Clinical Recognition of Frontotemporal Dementia with Right Anterior Temporal Predominance; Consensus Recommendations of the International Working Group
Ulugut, H.; Younes, K.; Montembeault, M.; Bertoux, M.; Irish, M.; Kumfor, F.; Fumagalli, G.; Samanci, B.; Illan-Gala, I.; Thompson, J. C.; Santillo, A. F.; Englund, E.; Landqvist-Waldo, M.; Riedl, L.; Vandenstock, J.; Vandenbulcke, M.; Vanderberghe, R.; Laforce, R.; Ducharme, S.; Pressman, P. S.; Caramelli, P.; deSouza, L. C.; Takada, L. T.; Gurvit, H.; Diehl-Schmid, J.; Galimberti, D.; Pasquier, F.; Weintraub, S.; Miller, B. L.; Sturm, V. E.; Whitwell, J.; Boeve, B.; Rohrer, J. D.; Piguet, O.; Gorno Tempini, M. L.; Josephs, K. A.; Snowden, J.; Rowe, J.; Warren, J.; Rankin, K. P.; Pijnenburg,
Show abstract
Accurate diagnosis of frontotemporal dementia (FTD) with right anterior temporal lobe (RATL) predominance remains challenging due to lack of clinical characterization, and standardized terminology. The recent research of the International Working Group (IWG) identified common symptoms but also unveiled broad terminologies lacking precision and operationalization, with risk of misdiagnoses, inappropriate referrals and poor clinical management. Based on the published evidence (91267 articles screened) and expert opinion (105 FTD specialists across 52 centers), the IWG delineates three primary domains of impairment causing behavioral, memory and language problems: (i) multimodal knowledge of non-verbal information including people, living beings, landmarks, flavors/odors, sounds, bodily sensations, emotions and social cues; (ii) socioemotional behavior encompassing emotion expression, social response and motivation; and (iii) prioritization for focus on specific interests, hedonic valuation and personal preferences. This study establishes a consensus on clinical profile, phenotypic nomenclature, and future directions to enhance diagnostic precision and therapeutic interventions.
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