Rapidly progressive aphasia and motor neuron disease: a clinical, radiological, and pathological study of an autopsy case with circumscribed lobar atrophy

This report concerns an autopsy case of rapidly progressive aphasia and mot or neuron disease. The patient was a Japanese woman who was 75 years old at the time of death. The family history did not reveal hereditary burden. Sh e developed language disturbances and difficulty in swallowing at age 74. N eurological examination 1 month after the disease onset revealed motor apha sia without dementia and bulbar sign, followed by muscle weakness of the fo ur extremities. Neuroradiological examination revealed progressive atrophy of the anterior part of the left temporal lobe. She died of respiratory dif ficulty 10 months after the disease onset. Macroscopically, neuropathologic al examination showed circumscribed atrophy of the left perisylvian region and, histologically, neuronal loss in the cerebral cortex, including the pr imary motor area, substantia nigra, brain stem motor nuclei, and anterior h orns of the spinal cord, in addition to obvious degeneration of the pyramid al tracts and presence of Bunina bodies. Ubiquitin-immunoreactive neuronal inclusions were present in the hippocampal dentate granular cells and front otemporal cortical layer II neurons. Based on these clinicopathological fin dings and a review of the literature, we concluded that our case is the fir st reported case of amyotrophic lateral sclerosis with dementia that clinic ally showed rapidly progressive aphasia.