A patient with histopathologically verified sporadic Creutzfeldt-Jakob
disease (CJD) presented initially with diplopia, sleep disturbances,
and L-dopa-responsive parkinsonism. After more than a year of slow pro
gression, he did not become demented, and failed to fulfill the clinic
al criteria for possible CJD. No clinical examinations currently propo
sed to detect CJD showed the disease. CJD should be in the differentia
l diagnosis of ''parkinson plus'' syndromes until a different etiology
has been found or a histopathologic examination performed.