[An elderly patient with progressive supranuclear palsy without neurological signs].

[An elderly patient with progressive supranuclear palsy without neurological signs].
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[一名无神经体征的进行性核上性麻痹老年患者]。

DOI:
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发表时间:
1997
期刊:
No to shinkei = Brain and nerve
影响因子:
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通讯作者:
K. Ikeda
K. Ikeda
中科院分区:
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文献类型:
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作者:
K. Niizato;N. Kuroki;T. Arai;K. Kase;S. Iritani;K. Ikeda

文献摘要

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我们报告了一位患有进行性核上性麻痹(PSP)的老年患者,但临床上没有表现出神经系统体征。一名70多岁的男子表现出烦躁和卫生条件差,随后表现出兴奋和暴力。头颅 CT 扫描显示双侧颞叶中度萎缩,侧脑室轻度增大。脑干轻度萎缩。尽管他去世时享年80岁,但在整个临床过程中他没有任何神经系统体征。神经病理学研究显示出典型的 PSP 表现,基底节、杏仁核、中脑、脑桥、齿状核和下橄榄核中神经元缺失伴神经胶质增生和神经原纤维缠结。 Gallyas-Braak 方法染色显示大脑皮层中存在嗜银和 tau 阳性神经胶质纤维缠结。在该年龄组的海马区和杏仁核中,神经原纤维缠结的生理水平显示出比平常更高的频率。表明精神病症状,主要是人格改变,可能与边缘系统的退化有关。由于之前没有任何关于不伴有神经系统症状的 PSP 的报道,因此该病例在 PSP 的临床病理变异方面具有重要意义。我们认为老年 PSP 患者的症状和神经病理学方面存在差异。
We report an elderly patient with progressive supranuclear palsy (PSP) who showed no neurological signs clinically. A 70-year-old man presented with irritation and poor hygiene, thereafter he showed excitement and violence. A cranial CT scan revealed bilateral moderate atrophy of the temporal lobes and slight enlargement of the lateral ventricle. The brain stem was slightly atrophic. Although he died at the age of 80 years, he had no neurological signs throughout the clinical course. Neuropathological study showed typical findings of PSP, neuronal loss with gliosis and neurofibrillary tangles in the basal ganglia, amygdala, midbrain, pons, dentate nucleus and inferior olivary nucleus. Staining by Gallyas-Braak methods revealed argyrophilic and tau-positive glial fibrillary tangles in the cerebral cortex. Neurofibrillary tangles showed greater frequency than usual for the physiological level in that age group in the hippocampus regions as well as in the amygdala. The possibility that the psychotic symptoms, mainly personality change, are connected with the degeneration of limbic system is indicated. Since there have not been any previous reports of PSP without neurological signs, this case represents an important in terms of clinico-pathological variation of PSP. We suggest that there is discrepancy between symptomatic and neuropathological aspects in elderly patients with PSP.