Вопросы диагностики и лечения лобно-височных дегенераций

2020 
Frontotemporal degeneration (FTD) is a heterogeneous group of progressive neurodegenerative diseases associated with a predominant lesion of the frontal and/or anterior temporal lobes of the brain. FTD is at the fourth place in the list of causes of severe neurocognitive disorders in all age groups and at the second place among patients of presenile age (up to 65 years). FTD is a genetically determined disease. In 30-40%, there is a family history, which is characterized by an autosomal dominant type of transmission in up to 15% of cases. Usually FTD begins in the fifth or sixth decade of life, but a later onset is possible. The article discusses the main clinical variants of FTD. The behavioral variant of FTD accounts for more than half of the cases of FTD and is characterized by a combination of cognitive, behavioral, and emotional-affective symptoms, atrophy of the frontal and temporal lobes of the brain. In primary progressive aphasia (PPA), speech disorders are the most significant clinical symptoms for at least two years. The clinical features of PPA depend on the localization of the pathological process. The division into clinical variants of FTD is relevant only in the first years of the disease. In the future, the differences between them are erased, and the status may contain several clinical variants simultaneously. In the treatment of FTD, a comprehensive approach is presented, including symptomatic therapy, as well as non-drug treatments (educational program, psychological support, cognitive training, speech therapy). Accurate analysis of emotional and behavioral symptoms in FTD can help predict the course of the pathological process, optimize symptomatic treatment, and there by improve the quality of life of patients and their relatives.
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