Pathological heterogeneity of clinically diagnosed corticobasal degeneration.
Two patients fulfilling suggested clinical diagnostic criteria for corticobasal degeneration (CBD) are presented, who were found at postmortem to have alternative pathological diagnoses not suspected during life, namely, Alzheimer's disease and Pick's disease, respectively. The nosological position of these cases is considered in light of a literature review of previous reports of clinically diagnosed corticobasal degeneration with atypical (not corticobasal degeneration) pathology. Since such phenocopies may be common, we suggest that all clinically diagnosed cases of corticobasal degeneration should initially be labelled as "corticobasal degeneration syndrome" (CBDS) to emphasize that this is a diagnosis based on clinical phenotype, with the term corticobasal degeneration being reserved for the specific neuropathological phenotype, which itself may have a variety of clinical presentations.
Doran
M
M
Cognitive Function Clinic, Walton Centre for Neurology and Neurosurgery, Lower Lane, Fazakerley, L9 7LJ, Liverpool, UK. larner-a@wcnn-tr.nwest.nhs.uk
du Plessis
D G
DG
Enevoldson
T P
TP
Fletcher
N A
NA
Ghadiali
E
E
Larner
A J
AJ
D000544:Alzheimer Disease / Q000473:pathology* / Q000503:physiopathology; D020240:Apraxia, Ideomotor / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D001921:Brain / Q000473:pathology* / Q000503:physiopathology; D001927:Brain Diseases / Q000145:classification / Q000473:pathology* / Q000503:physiopathology; D003937:Diagnosis, Differential; D003951:Diagnostic Errors; D004421:Dystonia / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D016874:Neurofibrillary Tangles / Q000473:pathology; D020734:Parkinsonian Disorders / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D010641:Phenotype; D020774:Pick Disease of the Brain / Q000473:pathology* / Q000503:physiopathology; D058225:Plaque, Amyloid / Q000473:pathology; D011237:Predictive Value of Tests