CORTICOBASAL DEGENERATION WITH PRIMARY PROGRESSIVE APHASIA AND ACCENTUATED CORTICAL LESION IN SUPERIOR TEMPORAL GYRUS - CASE-REPORT AND REVIEW

A 57-year-old woman showed progressive sensory aphasia as an initial s ymptom, and then developed total aphasia within 6 years and, finally s evere dementia. Neuropathologically, the cerebral cortex was most seve rely affected in the superior and transverse temporal gyri, and subseq uently in the inferior frontal gyrus, especially in the pars opercular is. The degeneration in the subcortical grey matter was most severe in the substantia nigra, and it was moderate to mild in the ventral part of thalamus, globus pallidus and striatum. Cytopathologically, in add ition to achromatic ballooned neurons, massive tau-positive types of c ytosekeletal abnormalities were observed both in neurons and glia, mai nly in the degenerating region. This cytoskeletal pathology coincided with that reported in corticobasal degeneration (CBD). On Bodian stain ing, only a few neurofibrillary tangles were found in the entorhinal p re-alpha layer and substantia nigra. Pick's bodies and senile plaques could not be found. This case is thought to represent a type of CBD, b ut with its cortical lesion focus located in the speech area instead o f the frontoparietal region. A survey of 28 pathologically evaluated c ases of CBD revealed two similar cases, both of which began with progr essive aphasia and presented cortical degeneration in the superior tem poral gyrus. An overview of CBD cases clarified the features in anothe r soup of cases, in which the cerebral accentuated focus was shifted f orward from the central region, clinically resembling Pick's disease. The clinical manifestations in CBD seem to be the expression of these diverse cortical lesions. Primary progressive aphasia may include case s of CBD with involvement of the language center.