An autopsy case of progressive supranuclear palsy. Pallido-nigro-luysian type with argyrophilic grains clinically presenting with personality and behavioral changes
An autopsy case of progressive supranuclear palsy. Pallido-nigro-luysian type with argyrophilic grains clinically presenting with personality and behavioral changes
复制标题
进行性核上性麻痹的尸检病例。
DOI：
10.1111/neup.12815
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发表时间：
2022
期刊：
影响因子：
2.3
通讯作者：
Hanajima R.
中科院分区：
文献类型：
--
作者：
Suzuki Y;Adachi T;Sakuwa M;Sakata R;Takigawa H;Hasegawa M;Hanajima R.
Pallido ‐ nigro ‐ luysian atrophy（PNLA）是进行性核上性麻痹（PSP）的一种变体。PSP患者有时会表现出精神症状，但很少有关于这些症状与PSP-PNLA相关的报道。在这里，我们报告了一例临床诊断为PSP-额颞叶痴呆（PSP-F）的患者的PSP-PNLA伴嗜银颗粒（AG）。一名被描述为"善良"的74岁男性出现记忆受损、易怒和冷漠。他表现出左旋多巴抵抗型帕金森综合征和姿势不稳脑磁共振成像显示中脑轻度萎缩，海马和颞叶右侧优势萎缩。患者被诊断为PSP伴额叶认知或行为表现（PSP-F）。他在81岁时死于吸入性肺炎。尸检时，肉眼检查显示黑质色素脱失，齿状核、苍白球和丘脑底核呈灰色变色。在相同区域观察到严重的胶质增生。苍白球中有许多磷酸化tau免疫反应可疑的簇状星形胶质细胞。在黑质和丘脑底核中观察到许多神经纤维缠结和神经纤维丝，在额叶皮质中观察到很少的tau聚集体。而杏仁核、内嗅皮层和扣带回前部则有大量的AG，且分布不对称。病理学观察结果使我们将诊断改为PSP ‐ PNLA伴AG。尽管大多数PSP ‐ F病例源自额叶皮质中的tau病理学，但该患者在该位置没有磷酸化tau免疫反应性聚集体。我们的观察结果表明，PSP-F的精神症状应被认为是由于边缘系统AG的存在，而不是额叶tau病理学。
Pallido‐nigro‐luysian atrophy (PNLA) is a variant of progressive supranuclear palsy (PSP). Patients with PSP sometimes show psychiatric signs, but there are few reports about such signs being associated with PSP‐PNLA. Here, we report a case of PSP‐PNLA with argyrophilic grains (AGs) in a patient clinically diagnosed as having PSP‐frontotemporal dementia (PSP‐F). A 74‐year‐old man described as “kind” presented with impaired memory, irritability, and apathy. He showed levodopa‐resistant parkinsonism and postural instability. Brain magnetic resonance imaging revealed mild atrophy of the midbrain and right‐side‐dominant atrophy of the hippocampus and temporal lobe. The patient was diagnosed as having PSP with frontal lobe cognitive or behavioral presentations (PSP‐F). He died of aspiration pneumonia at age 81. At autopsy, macroscopic examination revealed depigmentation of the substantia nigra and grayish discoloration of the dentate nucleus, globus pallidus, and subthalamic nucleus. Severe gliosis was observed in the same regions. There were many phosphorylated tau‐immunoreactive equivocal tufted astrocytes in the globus pallidus. Many neurofibrillary tangles and neuropil threads were observed in the substantia nigra and subthalamic nucleus, and few tau aggregates were observed in the frontal cortex. In contrast, AGs were abundant in the amygdala, entorhinal cortex, and anterior cingulate gyrus, with an asymmetric distribution. The pathological observations led us to change the diagnosis to PSP‐PNLA with AGs. Although most cases of PSP‐F derive from tau pathology in the frontal cortex, this patient did not have phosphorylated tau‐immunoreactive aggregates in that location. Our observations suggest that the psychiatric signs of PSP‐F should be considered as being due to the presence of limbic AGs, not frontal tau pathology.