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Permanent global amnesia with unknown etiology

Neurology Service (Dr. Kritchevsky) and the Psychiatry Service (Dr. Squire), Veterans Affairs Medical Center, San Diego, CA; and the Departments of Neurosciences (Dr. Kritchevsky) and Psychiatry (Dr. Squire), University of California at San Diego, La Jolla, CA.

Three patients developed severe and selective memory impairment with no known cause, one during a period of a few days and two others during a period of 1 to 2 years. In two of these patients, the amnesia has been stable and circumscribed for 5 to 6 years. The third patient appears to have declined in cognitive functions during the past year, at the age of 78, after 6 years of stable, circumscribed amnesia. Neuropsychological testing reveals severe impairment in the ability to learn verbal and nonverbal material as well as retrograde amnesia covering at least 20 years. CT and routine brain MRIs were uninformative. Subsequently, a high-resolution protocol for imaging human hippocampus with MR revealed that the hippocampal formation was markedly reduced in size in all three patients. The pattern of cognitive impairment and the MR findings are similar to the findings in other patients with chronic amnesia due to a known anoxic or ischemic episode, and differ from the findings in amnesic patients with alcoholic Korsakoff s syndrome. We suggest that the amnesia may be due to ischemic damage to medial temporal lobe brain structures important for memory.

Address correspondence and reprint requests to Dr. Mark Kritchevsky, Neurology Service (127), VA Medical Center, 3350 La Jolla Village Drive, San Diego, CA 92161.

Supported by the Medical Research Service of the Veterans Administration, NIMH Grant MH24600, the Office of Naval Research, the McKnight Foundation, and NIA Grant AGO 5131.

Presented in part at the 40th annual meeting of the American Academy of Neurology, Cincinnati, OH, April 1988.

Received March 3, 1992. Accepted for publication in final form July 14, 1992.

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