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Washington University Experience | NEURODEGENERATION | Hippocampal Sclerosis of Aging | 3D Case 3 Denouement
Neuro Final Diagnosis: Frontotemporal lobar degeneration with TDP-43 proteinopathy; Hippocampal sclerosis; Alzheimer’s disease changes (mild); Arteriolosclerosis (mild) ---- Neuropathology Diagnosis Comment:
The main neuropathological finding of this case is frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP). The pattern of neuronal loss and the distribution of neuronal cytoplasmic phosphorylated TDP-positive inclusions and dystrophic neurites indicate that this TDP-43 proteinopathy likely contributed to dementia and behavioral changes in this participant. This case also shows hippocampal atrophy with marked neuronal loss and gliosis in the subiculum and hippocampal subfield CA1. TDP-43- immunoreactive NCIs in the dentate gyrus and amygdala have been reported in the context of some cases of hippocampal sclerosis independent of FTLD. In this case both FTLD and HS may both contribute to this TDP-43 proteinopathy. There was minimal ADNC using the NIA-AA criteria (A1B1C0). Vascular pathology in the forms of mild arteriolosclerosis and mild atherosclerosis were also observed but no infarcts were seen. The vascular encephalopathy may have contributed to the patient’s cognitive deficits. In conclusion, the cognitive deficits experienced during life may be explained by FTLD with TDP-43 proteinopathy, with a modest contribution from vascular disease.
