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摘要:This publication covers the presentations held at the 'International Conference on Frontotemporal Dementias' (FTD) in Lund, Sweden, in 2003. The aim of the papers is not only to present recent progress of clinical and basic research, but also to give an overview of the genetics leading up to new entities such as FDT-17 and FDT-3. Clinical and pathological variants of the three key forms of FDT, Pick's disease, frontal lobe degeneration of non-Alzheimer type and motor neuron disease with dementia are described as well as associated conditions such as semantic dementia and progressive non fluent aphasia. The clinical analysis focuses upon cognition, personality, emotional and social behavior in FTD correlated with neuroimaging, pathology, and genetic and neurochemical findings. Further, the criteria of early diagnosis, pharmacological treatment and strategies for management and care are discussed and analyzed. The announcement of the Association for Frontotemporal Dementias in the USA and the establishment of the Swedish National Competence Centre for FDT in Lund mirrors the growing awareness of the importance of the topic.