FTD Matters

Defining bvFTD for the World

The International bvFTD Criteria Consortium

When doctors diagnose a patient's illness, they need to first rule out other explanations for the symptoms and then confirm the suspected diagnosis. To confirm a diagnosis, physicians rely on criteria that leaders in the field develop and then test and validate in studies around the world. These criteria define the disorder by listing the signs and symptoms that should be present as well as the ones that should not (they might be signs of something else). These criteria need regular updating as scientists make new discoveries.

The most widely established diagnostic criteria for the behavioral variant of frontotemporal dementia (bvFTD) have now been in use for a decade (Neary, et al., 1998). Although these consensus criteria provided a much needed standard for frontotemporal dementia research, a growing body of evidence suggests that revisions are needed to improve their applicability. In order for criteria to be useful, they should be simple, current and able to accurately differentiate bvFTD from other similar syndromes.

According to Dr. Katya Rascovsky of the UCSF Memory and Aging Center, "The past 10 years have seen considerable advances in the characterization and diagnosis of bvFTD. On the basis of recent findings, we believe that both researchers and clinicians would benefit from revised and simplified bvFTD criteria integrating the most salient clinical, genetic and imaging characteristics of this disorder."

With the efforts of Dr. Rascovsky, the UCSF Memory and Aging Center has taken a lead in revising the criteria for bvFTD. She coordinates the International bvFTD Criteria Consortium – a group of prominent frontotemporal dementia researchers who have come together to develop new consensus criteria for bvFTD. These updated criteria will

  • be easy to use,
  • allow greater flexibility in how patients can meet diagnostic criteria
  • provide clearer operational definitions,
  • incorporate new genetic and neuroimaging findings,
  • distinguish between probable/possible or bvFTD with definite FTLD pathology, depending on level of diagnostic certainty.

The consortium is currently revising the bvFTD criteria and is close to a consensus. A preliminary multi-site validation of the revised criteria will begin in 2009.

Consortium Members: Bradley F. Boeve MD; Stefano F. Cappa MD; Tiffany W. Chow MD; Charles DeCarli MD; Janine Diehl-Schmid MD; Douglas Galasko MD; Maria-Luisa Gorno-Tempini MD, PhD; Murray Grossman MD; Argye Hillis MD, PhD; John R. Hodges MD; Julene K. Johnson PhD; Keith Josephs MD; Andrew Kertesz MD; Christopher M. Kipps MD; David Knopman MD; Joel H. Kramer PsyD; Facundo Manes MD; Mario F. Mendez MD, PhD; Marsel Mesulam MD; Bruce Miller MD; Chiadi Onyike MD; Florence Pasquier MD; Oliver Piguet PhD; Yolande Pijnenburg MD; Katherine Rankin PhD; Katya Rascovsky PhD; Jonathan Rohrer MD; Howard Rosen MD; Martin Rossor MD; David P. Salmon PhD; William W. Seeley MD; John C. van Swieten MD; Sandra Weintraub PhD

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