This report concerns an autopsy case of rapidly progressive @entity2026 and @entity4681 . The @entity1 was a Japanese @entity1 who was 75 years old at the time of @entity204 . The family history did not reveal hereditary burden. She developed language disturbances and difficulty in swallowing at age 74. Neurological examination 1 month after the disease onset revealed @entity5785 without @entity303 and bulbar sign, followed by @entity425 of the four extremities. Neuroradiological examination revealed progressive @entity194 of the anterior part of the left temporal lobe. She died of respiratory difficulty 10 months after the disease onset. Macroscopically, neuropathological examination showed circumscribed @entity194 of the left perisylvian region and, histologically, @entity528 in the cerebral cortex, including the primary motor area, substantia nigra, brain stem motor nuclei, and anterior horns of the spinal cord, in addition to obvious degeneration of the pyramidal tracts and presence of Bunina bodies. Ubiquitin-immunoreactive neuronal inclusions were present in the hippocampal dentate granular cells and frontotemporal cortical layer II neurons. Based on these clinicopathological findings and a review of the literature, we concluded that our case is the first reported case of @entity746 with @entity303 that clinically showed rapidly progressive @entity2026 .

Rapidly progressive @entity2026 and XXXX : a clinical, radiological, and pathological study of an autopsy case with circumscribed @entity194 .

@entity1 :: ('9606', 'Species') :: ['woman', 'patient'] @entity303 :: ('MESH:D003704', 'Disease') :: ['dementia'] @entity5785 :: ('MESH:D001039', 'Disease') :: ['motor aphasia'] @entity425 :: ('MESH:D018908', 'Disease') :: ['muscle weakness'] @entity4681 :: ('MESH:D016472', 'Disease') :: ['motor neuron disease'] @entity2026 :: ('MESH:D001037', 'Disease') :: ['aphasia'] @entity194 :: ('MESH:D001284', 'Disease') :: ['atrophy'] @entity746 :: ('MESH:D000690', 'Disease') :: ['amyotrophic lateral sclerosis'] @entity204 :: ('MESH:D003643', 'Disease') :: ['death'] @entity528 :: ('MESH:D009410', 'Disease') :: ['neuronal loss']
@entity4681 :: (MESH:D016472,Disease) :: ['motor neuron disease']
