DISAPPEARANCE OF PERIODIC SHARP WAVE COMPLEXES IN CREUTZFELDT-JAKOB-DISEASE

Periodic sharp wave complexes (PSWC) are sensitive and specific of Cre utzfeldt-Jakob disease (CJD). Once they have emerged, PSWC may excepti onally disappear in the terminal stage of the disease, as a consequenc e of the flattening of scalp electroencephalogram (EEG). We document t he disappearance of PSWC in serial EEG during the clinical course in t wo women (57 and 70 years of age) with pathologically proven CJD. Desp ite PSWC disappearance, diffuse theta-delta activity was still well re cognizable. Moreover, external stimuli failed to trigger PSWC. The abs ence of PSWC in CJD might be due to the timing and frequency of EEG re cordings. PSWC disappearance should not be interpretated as evidence a gainst the diagnosis of CJD.