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Washington University Experience | NEURODEGENERATION | Multiple Systems Atrophy (MSA) | 3B6 MSA (Case 3) Pons aSYN 5

The pontine nuclei show severe and dramatic alpha-synuclein pathology consisting of numerous neuronal cytoplasmic inclusions (NCIs) and neurites in the pontine nuclei accompanied by descending and transverse GCI. (pSYN IHC) ---- Not shown: There are no diffuse beta-amyloid plaques and no neurofibrillary tangles. No cortical or brainstem Lewy bodies are seen. There is minimal neuronal loss from the striatum and pallidum and some mild gliosis. There is severe loss of neurons, gliosis and extracellular pigment in the substantia nigra. Numerous GCIs are present in the red nucleus and crus cerebri. There is evidence of neuronal loss in the medulla, including the inferior olivary nucleus. There is white matter pallor, axon loss, and striking loss of Purkinje cells, gliosis, and multiple GCI, mainly in the white matter. ---- Neuro Final Diagnosis Multiple system atrophy (alpha-synucleinopathy). As parkinsonian features predominated, according to the Gilman et al. criteria (PMID: 10223419), this case may be designated MSA-P.

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