LAUSR

The clinical disorders caused by frontotemporal lobar degeneration pathologies (FTLD) are highly heterogeneous in their pathology and phenotypes. Patients are typically diagnosed as having one of several clinical entities de fi ned by speci fi c clinical criteria, including behav-ioral variant frontotemporal dementia, primary progressive aphasia, progressive supranuclear palsy or corticobasal syndrome. 1 The clini-copathological correlations of these syndromes are often imprecise. 2 The main pathology observed in FTLD patients is FTLD-TDP, named after the TDP-43 protein aggregates found in the brain. According to the type and localization of TDP-43 aggregates in the cortical layers, FTLD-TDP is classi fi ed into fi ve pathological subtypes A to E, with A to C being the most common. A wide range of clinical phenotypes has been associated with FTLD-TDP-A pathology. 3 Patients harboring mutations in the gene encoding progranulin ( GRN ) invariably present with FTLD-TDP-A pathology. However, the majority of FTLD-TDP-A patients does not carry GRN mutations and remain genetically unexplained. Recently, Pottier et al explored transcriptional changes underlying FTLD-TDP, performing RNA-sequencing