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[进行性核上性麻痹中广泛存在的嗜银性结构——一例尸检病例报告]

[Widespread argentophilic structures in progressive supranuclear palsy--an autopsy case report].

作者信息

Hanihara T, Kubota H, Amano N, Yamaoka K, Yagishita S

机构信息

Division of Psychiatry, Kanagawa Rehabilitation Center, Japan.

出版信息

No To Shinkei. 1994 May;46(5):487-92.

PMID:8060688
Abstract

We report an autopsy case of progressive supranuclear palsy (PSP) with a five-year clinical course. A 67-year-old man was suffering from a gait disturbance and mental deterioration. Neurological examination at the age of 71 revealed pseudobulbar palsy, horizontal ophthalmoplegia, and truncal dystonia, and a diagnosis of PSP was made. Mental deterioration including forgetfulness and character change was also noted, and the patient sometimes exhibited intermittent stuporous states. Cranial computed tomography and magnetic resonance images revealed moderate brain atrophy, predominantly in the frontal lobes. The patient died of bronchopneumonia at the age of 71. Neuropathological examination confirmed typical pathological changes of PSP, such as neuronal loss, neurofibrillary tangles, and fibrillary gliosis in the subcortical nuclei. Gallyas-Braak silver impregnation revealed neurofibrillary tangles, silver-positive glia and thread-like structures in degenerating subcortical nuclei. In addition to these classical lesions, the argentophilic structures were detected in the cerebral cortex, cortical white matter and cerebellar white matter. In the cerebral cortex, they were abundant mostly in the precentral gyrus and subcortical white matter. Immunohistochemical studies revealed that most silver-positive structures were also tau 2 antibody-positive. Thus, these argentophilic structures seemed to be closely related to abnormal tau protein. Their distribution in this case implies that lesions related to abnormal tau protein may occur more extensively in the brains of PSP than expected.

摘要

我们报告一例病程长达五年的进行性核上性麻痹(PSP)尸检病例。一名67岁男性患有步态障碍和精神衰退。71岁时的神经学检查发现假性球麻痹、水平性眼球运动麻痹和躯干肌张力障碍,遂诊断为PSP。还注意到包括健忘和性格改变在内的精神衰退,患者有时会出现间歇性昏迷状态。头颅计算机断层扫描和磁共振成像显示中度脑萎缩,主要位于额叶。患者于71岁时死于支气管肺炎。神经病理学检查证实了PSP的典型病理变化,如皮质下核团的神经元丢失、神经原纤维缠结和纤维性胶质细胞增生。Gallyas-Braak银浸染显示在退化的皮质下核团中有神经原纤维缠结、银阳性胶质细胞和丝状结构。除了这些典型病变外,在大脑皮质、皮质白质和小脑白质中也检测到嗜银结构。在大脑皮质中,它们主要大量存在于中央前回和皮质下白质。免疫组织化学研究显示,大多数银阳性结构也为tau 2抗体阳性。因此,这些嗜银结构似乎与异常tau蛋白密切相关。它们在该病例中的分布表明,与异常tau蛋白相关的病变在PSP患者大脑中的发生范围可能比预期更广。

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