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

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