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NEUROLOGY 1991;41:882
© 1991 American Academy of Neurology

Primary progressive aphasia with focal neuronal achromasia

C. F. Lippa, MD, R. Cohen, PhD, T. W. Smith, MD and D. A. Drachman, MD

Departments of Neurology (Drs. Lippa, Cohen, Smith, and Drachman), and Neuropathology (Drs. Lippa and Smith), University of Massachusetts Medical Center, Worcester, MA.

We describe the clinical, radiologic, neuropsychological, and neuropathologic features of a 69-year-old man with a 3-year history of progressive transcortical expressive aphasia. Neuropsychological testing showed progressive dysfunction of expressive language. Neuropathologic examination demonstrated focal cortical degeneration involving the left superior frontal gyrus, with swollen achromasic neurons and no evidence of Alzheimer's disease, Pick's disease, Creutzfeldt-Jakob disease, Lewy-body disease, or other dementing disorders. This case adds to the known heterogeneity of the underlying pathology of patients with primary progressive aphasia.

Address correspondence and reprint requests to Dr. Carol F. Lippa, Department of Neurology, University of Massachusetts Medical Center, 55 Lake Avenue North, Worcester, MA 01655.

Received September 28, 1990. Accepted for publication in final form November 26, 1990.




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