SUBCORTICAL NEUROFIBRILLARY TANGLES, NEUROPIL THREADS, AND ARGENTOPHILIC GLIAL INCLUSIONS IN CORTICOBASAL DEGENERATION

This report concerns an autopsy case of corticobasal degeneration (CBD ). The patient was a 64-year-old woman with slowly progressing clumsin ess and gait disturbance who died approximately 7 years after the onse t of symptoms. The salient neuropathological findings were focal loss of neurons of the cerebral cortex, ballooned neurons and gliosis of th e subcortical white matter, thalamus, and basal ganglia. In addition, there were marked neuronal loss and gliosis in the substantia nigra. T he ballooned neurons were positively immunostained for phosphorylated neurofilament, alpha B-crystallin and synaptophysin; staining for the latter was intraneuronal. The examination of silver impregnated and Ta u immunostained preparations revealed numerous basal neurofibrillary t angles, neuropil threads, and glial inclusions; their presence was mos t pronounced in the brainstem tegmentum, basal ganglia, and thalamus. Neurofibrillary tangles consisting of straight tubules with a diameter of approximately 13 - 15 nm and constricted tubules were identified i n the substantia nigra. The results of the present study indicate that the cytopathology of the subcortical gray matter and brainstem in CBD patients resembles that of progressive supranuclear palsy.