[An autopsied case of multiple system atrophy with remarkable cerebral atrophy]

In this paper, we report the pathological findings of a multiple system atrophy (MSA) patient with remarkable frontal lobe atrophy. The patient was a 65-year-old woman with a 13-year history of untreatable parkinsonism, dysautonomia and progressive motor aphasia. The cranial imaging study disclosed progressive atrophy of the frontal lobe, striatum and pontocerebellar system. She died of pneumonia. The brain weight was 810 g, and there was remarkable atrophy of the cerebrum predominantly in the frontal lobe, striatum, pons and cerebellum. The microscopic examination revealed a preserved cortical structure with laminar gliosis in the sixth layer of the precentral and superior frontal gyri of the frontal lobe, and postcentral gyrus and inferior parietal lobule of the parietal lobe. The cortices of these regions also revealed to be in a spongy state in the second layer, and mild cell loss was seen in the fifth and six layers. The frontal lobe white matter showed a slight pallor, a mild loss of myelinated fiber and axon, and mild gliosis. Glial cytoplasmic inclusions (GCIs) were abundantly observed in the deep layer of the cortex in the regions mentioned above, and were more abundant in the white matter of the frontal and parietal lobes, callosal body, and internal, external and extreme capsules. There were severe degenerations in the olivopontocerebellar and striatonigral systems, and GCIs in widespread regions of the brain. We could not detect any Pick bodies, Lewy bodies, ballooned neurons, senile plaques, or any significant amounts of neurofibrillary tangles. There were no vascular changes. Thus, this was a verified MSA patient with progressive aphasia and remarkable frontal lobe atrophy. We indicate a possible involvement of the cerebral lobes in MSA.