[An autopsy case of progressive supranuclear palsy showing "pure akinesia without rigidity and tremor and with no effect by L-dopa therapy (Imai)"].
[An autopsy case of progressive supranuclear palsy showing "pure akinesia without rigidity and tremor and with no effect by L-dopa therapy (Imai)"].
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[一例进行性核上性麻痹的尸检病例显示“纯粹的运动不能，没有强直和震颤，左旋多巴治疗（今井）没有效果”]。
DOI：
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发表时间：
1987
期刊：
影响因子：
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通讯作者：
F. Ikuta
中科院分区：
文献类型：
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作者：
Y. Homma;H. Takahashi;S. Takeda;F. Ikuta
Imai于1980年首次报道了11例“纯肌动症，无强直和震颤，左旋多巴治疗无效”。Hayashi和Hayashi（1983）补充了3例。然而，到目前为止还没有尸检病例，因此该综合征的分类学位置尚不确定。作者有机会观察Hayashi和Hayashi报告中的第三例病例8年，并进行了尸检。病例报告患者为女性农民。从54岁开始，由于姿势反射障碍、斥力现象和脚冻，她很容易摔倒。出现这些症状8年后，还增加了垂直性动眼肌麻痹、假性球麻痹和痴呆；她被诊断为进行性核上性麻痹。在此诊断之前，她的疾病被认为是“纯粹的运动障碍，没有僵硬和震颤，左旋多巴治疗没有效果”。即使在终末期也未观察到颈部肌张力障碍。她在65岁时去世。临床总病程约11年。病理观察固定前脑重1170 g。丘脑下核、苍白球和脑桥被明显萎缩。黑质严重脱色。显微镜下可见丘脑下核、苍白球、黑质、下丘脑、上丘、中央灰质、脑干网状结构、小脑齿状核等神经元丢失和胶质细胞增生。特征性表现为这些区域出现神经原纤维缠结。（摘要删节250字）
Eleven cases of "pure akinesia without rigidity and tremor and with no effect by L-dopa therapy" were first reported by Imai in 1980. Three cases were added by Hayashi and Hayashi (1983). However there have been so far no autopsy cases, remaining the nosological position of this syndrome uncertain. The authors have had an opportunity of observing the third case in the report by Hayashi and Hayashi for 8 years and autopsy was done as well. Case report The patient was a female farmer. On account of postural-reflex troubles, the pulsion phenomenon and feet freezing, which had progressed since the age of 54, she easily tumbled over. Eight years after the beginning of those symptoms, vertical oculomotor palsy, pseudobulbar palsy and dementia were added; she was diagnosed as a progressive supranuclear palsy. Before this diagnosis, her illness was being regarded as "pure akinesia without rigidity and tremor and with no effect by L-dopa therapy". Neck dystonia was not observed even in the terminal stage. She died at the age of 65. The total clinical course was about 11 years. Pathological observation The brain weighed 1,170 g before fixation. Marked atrophy of the subthalamic nucleus, globus pallidus and pontine tegmentum was observed. The substantia nigra was shown to be severely depigmented. Microscopically, loss of neurons and gliosis were seen in the subthalamic nucleus, globus pallidus, substantia nigra, hypothalamus, superior colliculus, central grey matter, brain stem reticular formation, cerebellar dentate nucleus, etc. The characteristic finding was the appearance of neurofibrillary tangles in these regions.(ABSTRACT TRUNCATED AT 250 WORDS)