Table of Contents
Washington University Experience | NEURODEGENERATION | Argyrophilic Grain Disease (AGD) | 1G Case 1 Denouement
The diagnosis made was: - Argyrophilic grain disease (severe, stage 3) and - Subcortical arteriolosclerotic leukoencephalopathy ---- Comment: On the basis of clinical data, the premortem diagnoses of Alzheimer disease and Creutzfeldt-Jakob disease (CJD) were favored. However, Western blot and molecular results from the Prion Surveillance Center ruled out CJD. Similarly, the absence of beta-amyloid plaques and the mild burden of neurofibrillary tangle pathology in the medial temporal lobe was thought consistent with Braak and Braak neurofibrillary tangle stage II (range: 0, I-VI) which excludes a neuropathologic diagnosis of Alzheimer disease by Khachaturian, CERAD, NIA-Reagan Institute and the NIA-AA criteria (A0,B1,C0). Vascular pathology in the form of moderate to severe arteriolosclerosis, with associated subcortical arteriolosclerotic leukoencephalopathy, is an additional feature of this case that is likely to have contributed in part to cognitive and motor dysfunction.
