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Pathologic findings in a case of primary progressive aphasia

Departments of Neurology (Dr. Scheltens) and Neuro-Pathology (Dr. Kamphorst), Free University Hospital, Amsterdam; and the Netherlands Brain Bank (Dr. Ravid), Netherlands Institute for Brain Research, Amsterdam, The Netherlands.

We report the histopathologic and immunohistochemical findings of a patient who suffered from primary progressive aphasia for 13 years. During the course of his illness, he was diagnosed as having Pick's disease, based on gradually progressive mild personality changes and MRI findings of severe bilateral temporal lobe atrophy. There was severe neuronal loss in the temporal gyri, intense gliosis throughout the cortex, and mild gliosis of the temporal white matter, without any changes typical for Alzheimer's disease or Pick's disease. Using the antibody Alz-50, we found many Alz-50-positive neurons that exhibited a granular or diffuse cytoplasmic stain without fibrous structures in the temporal and parietal cortex, but no ubiquitin or ß (A4) protein-reactive cells, nor spongiform changes. Staining for Alz-50 and ubiquitin did not reveal the presence of Pick bodies or Lewy bodies. We consider this case to be an example of nonspecific cortical degeneration. Our findings stress the need for histopathologic verification of the primary progressive aphasia syndrome.

Address correspondence and reprint requests to Dr. Philip Scheltens, Department of Neurology, Free University Hospital, PO Box 7057, 1007 MB Amsterdam, The Netherlands.

Received April 13, 1993. Accepted for publication in final form July 21,1993.

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