A CASE OF PRIMARY PROGRESSIVE APHASIA WITH ABNORMALLY UBIQUITINATED NEURITES IN THE CEREBRAL-CORTEX

We report the histopathological and immunohistochemical findings in a patient with primary progressive aphasia and abnormally ubiquitinated neurites in the cerebral cortex. Neuropathological examination showed severe neuronal loss and astrocytosis with a spongy change in the fron tal cortex and neostriatum. Immunohistochemistry for ubiquitin antibod y showed many immunoreactive dystrophic neurites in the superficial la yer of the affected cortices and putamen. Those neurites were neither argentophilic nor stained with other antibodies against neurofilament, tau, or microtubule-associated protein-2. There were no neuropatholog ical changes characteristic of Alzheimer's disease, Pick's disease, or Creutzfeldt-Jakob disease. Immunoelectron microscopy using anti-ubiqu itin antibody showed inclusions in the dendrites, consisting mainly of granular and filamentous material. These pathological features, unusu al in primary progressive aphasia, indicate the neuropathological hete rogeneity of this disease condition.