Table of Contents
Washington University Experience | NEURODEGENERATION | Argyrophilic Grain Disease (AGD) | 4B Case Denouement
Neuro Final Diagnosis: Hippocampal sclerosis, severe; Argyrophilic grain disease; Arteriolosclerosis with infarcts in striatum, thalamus and occipital lobe ---- Neuro Diagnosis Comment ---- Microscopic examination of representative neocortical areas show no beta-amyloid plaques, no neuritic plaques, and relatively few neurofibrillary tangles, consistent with stage I of a six-stage scale (range: 0, I-VI) in the Braak and Braak staging of tangles and amyloid stage 0 (range: 0, A-C). The absence of beta-amyloid plaques excludes a neuropathological diagnosis of Alzheimer's disease. The presence of tau-positive inclusions involving both neurons, as punctate grains, and glial cells (astrocytes and coiled bodies in oligodendrocytes), in amygdala and entorhinal cortex support the diagnosis of argyrophilic grain disease (AGD), stage II (range: 0, I-III), which accounts for ~5% of all dementia cases. It typically presents as a late-onset dementia and may account for ~12% of demented individuals older than 65. The presence of hippocampal sclerosis is consistent with a rare entity "dementia with hippocampus sclerosis" (DHS), which accounts for 0.5-2% of dementia cases. No TDP-43-immunoreactive inclusions were seen in this case. In conclusion, the patient’s cognitive deficits can be explained by hippocampal sclerosis and argyrophilic grain disease. Small vessel disease and small infarcts probably were relatively modest and unlikely to have had a significant contribution to the cognitive deficits.
