Frontotemporal Lobar Degeneration (FTLD) is the term used to describe a group of neurodegenerative disorders with predominant involvement of frontal and temporal lobes.3 It is a pathological and genetically heterogeneous condition4 associated with the clinical entity of frontotemporal dementia (FTD) which represents one of the most common neurodegenerative disorders, generally having an early onset and impairment of cognition, language or behavior.5 Histologically, almost half of such cases have tau-based pathology, half TDP-43-based pathology, and about 5% have FUS-based pathology.6 Here, MAPT is linked to frontotemporal dementia.