TY - JOUR T1 - Preparing for the age of therapeutic trials in frontotemporal lobar degeneration JF - Journal of Neurology, Neurosurgery & Psychiatry JO - J Neurol Neurosurg Psychiatry SP - 115 LP - 115 DO - 10.1136/jnnp-2021-327497 VL - 93 IS - 2 AU - David Irwin Y1 - 2022/02/01 UR - http://jnnp.bmj.com/content/93/2/115.abstract N2 - Frontotemporal lobar degeneration (FTLD) comprises a spectrum of heterogenous clinicopathological neurodegenerative disorders and neuropathological examination at autopsy remains the gold standard for diagnosis.1 However, detection of pathogenic mutations in genes known to associate with either FTLD-Tau or FTLD-TDP neuropathology in ~20%–30% of all FTLD with familial disease provides an accurate antemortem molecular diagnosis in these individuals, even in the presymptomatic stage. Therefore, several clinical trials for agents targeting disease-specific mechanisms associated with forms of familial FTLD are planned or currently underway. Reliable clinical outcome measures to detect and track early disease are crucial to facilitate the success of these efforts. Peakman et al 2 perform a detailed cross-sectional and longitudinal analysis of two commonly used clinical rating scales (ie, extended clinical dementia rating scale (CDR+NACC FTLD) and the Frontotemporal Dementia Rating Scale (FRS)) in the large international … ER -