[Neuropathological features in corticobasal degeneration and progressive supranuclear palsy]

Abstract

Corticobasal degeneration (CBD) is a sporadic neurodegenerative disorder of late life with a range of clinical presentations such as progressive asymmetrical rigidity and apraxia, progressive aphasia or dementia. Focal cortical atrophy, ballooned neurons and degeneration of the substantia nigra and globus pallidus have been emphasized in previous descriptions. Recent immunohistochemical studies revealed that tau-positive neuronal and glial lesions in both gray and white matter, especially astrocytic plaques in the affected cerebral cortex, are the characteristic features in CBD. While cortical involvement is also recognized in progressive supranuclear palsy, ballooned neurons are sparse and limited to the paralimbic areas and tufted astrocytes are abundant in the precentral gyrus and striatum. From a neuropathological viewpoint, CBD is distinct from other sporadic tauopathies.