Criteria for the diagnosis of corticobasal degeneration
Abstract
Current criteria for the clinical diagnosis of pathologically confirmed corticobasal degeneration (CBD) no longer reflect the expanding understanding of this disease and its clinicopathologic correlations. An international consortium of behavioral neurology, neuropsychology, and movement disorders specialists developed new criteria based on consensus and a systematic literature review. Clinical diagnoses (early or late) were identified for 267 nonoverlapping pathologically confirmed CBD cases from published reports and brain banks. Combined with consensus, 4 CBD phenotypes emerged: corticobasal syndrome (CBS), frontal behavioral-spatial syndrome (FBS), nonfluent/agrammatic variant of primary progressive aphasia (naPPA), and progressive supranuclear palsy syndrome (PSPS). Clinical features of CBD cases were extracted from descriptions of 209 brain bank and published patients, providing a comprehensive description of CBD and correcting common misconceptions. Clinical CBD phenotypes and features were combined to create 2 sets of criteria: more specific clinical research criteria for probable CBD and broader criteria for possible CBD that are more inclusive but have a higher chance to detect other tau-based pathologies. Probable CBD criteria require insidious onset and gradual progression for at least 1 year, age at onset ≥50 years, no similar family history or known tau mutations, and a clinical phenotype of probable CBS or either FBS or naPPA with at least 1 CBS feature. The possible CBD category uses similar criteria but has no restrictions on age or family history, allows tau mutations, permits less rigorous phenotype fulfillment, and includes a PSPS phenotype. Future validation and refinement of the proposed criteria are needed.
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Supplementary Material
STUDY FUNDING
The 1st International CBD Investigators Meeting was funded by a donation from Irene Pollin and the late Abe Pollin and from the Litvan Neurological Research Foundation. Melissa J. Armstrong received support as an Edmond J. Safra fellow at Toronto Western Hospital while working on this project.
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© 2013 American Academy of Neurology.
Publication History
Received: June 6, 2012
Accepted: August 30, 2012
Published online: January 28, 2013
Published in print: January 29, 2013
Disclosure
The authors report no disclosures relevant to the manuscript. Go to Neurology.org for full disclosures.
Authors
Author Contributions
Melissa J. Armstrong, MD: analysis or interpretation of the data, drafting the manuscript, revising the manuscript. Irene Litvan, MD: design or conceptualization of the study, analysis or interpretation of the data, drafting the manuscript, revising the manuscript. Anthony E. Lang, MD: analysis or interpretation of the data, drafting the manuscript, revising the manuscript. Thomas H. Bak, MD: analysis or interpretation of the data, revising the manuscript. Kailash P. Bhatia, MD: analysis or interpretation of the data, revising the manuscript. Barbara Borroni, MD: analysis or interpretation of the data, revising the manuscript. Adam L. Boxer, MD, PhD: analysis or interpretation of the data, revising the manuscript. Dennis W. Dickson, MD: analysis or interpretation of the data, revising the manuscript. Murray Grossman, MD: analysis or interpretation of the data, revising the manuscript. Mark Hallett, MD: analysis or interpretation of the data, revising the manuscript. Keith A. Josephs, MD: analysis or interpretation of the data, revising the manuscript. Andrew Kertesz, MD: analysis or interpretation of the data, revising the manuscript. Suzee E. Lee, MD: analysis or interpretation of the data, revising the manuscript. Bruce L. Miller, MD: analysis or interpretation of the data, revising the manuscript. Stephen G. Reich, MD: analysis or interpretation of the data, revising the manuscript. David E. Riley, MD: analysis or interpretation of the data, revising the manuscript. Eduardo Tolosa, MD: analysis or interpretation of the data, revising the manuscript. Alexander I. Tröster, PhD: analysis or interpretation of the data, revising the manuscript. Marie Vidailhet, MD: analysis or interpretation of the data, revising the manuscript. William J. Weiner, MD: analysis or interpretation of the data, revising the manuscript.
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