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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.
GLOSSARY
- AD=
- Alzheimer disease;
- AOS=
- apraxia of speech;
- CBD=
- corticobasal degeneration;
- CBS=
- corticobasal syndrome;
- CJD=
- Creutzfeldt-Jakob disease;
- cr-CBD=
- clinical research criteria for probable corticobasal degeneration;
- DLB=
- dementia with Lewy bodies;
- FTD=
- frontotemporal dementia;
- FTLD-TDP=
- frontotemporal lobar degeneration with TDP-43 immunoreactive inclusions;
- GRN=
- granulin;
- p-CBD=
- possible corticobasal degeneration criteria;
- PD=
- Parkinson disease;
- PNFA=
- progressive nonfluent aphasia;
- PPA=
- primary progressive aphasia;
- PSP=
- progressive supranuclear palsy;
- PSPS=
- progressive supranuclear palsy syndrome
Footnotes
Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
Supplemental data at www.neurology.org
- Received June 6, 2012.
- Accepted August 30, 2012.
- © 2013 American Academy of Neurology
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