Clinical recognition of frontotemporal dementia with right temporal predominance: a consensus statement from the International Working Group
Ulugut H., Younes K., Montembeault M., Bertoux M., Irish M., Kumfor F., Fumagalli GG., Samanci B., Illán-Gala I., Thompson JC., Santillo AF., Englund E., Landqvist Waldö M., Riedl L., Van den Stock J., Vandenbulcke M., Vandenberghe R., Laforce R., Ducharme S., Pressman PS., Caramelli P., Cruz de Souza L., Takada LT., Gurvit H., Diehl-Schmid J., Galimberti D., Pasquier F., Weintraub S., Miller BL., Sturm VE., Whitwell JL., Boeve B., Rohrer JD., Piguet O., Gorno-Tempini ML., Josephs KA., Snowden J., Rowe JB., Warren JD., Rankin KP., Pijnenburg YAL., Pijnenburg Y., Huey ED., Krudop W., Miyagawa T., Lebouvier T., Lee SM., Moon SY., Thompson S., Ramanan S., Henderson S., Loi S., Ossenkoppele R., Ellajosyula R., Migliaccio R., Pressman P., Hansson O., Piguet O., Jung NY., Emre M., Didic M., Montembeault M., Tastevin M., Schroeter ML., Husain M., Rouse M., Jones M., Filippi M., Mesulam M., Mendez MF., Tempini MG., Pintus M., Sacchi L., Russell L., Jiskoot L., de Souza LC., Horne K., Josephs KA., Rascovsky K., Narayanan J., Lagarde J., Fields J., Hazelton J., Schmid JD., den Stock JV., Rowe J., Gala II., Rosen H., Seelaar H., Quang H., Kuchcinski G., Piga G., Fumagalli G., Robinson G., Barkhof F., Di Lorenzo F., Duits F., Agosta F., Kim EJ.
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) by using the nominal group technique, 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.