Pathological heterogeneity of clinically diagnosed corticobasal degeneration

J Neurol Sci. 2003 Dec 15;216(1):127-34. doi: 10.1016/s0022-510x(03)00232-6.


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.

Publication types

  • Case Reports

MeSH terms

  • Alzheimer Disease / pathology*
  • Alzheimer Disease / physiopathology
  • Apraxia, Ideomotor / etiology
  • Apraxia, Ideomotor / pathology
  • Apraxia, Ideomotor / physiopathology
  • Brain / pathology*
  • Brain / physiopathology
  • Brain Diseases / classification
  • Brain Diseases / pathology*
  • Brain Diseases / physiopathology
  • Diagnosis, Differential
  • Diagnostic Errors*
  • Dystonia / etiology
  • Dystonia / pathology
  • Dystonia / physiopathology
  • Humans
  • Male
  • Middle Aged
  • Neurofibrillary Tangles / pathology
  • Parkinsonian Disorders / etiology
  • Parkinsonian Disorders / pathology
  • Parkinsonian Disorders / physiopathology
  • Phenotype
  • Pick Disease of the Brain / pathology*
  • Pick Disease of the Brain / physiopathology
  • Plaque, Amyloid / pathology
  • Predictive Value of Tests