Table of Contents
Washington University Experience | NEURODEGENERATION | Hippocampal Sclerosis of Aging | 4D Case 4 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). 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 "dementia with hippocampus sclerosis" (DHS), which accounts for 0.5-2% of dementia cases. There were a few TDP-43-immunoreactive inclusions in the dentate 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 were relatively modest and unlikely to have had a significant contribution to the cognitive deficits. Although DHS clinically presents as FTLD in most cases, dementia of the AD-type is also found in a substantial portion of cases with hippocampal sclerosis as demonstrated in this participant. Together with the neurodegenerative changes described above, there is small vessel disease (arteriolosclerosis) with small infarcts in the striatum and occipital lobe. In conclusion, the Participant’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.
