Jakob-Creutzfeldt disease. Modification of clinical and electroencephalographic activity with methylphenidate and diazepam.

The electroencephalogram in three patients with Jakob-Creutzfeldt disease showed two separate abnormalities-namely, progressive background suppression and periodic generalized synchronous triphasic sharp wave complexes which evolve to a uniform morphology and periodicity. The abnormalities, when found in the EEG of a patient in middle-age with a dementing illness, should not be confused with other periodic electroencephalographic phenomena. Since the neuropathological abnormalities of Jakob-Creutzfeldt disease are non-specific, the electroencephalogram is essential for the recognition of this disorder, although serial recordings may be necessary to establish the diagnosis. Modification of the electroencephalographic abnormalities occurs with afferent stimuli and with methylphenidate or diazepam, suggesting that the phenomenon of background suppression is independent of the presence of the periodic complexes. Modification of clinical activity with methylphenidate suggests that some degree of reversibility of function exists in this inexorably fatal disorder. Further detailed studies of the electroencephalogram in cases of Jakob-Creutzfeldt disease are indicated.