Institute for Medical Science of Aging, Aichi Medical University, Nagakute-cho, Aichigun, Aichi-ken 480-1195, Japan.
J Neurol Sci. 2010 Mar 15;290(1-2):177-82. doi: 10.1016/j.jns.2009.11.002.
Frontotemporal lobar degeneration-tau, Pick's disease subtype (PiD) is one of the major types of frontotemporal dementia, but its pathogenesis and disease mechanisms remain unclear. Here, we report a case of very early PiD. The patient was a 63-year-old healthy woman without dementia or any apparent psychosis. She was admitted to the hospital with multiple organ failure, and died three days later. The brain weighed 1050g and showed focal atrophy of the parahippocampal gyrus and right medial temporal lobe. Microscopically, neuronal loss and gliosis were limited to the atrophic areas. Surprisingly, Pick bodies (PiBs) and ballooned neurons were abundant throughout the bilateral temporal cortices, including the dentate gyrus. Cortical lamination of PiBs was predominant in the upper layer (layer II>VI), and the size of early PiBs tended to be smaller than that in severely affected areas. Numerous glial tau-positive inclusions (astrocytic inclusions, oligodendroglial coiled bodies, and threads) were found not only in the cerebral cortex but also in the temporal white matter. The neuropathological findings in this case suggest that PiB formation started long before the appearance of clinical symptoms and that PiB formation originating from small neurons may differ from other tau aggregations such as neurofibrillary tangles.
额颞叶变性-tau，皮克病亚型（PiD）是额颞痴呆的主要类型之一，但其发病机制和疾病机制仍不清楚。在这里，我们报告了一例非常早期的 PiD。患者为 63 岁健康女性，无痴呆或任何明显精神病。她因多器官衰竭入院，三天后死亡。脑重 1050g，表现为海马旁回和右侧内侧颞叶局限性萎缩。显微镜下，神经元丢失和神经胶质增生仅限于萎缩区。令人惊讶的是，Pick 体（PiBs）和气球样神经元大量存在于双侧颞叶皮层，包括齿状回。PiBs 的皮层分层以上层（II>VI 层）为主，早期 PiBs 的大小往往比受影响严重的区域小。不仅在大脑皮层，而且在颞叶白质中都发现了大量的胶质tau 阳性包涵体（星形胶质包涵体、少突胶质螺旋体和纤维）。该病例的神经病理学发现表明，PiB 的形成早在临床症状出现之前就已经开始，并且起源于小神经元的 PiB 形成可能与神经原纤维缠结等其他 tau 聚集不同。
