Table of Contents
Washington University Experience | NEURODEGENERATION | Corticobasal Degeneration (CBD) | 6H Case 6 Denouement
Neuro Diagnosis Comment This case displays three types of molecular pathology: tauopathy; beta-amyloidosis, and TDP-43 proteinopathy. Assessed together, they support several diagnoses. First, histological slides show the stereotypic features of corticobasal degeneration (CBD) including widespread neuronal loss, gliosis, tau-positive neuronal and glial cytoplasmic inclusions in cortical and subcortical nuclei. Second, representative neocortical areas have sparse to focally moderate beta-amyloid plaques, but no neuritic plaques and only sparse neurofibrillary tangles, consistent with stage I of a seven-stage scale (range:0, I-VI) in the Braak and Braak staging of tangles and amyloid stage A (range: 0, A-C). These findings are sufficient to meet the neuropathological criteria for the diagnosis of Alzheimer's disease (AD) according to the criteria of Khachaturian but not the by CERAD, NIA-Reagan Institute and NIA-AA criteria (A1,B1,C0). criteria. There are also some NCIs in the hippocampal dentate gyrus. These features are consistent with a diagnosis of hippocampal sclerosis In addition to the molecular pathologies described above, this case has changes consistent with a the entity called TDP-43 proteinopathy.
