Tsuchiya Kuniaki, Nakayama Hiroshi, Haga Chie, Oshima Kenichi, Niizato Kazuhiro, Arai Tetsuaki, Matsushita Masaaki, Akiyama Haruhiko
Department of Laboratory Medicine and Pathology, Tokyo Metropolitan Matsuzawa Hospital, Kamikitazawa, Setagaya-ku, Japan.
Acta Neuropathol. 2005 Jul;110(1):57-68. doi: 10.1007/s00401-005-1025-5. Epub 2005 Jun 17.
We investigated clinicopathologically four Japanese autopsy cases of diffuse neurofibrillary tangles with calcification (DNTC), which has been believed to be characterized by temporal or temporofrontal circumscribed lobar atrophy, and examined the distribution of their cerebral cortical lesions using hemisphere specimens. The lesions were classified into three categories (slight, moderate, and severe). Severe lesions were present in the temporal lobes and insular gyri of all four cases, consistent with the studies reported to date. In contrast, severe lesions were encountered in the parietal lobe of case 1 and moderate lesions were found in the parietal lobes of cases 2-4. Furthermore, moderate lesions of the precentral gyrus were present in cases 2-4, and moderate lesions of the postcentral gyrus were encountered in all four cases. We postulate that the distribution of cerebral cortical lesions in DNTC is more widespread than previously assumed. Our data also indicate that the unusual clinical signs of DNTC reported by several Japanese researchers, including parietal signs such as apraxia and agnosia, are roughly consistent with the topographic distribution of cerebral cortical lesions in DNTC elucidated in this study.
我们对4例日本弥漫性神经原纤维缠结伴钙化（DNTC）的尸检病例进行了临床病理研究，该病被认为以颞叶或颞额叶局限性脑叶萎缩为特征，并使用大脑半球标本检查了其大脑皮质病变的分布情况。病变分为三类（轻度、中度和重度）。所有4例患者的颞叶和岛叶均存在重度病变，这与迄今为止报道的研究结果一致。相比之下，病例1的顶叶出现重度病变，病例2-4的顶叶发现中度病变。此外，病例2-4的中央前回存在中度病变，所有4例患者的中央后回均出现中度病变。我们推测，DNTC中大脑皮质病变的分布比之前认为的更为广泛。我们的数据还表明，几位日本研究人员报告的DNTC异常临床体征，包括失用症和失认症等顶叶体征，与本研究中阐明的DNTC大脑皮质病变的地形分布大致一致。
