Selective loss of the electroretinogram B-wave in a patient with Creutzfeldt-Jakob disease

Creutzfeldt-Jakob disease (CJD) is a neurodegenerative disease characterize d by movement abnormalities and dementia that inevitably progress to death. Familial, infectious, and sporadic forms of the disease are recognized. Th e worldwide incidence of CJD is estimated at 1:1,000,000 per year, and it a ffects middle-aged men and women in roughly equal proportions. The disease is caused by a unique infectious vector, the prion, which is a mutant form of a normally occurring cell surface protein found predominantly in the cen tral nervous system. A significant proportion of patients with CJD will hav e visual disturbances at some point in their illness and may therefore cons ult a neuro-ophthalmologist. The case of a woman in whom the diagnosis of C JD was not known until autopsy is reported. Early in the course of her dise ase, she sought ophthalmic consultation because of vision problems.