A. Kinoshita et al., A CASE OF PRIMARY PROGRESSIVE APHASIA WITH ABNORMALLY UBIQUITINATED NEURITES IN THE CEREBRAL-CORTEX, Acta Neuropathologica, 92(5), 1996, pp. 520-524
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.