Department of Neurology, Vanderbilt University Medical Center, Nashville, TN 37232, USA.
Curr Neurol Neurosci Rep. 2010 Nov;10(6):504-11. doi: 10.1007/s11910-010-0145-z.
Frontotemporal dementias are syndromes of progressive dysfunction of the frontal and/or temporal lobes, either unilaterally or bilaterally. These syndromes were described clinically under the terms "primary progressive aphasia" in the United States and "frontotemporal dementia" in Europe and the United Kingdom. They are diagnosed by the clinical features of a frontal lobe neurobehavioral syndrome, or a language and cognitive deterioration. In recent years, molecular genetic findings in these syndromes, especially the tau and progranulin mutations on chromosome 17, have provided a molecular and genetic foundation for the understanding of frontotemporal dementia. These disorders are distinct from Alzheimer's disease but have some overlap with the syndrome of corticobasal degeneration, and with motor neuron disease. Treatments remain very limited, mainly involving therapy for the mood and behavioral symptoms, but advances in the molecular and genetic understanding of these conditions will hopefully lead to more specific therapies in the future.
额颞叶痴呆是一种以额颞叶功能进行性障碍为特征的综合征，可单侧或双侧受累。这些综合征在美国被描述为“原发性进行性失语症”，在欧洲和英国则被描述为“额颞叶痴呆”。它们是根据额前神经行为综合征的临床特征或语言和认知功能恶化来诊断的。近年来，这些综合征的分子遗传学发现，特别是 17 号染色体上的 tau 和颗粒蛋白基因突变，为理解额颞叶痴呆提供了分子和遗传基础。这些疾病与阿尔茨海默病不同，但与皮质基底节变性和运动神经元病有一些重叠。治疗方法仍然非常有限，主要涉及情绪和行为症状的治疗，但对这些疾病的分子和遗传理解的进展有望在未来带来更具体的治疗方法。
