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This Article Figures Only Full Text Full Text (PDF) CME: Take the course for this article: Volume 72, Number 8, February 24, 2009 JapaneseTranslation Correspondence: Submit a response Alert me when this article is cited Alert me when Correspondence are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Piguet, O. Articles by Hodges, J. R. Search for Related Content PubMed PubMed Citation Articles by Piguet, O. Articles by Hodges, J. R. Related Collections Clinical neurology examination Frontotemporal dementia

Sensitivity of current criteria for the diagnosis of behavioral variant frontotemporal dementia

From the Prince of Wales Medical Research Institute and the University of New South Wales (O.P., M.H., J.R.H.), Australia; Department of Neurology (B.P.S.), Father Muller Medical College, Mangalore, Karnataka, India; Department of Clinical Neurosciences (B.P.S., J.R.H.), Addenbrooke’s Hospital; and Neurology Department (C.M.K.), Southampton General Hospital, UK.

Address correspondence and reprint requests to Prof. John Hodges, Prince of Wales Medical Research Institute, Barker St, Randwick NSW 2031 Australia j.hodges{at}powmri.edu.au

Background: Diagnosis of behavioral variant frontotemporal dementia (bvFTD) relies on criteria that are constraining and potentially ambiguous. Some features are open to clinical interpretation and their prevalence unknown. This study investigated the sensitivity of current diagnostic criteria in a large group of patients with bvFTD.

Methods: Forty-five patients with clear evidence of bvFTD as judged by progressive clinical decline (>3 years) with marked frontal features and significant frontal brain atrophy on brain MRI were included. Thirty-two have died; pathologic confirmation of frontotemporal lobar degeneration was found in all 18 coming to autopsy. We established the prevalence of core and supportive diagnostic features at presentation and with disease progression.

Results: Only 25/45 patients (56%) showed all five core features necessary for a diagnosis of bvFTD at initial presentation and 33/45 (73%) as their disease progressed. Two core features, emotional blunting and loss of insight, were never observed in 25% and 13% of cases. Executive dysfunction, hyperorality, mental inflexibility, and distractibility were the only supportive features present in >50% of cases at initial presentation. Although not a diagnostic feature, impaired activities of daily living was present in 33/45 patients (73%).

Conclusions: Strict application of the criteria misses a significant proportion of patients. Many supportive features have low prevalence and are clinically not useful. Revision of the criteria to include level of certainty (definite, probable, possible) dependent on the number of features present and the presence of ancillary information (e.g., brain atrophy, neuropsychological abnormalities, impaired activities of daily living) is encouraged.

ACE = Addenbrooke’s Cognitive Examination; ADL = activities of daily living; bvFTD = behavioral variant frontotemporal dementia; MMSE = Mini-Mental State Examination.

Funded in part by an MRC program grant to J.R.H. J.R.H. is supported by an Australian Research Council Federation Fellowship. O.P. is supported by a National Health and Medical Research Council of Australia Clinical Career Development Award Fellowship (#510184).

Disclosure: The authors report no disclosures.

Received August 3, 2008. Accepted in final form November 12, 2008.

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