Right temporal lobe variant of frontotemporal dementia.

We present two women with the right temporal lobe variant (RTLV) of frontotemporal dementia (FTD) and analyse the clinical features that are determined by the anatomical distribution of atrophy. Each of our patients displayed different clinical and radiological profiles which were in line with findings reported by other authors. One of two patients carries a novel mutation in the granulin gene. FTD is heterogeneous with regard to clinical manifestation, genetics, distribution of cortical atrophy and underlying disease. Its clinical manifestations are related to the distribution of the cortical atrophy. The RTLV of FTD is an uncommon entity. There is no consensus about its name despite the fact that its clinical and radiological features are well-defined and distinguish it from other types of FTD including semantic dementia.