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Washington University Experience | NEURODEGENERATION | NBIA1 (PKAN, Hallervorden-Spatz Dz) | 3G3 Hallervorden-Spatz Dz (Case 3) L9 SN UBIQ 40X 2
Axonal spheroids show prominent ubiquitin immunoreactivity as do adjacent neurons (Ubiquitin IHC) ---- Comment: Microscopic examination of representative neocortical areas showed numerous beta-amyloid plaques, neuritic plaques, and neurofibrillary tangles, consistent with stage V in the Braak and Braak staging of tangles and amyloid stage C. These findings are sufficient to meet the neuropathological criteria for the diagnosis of Alzheimer's disease (AD) according to the criteria of Khachaturian, CERAD, and the NIA-Reagan Institute. No Lewy bodies were identified by H&E or alpha-synuclein immunohistochemistry excluding Parkinson disease dementia. The cognitive changes are explained by AD and the parkinsonian symptoms likely reflect neurodegeneration with brain iron accumulation.
