Lillo Patricia, Hodges John R
Prince of Wales Medical Research Institute, Barker St, Randwick, New South Wales 2031, Australia.
J Clin Neurosci. 2009 Sep;16(9):1131-5. doi: 10.1016/j.jocn.2009.03.005. Epub 2009 Jun 24.
Advances in genetics and pathology have supported the idea of a continuum between frontotemporal dementia (FTD) and motor neurone disease (MND), which is strengthened by the discovery of the trans-activating responsive (Tar) sequence DNA binding protein (TDP-43) as a key component in the underlying pathology of FTD, FTD-MND and sporadic and familial MND patients. MND is a multisystem disorder associated with cognitive and behavioural changes which in some instances reaches the criteria for FTD, while a proportion of patients with FTD develop frank MND. We review the overlap between FTD and MND, emphasizing areas of controversy and uncertainty.
遗传学和病理学的进展支持了额颞叶痴呆（FTD）和运动神经元病（MND）之间存在连续性的观点，反式激活应答（Tar）序列DNA结合蛋白（TDP-43）作为FTD、FTD-MND以及散发性和家族性MND患者潜在病理学中的关键成分被发现，这进一步强化了该观点。MND是一种与认知和行为改变相关的多系统疾病，在某些情况下符合FTD的标准，而一部分FTD患者会发展为明显的MND。我们综述了FTD和MND之间的重叠，强调了存在争议和不确定性的领域。
