Table of Contents
Washington University Experience | NEURODEGENERATION | Lewy Body Disease (LBD) | 4 LBD - Gross Pathology - LBD-Dementia with AD | 4B Case 4 Denouement
Neuro Final Diagnosis: Alzheimer's disease; Dementia with Lewy bodies; TDP-43 neuronal cytoplasmic inclusions (NCI) in the medial temporal lobe ---- Frequent beta-amyloid plaques, neuritic plaques and neocortical neurofibrillary tangles are present, consistent with Braak and Braak neurofibrillary tangle stage VI (range: 0, I-VI) and amyloid stage C (range: 0, A-C) (1). These findings substantiate the diagnoses of: "Alzheimer's disease" (AD) by Khachaturian, "definite AD" by CERAD, and "high probability" by NIA-Reagan criteria. In addition to beta-amyloid and tau pathology, this case also displays alpha-synuclein-positive inclusions in the form of Lewy bodies and Lewy neurites in brain stem, limbic, and neocortical areas. The density and distribution of these lesions is sufficient to meet the criteria for dementia with Lewy bodies (neocortical type). In the staging of Lewy body pathology in Parkinson’s disease according to Braak et al. this case is stage 6 (range: 0,1-6). This case also shows TDP-43 proteinopathy in the medial temporal lobe. TDP-43 proteinopathy has been described as a co-morbidity in other diseases. However, the TDP-43 proteinopathy in this case is very modest and unlikely to have contributed significantly to clinical decline. In conclusion, the cognitive and motor deficits described in this case may be explained by Alzheimer's disease and diffuse Lewy body disease, with very minor contributions from TDP-43 proteinopathy and vasculopathy.
