[A clinicopathological case of progressive aphasia]

We report the case of a 59-year old right-handed woman presenting with progressive aphasia without any other neurological deficit and characterized by anomia, agramatism and auditory comprehension difficulties. CT scan showed no abnormalities. Four years later, aphasia was complete but neurological examination was normal. CT scan disclosed a mild cortico-subcortical cerebral atrophy with slight widening of both sylvian fissures. The results obtained with the Wisconsin Card Sorting Test and the Raven's Matrices showed only minor deficits. The IQ (WAIS) was 90. During the following 6 years the patient remained fully self-sufficient and carried out her homework normally. At that time MRI showed progression of cerebral atrophy more pronounced on the left side. Besides a rapid deterioration, twelve months later she developed severe dementia and died 13 years after the onset of the illness. Brain examination disclosed a severe atrophy (brain weight: 880 g) prominent in both frontal lobes and in the anterior perisylvian structures, more pronounced on the left side. There were no vascular lesions. Microscopy revealed widespread neuronal loss and astrocytic fibrillary gliosis confined to cortical areas and vacuolation in the superficial layers. Neurofibrillary tangles and neuritic plaques were found in the most atrophic areas but not in sufficient number to fulfill the histological criteria for Alzheimer's disease. There were no neuropathological changes of Pick's disease or subcortical degeneration. Previous microscopical studies of primary progressive aphasia showed non specific, mostly lobar atrophy similar to that observed in our case, although Alzheimer, Pick and Creutzfeldt-Jakob diseases have been reported. This neuropathological heterogeneity confirms that progressive aphasia is a non-specific language disorder mostly observed in lobar forms of brain degeneration.