Pressman Peter S, Miller Bruce L
Memory and Aging Center, Department of Neurology, University of California at San Francisco, San Francisco, California.
Memory and Aging Center, Department of Neurology, University of California at San Francisco, San Francisco, California.
Biol Psychiatry. 2014 Apr 1;75(7):574-81. doi: 10.1016/j.biopsych.2013.11.006. Epub 2013 Nov 13.
Frontotemporal dementia was documented over a century ago. The last decade, however, has seen substantial changes in our conceptions of this increasingly recognized disorder. Different clinical variants have been delineated, the most common of which is the behavioral variant (bvFTD). Updated diagnostic criteria have been established. New histopathological findings and genetic etiologies have been discovered. Research continues to uncover molecular mechanisms by which abnormal proteins accumulate in degenerating brain tissue. Novel neuroimaging techniques suggest that functional networks are diminished in bvFTD that might be relevant to empathy and social behavior. Despite rapid advances in our understanding of bvFTD, the disease is still under-recognized and commonly misdiagnosed. The result is inappropriate patient care. Recognizing the various presentations of bvFTD and its histological and genetic subtypes might further diagnosis, treatment, and research.
额颞叶痴呆在一个多世纪前就有记载。然而，在过去十年中，我们对这种日益受到认可的疾病的认识发生了重大变化。已经明确了不同的临床变体，其中最常见的是行为变异型（bvFTD）。已经建立了更新的诊断标准。发现了新的组织病理学发现和遗传病因。研究继续揭示异常蛋白质在退化脑组织中积累的分子机制。新的神经成像技术表明，bvFTD中的功能网络有所减少，这可能与同理心和社会行为有关。尽管我们对bvFTD的理解有了迅速进展，但该疾病仍未得到充分认识，且常被误诊。结果是患者护理不当。认识到bvFTD的各种表现及其组织学和遗传亚型可能会进一步促进诊断、治疗和研究。
