Diagnosis and treatment for frontotemporal degenerations

Abstract

Frontotemporal degeneration (FTD) is a heterogeneous group of diseases causing neurodegeneration on the frontal and/or anterior temporal lobes. FTD is the second most common dementia in presenile age (up to 65 years) after Alzheimer’s disease. Usually, FTD is diagnosed at the age of 45-65 years, but an earlier and later onset is possible. Up to 40% of FTD cases have a positive family history. According to the current classification, the following clinical variants of FTD are distinguished: behavioral variant of FTD, agrammatic variant of primary progressive aphasia (PPA) and semantic variant of PPA. As they develop, these clinical syndromes overlap widely with each other and with atypical parkinsonism syndromes (progressive supranuclear paralysis, cortico-basal syndrome) and less often with motor neuron disease. Variations in the clinical features are determined by the localization of the degenerative-atrophic process. 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 disorders. In PPA, speech disorders appear for no apparent reason and progress continuously in the absence or with minimal severity of other cognitive and behavioral disorders for two or more years. The clinical features of PPA depend on the localization of the pathological process. The article reviews a clinical case of an agrammatic variant of PPA. Modern approaches to the diagnosis and management of this group of patients are shown. It seems appropriate to use memantin in patients with agrammatic variant of PPA.