Frontotemporal lobar degeneration (FTLD) encompasses a variety of clinical and genetic progressive neurodegenerative syndromes, which include the behavioural variant of frontotemporal dementia (bvFTD), primary progressive aphasia (PPA), corticobasal syndrome (CBS), and progressive supranuclear palsy (PSP). Tomislav Babic, Natalia E. Drosopoulou and Henry J. Riordan, at Worldwide Clinical Trials point out the challenges of drug development in behavioural variant frontotemporal dementia.