Document Detail

Criteria for the diagnosis of corticobasal degeneration.
MedLine Citation:
PMID:  23359374     Owner:  NLM     Status:  MEDLINE    
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.
Melissa J Armstrong; Irene Litvan; Anthony E Lang; Thomas H Bak; Kailash P Bhatia; Barbara Borroni; Adam L Boxer; Dennis W Dickson; Murray Grossman; Mark Hallett; Keith A Josephs; Andrew Kertesz; Suzee E Lee; Bruce L Miller; Stephen G Reich; David E Riley; Eduardo Tolosa; Alexander I Tröster; Marie Vidailhet; William J Weiner
Related Documents :
23686794 - Xy sex reversal, pontocerebellar hypoplasia and intellectual disability: confirmation o...
24321544 - Recurrent miller fisher syndrome in children.
24126314 - Familial isolated primary hyperparathyroidism due to hrpt2 mutation.
23374744 - Hereditary hyperparathyroidism syndromes.
6696034 - Leber's congenital amaurosis associated with familial juvenile nephronophthisis and con...
14962704 - Dress syndrome associated with hhv-6 reactivation.
Publication Detail:
Type:  Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Neurology     Volume:  80     ISSN:  1526-632X     ISO Abbreviation:  Neurology     Publication Date:  2013 Jan 
Date Detail:
Created Date:  2013-01-29     Completed Date:  2013-03-21     Revised Date:  2014-03-26    
Medline Journal Info:
Nlm Unique ID:  0401060     Medline TA:  Neurology     Country:  United States    
Other Details:
Languages:  eng     Pagination:  496-503     Citation Subset:  AIM; IM    
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
Age of Onset
Basal Ganglia / pathology*
Cerebral Cortex / pathology*
Cognition Disorders / diagnosis,  etiology
Databases, Factual / statistics & numerical data
Family Health
Language Disorders / diagnosis,  etiology
Middle Aged
Neurodegenerative Diseases / classification,  complications,  diagnosis*
Neurologic Examination / methods*,  standards*
Retrospective Studies
Grant Support
K23 AG039414/AG/NIA NIH HHS; P01 AG032953/AG/NIA NIH HHS; R01 AG031278/AG/NIA NIH HHS; R01 AG038791/AG/NIA NIH HHS; R01 NS044266/NS/NINDS NIH HHS

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

Previous Document:  Adjunctive behaviors are operants.
Next Document:  Clinical Reasoning: A girl presenting with stiffness episodes during sleep, cafe-au-lait spots, and ...