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NEUROLOGY 1994;44:1270
© 1994 American Academy of Neurology

Paraneoplastic temporal lobe epilepsy with testicular neoplasm and atypical amnesia

G. L. Ahern, MD, PhD, M. O'Connor, PhD, J. Dalmau, MD, A. Coleman, MD, J. B. Posner, MD, D. L. Schomer, MD, A. G. Herzog, MD, D. A. Kolb, MD and M. M. Mesulam, MD

Behavioral Neurology Unit (Dr. Ahern), University of Arizona Health Sciences Center, Tucson, AZ; the Department of Neurology (Drs. O'Connor, Coleman, Schomer, Herzog, and Mesulam), Beth Israel Hospital, Boston, MA; the Department of Neurology (Drs. Dalmau and Posner), Memorial Sloan-Kettering Cancer Center, New York, NY; and Beverly Hospital (Dr. Kolb), Beverly, MA.

We report a patient who, at age 38, presented with temporal lobe seizures and an atypical memory disorder 3 years before the discovery of a testicular tumor. Detailed neuropsychological testing revealed a relatively isolated amnestic syndrome. The amnesia was atypical as the patient could retain information for hour-long periods, only to lose it later. Serologic studies revealed the presence of a circulating autoantibody that demonstrated an unusual affinity for the nucleolus of cerebral cortical neurons. Western blot analysis of cortical neurons revealed that this antibody reacted with proteins different from other previously identified paraneoplastic neurologic antigens (Hu, Yo, Ri). We believe this to be a case of paraneoplastic limbic encephalitis secondary to the testicular tumor. This patient presents unique characteristics with respect to the mode of presentation, features of the amnesia, and the presence of a circulating antibody with an unusual reactivity pattern.

Address correspondence and reprint requests to Dr. Geoffrey L. Ahern, Behavioral Neurology Unit, Department of Neurology, University of Arizona Health Sciences Center, 1501 North Campbell Avenue, Tucson, AZ 85724.

Received June 7, 1993. Accepted in final form December 13, 1993.




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