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NEUROLOGY 1998;51:1180-1182
© 1998 American Academy of Neurology

Kufs' disease presenting as late-onset epilepsia partialis continua

A. Gambardella, MD, G. Pasquinelli, MD, R. Cittadella, PhD, F. Bono, MD, R. L. Oliveri, MD, P. Valentino, MD, M. Zappia, MD, A. Quattrone, MD and U. Aguglia, MD

From the Institute of Neurology (Drs. Gambardella, Bono, Oliveri, Valentino, Zappia, Quattrone, and Aguglia), School of Medicine Catanzaro, Catanzaro; Institute of Experimental Medicine and Biotechnology (Drs. Gambardella, Cittadella, Oliveri, and Quattrone), National Research Council, Piano Lago di Mangone-Cosenza; and Laboratory of Cytopathology (Dr. Pasquinelli), Policlinico S. Orsola Malpighi, Bologna, Italy.

Address correspondence and reprint requests to Dr. Antonio Gambardella, Cattedra ed U.O. di Neurologia, Facoltà di Medicine e Chirurgia, Via Tommaso Campanella, 88100 Catanzaro, Italy.

A 64-year-old woman, who had no personal or family history of neurologic diseases, had an 18-month history of epilepsia partialis continua (EPC) associated with a moderate intellectual deterioration and subtle extrapyramidal rigidity. There was no photosensitive response. A thorough laboratory investigation was unremarkable. A biopsy of the rectal mucosa revealed abundant fingerprint profiles diagnostic of Kufs' disease (KD). Our case expands the clinical picture of KD and suggests that such a diagnosis should be considered in adult-onset EPC.


Received February 5, 1998. Accepted in final form May 29, 1998.




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