Frontotemporal dementia: clinical features, diagnosis, and treatment

Abstract

Frontotemporal dementia (FTD) is the second most common cause of neurodegenerative dementia affecting patients before 65. The classic clinical phenotypes of the disease include the behavioral variant of FTD and variants with a predominant speech disorder - primary progressive aphasia (agrammatic, semantic and logopenic variants). Often, classic FTD phenotypes can be associated with atypical parkinsonism such as corticobasal syndrome and progressive supranuclear palsy, and motor neuron disease. The disease is also heterogeneous from a pathophysiological point of view. It may be based on one of three pathological processes, while up to 40% of cases have a hereditary burden. Currently there have been described mutations in about 20 genes associated with FTD. Given the wide variety of clinical presentation, FTD may be a phenocopy of other diseases, which makes it difficult to diagnose, complicates the differential diagnosis and delays the correct diagnosis for several years. Poor awareness of the disease and its clinical features among clinicians is one of the reasons for the lack of data on the prevalence of the disease in the Russian Federation. In addition, the identification of families with genetic forms of the disease and asymptomatic carriers is an important step in the formation of a strategy for helping this category of patients when approaches to pathogenetic therapy appear. This review of the literature presents modern ideas about the clinical picture, features of diagnosis and differential diagnosis of various clinical variants of FTD. The current understanding of approaches to pharmacological and non-pharmacological therapy is also presented.