This is an important new article on corticobasal degeneration. This is a revision of the diagnostic criteria for CBD. This article looks to be an amalgam of thinking of movement disorder specialist (such as our friend Irene Litvan, MD) and memory disorder specialists (such as our friends at UCSF Memory & Aging Center, including Adam Boxer, MD). It looks like compromises were made to keep both sides happy.
Researchers looked at 267 autopsy-confirmed cases of CBD, trying to glean overlaps in clinical features. Four phenotypes emerged for CBD:
- corticobasal syndrome (CBS)
- frontal behavioral-spatial syndrome (FBS)
- nonfluent/agrammatic variant of primary progressive aphasia (naPPA)
- progressive supranuclear palsy syndrome (PSPS). Note that this type can only be diagnosed as possible CBD.
This research and the update of the diagnostic criteria is only possible through brain donation. If you would like to make arrangements for your own brain donation or a family member’s brain donation, start with Brain Support Network.
The abstract is copied below.
The full article is available at no charge here:
Neurology. 2013 Jan 29;80(5):496-503.
Criteria for the diagnosis of corticobasal degeneration.
Armstrong MJ1, Litvan I, Lang AE, Bak TH, Bhatia KP, Borroni B, Boxer AL, Dickson DW, Grossman M, Hallett M, Josephs KA, Kertesz A, Lee SE, Miller BL, Reich SG, Riley DE, Tolosa E, Tröster AI, Vidailhet M, Weiner WJ.
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.
PubMed ID#: 23359374 (see pubmed.gov for the abstract)