus-thalamus-cerebral cortex 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 pallidus, then to the subthalamic nucleus, and finally to the internal segment of globus pallidus. 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)、γ-氨基丁酸 (GABA) 、谷氨酸等。Neurotransmitter(NT): acetylcholine, dopamine, noradrenaline, 5- hydroxytryptamine(serotonin), gamma-aminobutyric acid, aminoglutaminic acid, etc. 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, tic2．运动减少：帕金森病和帕金森综合征。2. hypokinesia: Parkinson’s disease and Parkinson’s syndrome 3. 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：Therapeutic principle: 病因治疗、药物治疗、神经外科治疗、个体化原则。Et