Yokoo H, Oyama T, Hirato J, Sasaki A, Nakazato Y
Department of Pathology, Gunma University School of Medicine, Japan.
Acta Neuropathol. 1994;88(3):267-72. doi: 10.1007/BF00293404.
An autopsy case of unusual Pick's disease in a 61-year-old male is described. Findings included severe atrophy of the frontal and temporal lobes, pyramidal tracts and basal ganglia accompanied by numerous intraneuronal argyrophilic hyaline inclusions. His neurological symptoms were constantly progressive during the 12-year course, characterized by akinesia and emotional incontinence. The inclusions were round, well-demarcated, slightly eosinophilic and intensely argyrophilic bodies in the perikarya, and distributed mainly in the subiculum and Sommer's sector of the hippocampus, amygdala and affected gyri. Immunocytochemically, they contain antigenic determinants of both phosphorylated and nonphosphorylated neurofilaments, but were negative for ubiquitin. Ultrastructurally, they were composed primarily of skeins of neurofilaments intermingled with cell organelles. Tubular profiles studded with granular substances, previously reported as a feature of the generalized variant of Pick's disease, and Hirano body-like lattice structures were occasionally observed in the inclusions. This case represents a slowly progressive neurodegenerative disorder characterized by fronto-temporal lobar atrophy and might by categorized as a variant of Pick's disease. However, some unusual properties of neuronal inclusions may suggest a different pathogenesis from that in classical Pick's disease.
本文描述了一例61岁男性的罕见皮克病尸检病例。检查结果包括额叶、颞叶、锥体束和基底神经节严重萎缩，并伴有大量神经元内嗜银性透明包涵体。在12年病程中，其神经症状持续进展，表现为运动不能和情感失禁。这些包涵体呈圆形，边界清晰，在核周体中呈轻度嗜酸性、强嗜银性，主要分布于海马的下托和 Sommer 区、杏仁核及受累脑回。免疫细胞化学显示，它们含有磷酸化和非磷酸化神经丝的抗原决定簇，但泛素检测呈阴性。超微结构上，它们主要由与细胞器交织的神经丝束组成。包涵体中偶尔可见散在颗粒物质的管状结构（先前报道为皮克病泛发型的特征）以及 Hirano 小体样晶格结构。该病例代表一种以额颞叶萎缩为特征的缓慢进展性神经退行性疾病，可能归类为皮克病的一种变异型。然而，神经元包涵体的一些不寻常特性可能提示其发病机制与经典皮克病不同。
