Psychogeriatric Center of Daigo Hospital, Miyazaki, Japan.
Neuropathology. 2009 Dec;29(6):649-54. doi: 10.1111/j.1440-1789.2009.01059.x. Epub 2009 Sep 22.
Non-Alzheimer-type dementias occur in association with a variety of pathological conditions that include a group of diseases characterized by atrophy of the frontal and temporal lobes. Frontotemporal dementia (FTD) is a clinical entity that comprises at least two distinct diseases: Pick's disease with Pick bodies and frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U). The vast majority of FTLD-U is now referred to as FTLD-TDP, following the recent discovery of TAR DNA-binding protein of 43 kDa (TDP-43) as the major constituent of the ubiquitin-positive inclusions. FTLD-TDP, but not Pick's disease with Pick bodies, is often associated with motor neuron disease (MND). MND is a group of diseases in which the central nervous system lesions were long believed to be confined to the motor neuron system. In other words, MND was not considered to be associated with other neurological symptoms such as dementia. Nevertheless, more than 200 FTD cases associated with clinical MND have been reported in Japan since 1964. Neuropathologically, MND in such FTD cases was essentially similar to MND in cases without dementia. The combination of FTD and MND was so characteristic that we considered these cases comprise a unique clinicopathological subgroup of FTD. FTD with MND and the classical MND without dementia share the occurrence of ubiquitinated TDP-43-positive inclusions, a finding that could be a key to unlock the pathological backgrounds of both diseases.
非阿尔茨海默病型痴呆与多种病理状况相关，其中包括一组以额颞叶萎缩为特征的疾病。额颞叶痴呆（FTD）是一种临床实体，至少包括两种不同的疾病：Pick 病伴 Pick 小体和额颞叶变性伴泛素阳性包涵体（FTLD-U）。现在，绝大多数 FTLD-U 被称为 FTLD-TDP，这是在最近发现 TAR DNA 结合蛋白 43 kDa（TDP-43）为泛素阳性包涵体的主要成分之后。FTLD-TDP 但不是 Pick 病伴 Pick 小体，常与运动神经元病（MND）相关。MND 是一组疾病，其中中枢神经系统病变长期以来被认为局限于运动神经元系统。换句话说，MND 不被认为与其他神经症状如痴呆有关。然而，自 1964 年以来，在日本已经报告了超过 200 例与临床 MND 相关的 FTD 病例。神经病理学上，此类 FTD 病例中的 MND 与无痴呆的 MND 基本相似。FTD 伴 MND 和经典的无痴呆 MND 共同具有泛素化 TDP-43 阳性包涵体的发生，这一发现可能是揭示这两种疾病病理背景的关键。
