Tauopathies are neurodegenerative disorders characterized by accumulation of abnormally modified forms of the tau-protein, predominantly in frontal, temporal and parietal cortical regions, basal ganglia and in the midbrain. Tauopathies are well defi ned from the molecular biological and biochemical point of view; clinical symptoms may be, however, heterogeneous.
Common signs of frontotemporal lobar degenerations include a more or less prominent syndrome of frontotemporal dementia. On the other hand, the clinical picture of frontotemporal dementia may not be caused by a tauopathy only; in many cases the underlying etiopathogenic cause is diff erent.
The aim of our review is to classify the relationship between tauopathies and frontotemporal lobar degenerations. This review contains a proposal of a practical approach to refi ning clinical diagnosis of the diff erent tauopathies