Progressive dysphasic dementia with localized cerebral atrophy: report of an autopsy.

A 64-year-old Japanese woman showed an initially aphasic disturbance followed by complete mutism, progressive dementia, parkinsonism and muscular atrophy. Autopsy revealed localized cortical atrophy confined to the pars triangularis, pars opercularis of the inferior frontal gyrus, supramarginal and angular gyri of the inferior parietal lobe, precuneus and posterior half of the middle and inferior temporal gyrus predominantly on the left hemisphere. The right cerebellar hemisphere showed crossed cerebellar atrophy with shrinkage of the right middle cerebellar peduncle. In the atrophied cerebral areas there were diffuse outfall of neuronal cells in all cortical layers and remaining neurons generally showed simple atrophy, and there were a few swollen neurons. Gliosis of the subcortical white matter was confined to the affected gyri and GFAP positive astrocytes were observed in the 1st, 2nd, 5th and 6th layers of the cortex. In addition, the degenerative changes of the substantia nigra, gliosis of the amygdaloid complex and inferior olivary nucleus were bilaterally observed. The distribution and characteristics of the cortical and white matter degeneration are different from those of Pick's disease, and it is likely that this case belongs to a group of so-called degenerative dysphasic dementias.