Tan Chun-Feng, Toyoshima Yasuko, Kakita Akiyoshi, Takahashi Hitoshi
Department of Pathology, Brain Research Institute, Niigata University, 1-757 Asahimachi, Chuo-ku, Niigata 951-8585, Japan.
Brain Nerve. 2009 Nov;61(11):1319-27.
Findings of clinical, neuropathological and biochemical studies have supported the idea that frontotemporal lobar degeneration with ubiquitin inclusions (FTLD-U) and amyotrophic lateral sclerosis (ALS) are part of a neurological disease spectrum. This concept is now further strengthened by the recent discovery of a 43-kDa transactivating responsive sequence DNA-binding protein (TDP-43) as a key component of the underlying neuropathology of FTLD-U, ALS with dementia (ALS-D) and ALS. Here we describe the clinicopathological features of selected autopsy cases belonging to this disease spectrum, and discuss the neuropathological similarities and differences between FTLD-U and ALS-D, with special reference to the morphology, distribution and density of ubiquitin/TDP-43-positive abnormal structures, along with a review of the literature.
临床、神经病理学和生物化学研究结果支持了这样一种观点，即伴有泛素包涵体的额颞叶变性（FTLD-U）和肌萎缩侧索硬化症（ALS）是神经疾病谱的一部分。最近发现一种43 kDa的反式激活应答序列DNA结合蛋白（TDP-43）是FTLD-U、伴痴呆的肌萎缩侧索硬化症（ALS-D）和肌萎缩侧索硬化症潜在神经病理学的关键组成部分，这一概念现在得到了进一步强化。在此，我们描述了属于该疾病谱的部分尸检病例的临床病理特征，并讨论了FTLD-U和ALS-D之间神经病理学上的异同，特别提及泛素/TDP-43阳性异常结构的形态、分布和密度，并对相关文献进行了综述。
