title: Selective loss of the electroretinogram B-wave in a patient with Creutzfeldt-Jakob disease.
abstract: Creutzfeldt-Jakob disease (CJD) is a neurodegenerative disease characterized by movement abnormalities and dementia that inevitably progress to death. Familial, infectious, and sporadic forms of the disease are recognized. The worldwide incidence of CJD is estimated at 1:1,000,000 per year, and it affects 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 central nervous system. A significant proportion of patients with CJD will have visual disturbances at some point in their illness and may therefore consult a neuro-ophthalmologist. The case of a woman in whom the diagnosis of CJD was not known until autopsy is reported. Early in the course of her disease, she sought ophthalmic consultation because of vision problems.
authors.affiliation: Department of Ophthalmology, University of Utah Health Sciences Center, Salt Lake City, USA.
authors.forename: B J
authors.initials: BJ
authors.lastname: Katz
authors.forename: J E
authors.initials: JE
authors.lastname: Warner
authors.forename: K B
authors.initials: KB
authors.lastname: Digre
authors.forename: D J
authors.initials: DJ
authors.lastname: Creel
mesh_terms: D000328:Adult; D001921:Brain / Q000473:pathology; D001923:Brain Chemistry; D007562:Creutzfeldt-Jakob Syndrome / Q000175:diagnosis* / Q000503:physiopathology; D004596:Electroretinography; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D011328:Prions / Q000032:analysis; D012160:Retina / Q000503:physiopathology; D012164:Retinal Diseases / Q000175:diagnosis* / Q000503:physiopathology; D014786:Vision Disorders / Q000175:diagnosis* / Q000503:physiopathology
chemical_list: D011328:Prions