Primary progressive aphasia, left anterior atrophy, and neurofibrillary hippocampal pathology : Observations in an unusual case

摘要: A 71-year-old, right-handed woman experienced onset of a slowly progressive, nonfluent language disorder. She maintained normal cognitive abilities until age 80 and developed mild spastic right hemiparesis the following year. By 82, she had become severely demented, mute, akinetic. Postmortem brain examination showed moderate asymmetric atrophy (wt = 1000 g), which was most prominent in left perisylvian posterior frontal regions, with disproportionate enlargement lateral ventricle. Microscopically, high density neurofibrillary tangles present hippocampal pyramidal cell layer entorhinal cortex (up to 45/400 x field, R > L). Gliosis patchy neuronal loss were symmetrically parietal lobes including speech areas, but neuritic plaques only small numbers (0-1/400 field). In this case primary progressive aphasia, pathologic changes associated Alzheimer's disease occurred allocortex not neocortex. These clinical neuropathologic findings expand on those previously reported aphasia suggest need for further study syndrome.