Des troubles comportementaux aux criteres de demence fronto-temporale en pratique

Since the first descriptions of circumscribed frontotemporal atrophies, and the first state-ment published by the Lund and Manchester groups, consensus clinical and pathologicalcriteria for frontotemporal dementia (FTD) have been increasingly refined. The last inter-national behavioural variant FTD criteria (FTDC) (Rascovsky et al., 2011) are the mostsensitive, operational and reliable, for the clinical syndrome. Previously exclusion features,like early and severe amnestic syndrome or spatial disorientation, which turn out to be notso rare, are taken into account, as well as imaging, and biomarkers suggestive of otherpathologies like Alzheimer’s disease. So far, clinical features do not seem very helpful inpredicting the underlying histopathology, although there are some clues, mainly related toneurological features (e.g. motor neuron disease, extra-pyramidal symptoms or languagedisorders), or associated disorders (e.g. Paget disease of bone) or genetics. BvFTD remains adifficultdiagnosisatveryearlystage,whichaccountsforthedelayofdiagnosis,especiallyinlate onset, where the frontotemporal atrophy may not be striking. At very young onset,psychiatric diseases must be ruled out. More systematic assessment of social cognitioncould be helpful. Further biomarkers are expected. Systematic use of recent criteria, forBvFTD and other neurodegenerative diseases especially AD, will contribute to make earlyand correct diagnoses in excluding or suggesting alternative diagnoses. Post-mortemassessment, with detailed recording of clinical information, is essential to progress.# 2013 Elsevier Masson SAS. All rights reserved.