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Washington University Experience | NEURODEGENERATION | Progressive Supranuclear Palsy (PSP) | 8D2 PSP (Case 8) ION Biels 13
Tangles in the ION are argyrophilic in Bielschowsky preparations. (Bielschowsky) ---- Not shown: Multiple sections of frontal, parietal, temporal and occipital neocortices show a relatively well maintained complement of neurons without senile plaques or neurofibrillary tangles by conventional Bielschowsky stain, although a small amount of BAP deposition and a few PHF containing neurons are demonstrated. The underlying white matter shows glial inclusions and in neurites, but not alpha-synuclein containing glial inclusions: The number of senile plaques does not meet the CERAD or Khachaturian criteria for the diagnosis of Alzheimer's disease. ---- :Neuro Final Diagnosis: 1) PSP; 2) Excluded NBIA-1 (previously Hallervorden-Spatz Disease); 3) Senile plaques and neurofibrillary tangles (does not reach diagnostic threshold for Alzheimer’s disease) ---- Neuro Diagnosis Comment: Sections of the basal ganglia demonstrate neuron loss, astrocytosis, pigment deposition (demonstrated to be neuromelanin and an iron containing pigment as shown by Prussian blue histochemical stain), scattered tufted astrocytes and granular spheroids as well as rare NFT, mostly in the putamen. The globus pallidus shows neuron loss, astrocytosis and iron containing pigment. This is an interesting and complex neurodegenerative disease which has features most commonly seen in PSP in the distribution of NFT and in the demonstrated immunohistochemical presence of “tufted” astrocytes, tau+ neurons in the substantial nigra, tau+ coiled bodies and white matter glial inclusions, dentate and olivary tau+ neuronal inclusions. Ballooned neurons, as might be expected in corticobasal degeneration, were found only in the amygdala. I think that the original diagnosis of NBIA is incorrect with only a small amount of pigment and overinterpretation of granular spheroids.
