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NEUROLOGY 1990;40:620
© 1990 American Academy of Neurology

Progressive aphasia in a patient with Pick's disease

A neuropsychological, radiologic, and anatomic study

N. R. Graff-Radford, MBBCh, MRCP(UK), A. R. Damasio, MD, PhD, B. T. Hyman, MD, PhD, M. N. Hart, MD, D. Tranel, PhD, H. Damasio, MD, G. W. Van Hoesen, PhD and K. Rezai, MD

Departments of Neurology (Drs. Graff-Radford, A. Damasio, Hyman, Tranel, and H. Damasio), Pathology (Dr. Hart), Anatomy (Dr. Van Hoesen), and Radiology (Dr. Rezai), The University of Iowa College of Medicine, Iowa City, IA.

Although Pick's disease is generally considered as a dementia characterized by signs of frontal lobe dysfunction, it can present with selective language defects rather than with cognitive decline. In this study, we report prospective and serial clinical, neuropsychological, and neuroradiologic observations in a 59-year-old man whose prominent disturbance was in the retrieval and learning of names denoting concrete entities and actions. Postmortem study confirmed the diagnosis of Pick's disease and revealed that neuronal loss and gliosis were most prominent in left anterior temporal cortices. The findings are in keeping with evidence that the left anterior temporal cortices and interconnected hippocampal System are critically involved in the learning and retrieval of verbal lexical items. The evidence from this patient, along with similar evidence from the literature we reviewed, suggests that when patients present with a progressive aphasia characterized by anomia, Pick's disease should be considered as the probable diagnosis.

Address correspondence and reprint requests to Dr. Neill R. Graff-Radford, Department of Neurology, Mayo Clinic, Jacksonville, 4500 San Pablo Drive, Jacksonville, FL 32224.

Supported by Kiwanis International (Illinois-Eastern Iowa District), Spastic Paralysis Research Foundation, and NINCDS grant PO1 NS19632.

Received July 6, 1989. Accepted for publication in final form September 27, 1989.




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