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Washington University Experience | NEURODEGENERATION | Progressive Supranuclear Palsy (PSP) | 17C2 Progressive Supranuclear Palsy (PSP) (Case 17) N6 SN Biels 60X 1
A prominent neuronal tangle within a neuron of the substantia nigra. (Bielschowsky) ---- Not shown: The neocortices show a generally well-maintained complement of neurons without concomitant gliosis. No cortical Lewy bodies are detected. A Bielschowsky silver stain highlights only very rare neocortical neurofibrillary tangles, particularly within the entorhinal cortex as well as cortical plaques, predominantly diffuse in nature. Sections from the hippocampus show a generally well-maintained complement of pyramidal neurons. Only scattered neurofibrillary tangles and occasional plaques (mostly diffuse>>neuritic) are seen within the CA-1 sector and more prominently within the entorhinal cortex with Bielschowsky stains. Pick bodies are not detected. Thick-walled arterioles, some with calcification, are seen within the basal ganglia as is a resolving microinfarct with tissue collapse and gliosis. ---- Neuro Final Diagnosis: Progressive supranuclear palsy ---- Neuro Diagnosis Comment: The presence of globose neurofibrillary tangles within many of the subcortical and brainstem nuclei, together with this patient's clinical presentation are indicative of progressive supranuclear palsy. The absence of Lewy bodies, both cortical and brainstem, negates the possibility of idiopathic Parkinson's disease and diffuse Lewy body disease. Likewise the general absence of neocortical and hippocampal neuritic plaques with only occasional neurofibrillary tangles rules out Alzheimer's disease. It is likely that the occasional neocortical neurofibrillary tangles that are detected are part of the spectrum of progressive supranuclear palsy.
