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Creutzfeldt-Jakob disease without periodic sharp wave complexes

A clinical, electroencephalographic, and pathologic study

From the Department of Clinical Neurological Sciences, the University of Western Ontario, London, Ontario, Canada. Dr. D. Zochodne has been grateful for financial support from the Medical Research Council of Canada.

A comparison of clinical, EEG, and pathologic features was carried out on all cases of autopsy-proven Creutzfeldt-Jakob disease (CJD) studied over the last 10 years. Periodic sharp wave complexes (PSWCs) were present in three but absent in seven patients. Myoclonus was documented in two of the three with and in only one of the seven without PSWCs. The three with PSWCs had severe neocortical and at least mild thalamic involvement; those without PSWCs had more variable disease topography. The diagnosis of CJD should not be rejected if PSWCs are absent.

Address correspondence and reprint requests to Dr. Young, Department of Clinical Neurological Sciences, Victoria Hospital, 375 South Street, London, Ontario N6A 4G5, Canada.

Received April 29,1987. Accepted for publication in final form November 24, 1987.