Selective Loss of the Electroretinogram B-wave in a Patient With Creutzfeldt-Jakob Disease

• Published in: Journal of neuro-ophthalmology Vol. 20; no. 2; pp. 116 - 118
• Main Authors: Katz, Bradley J, Warner, Judith E. A, Digre, Kathleen B, Creel, Donnell J
• Format: Journal Article
• Language: English
• Published: Hagerstown, MD Lippincott Williams & Wilkins, Inc 01.06.2000; Lippincott Williams & Wilkins
• Subjects: Adult; Biological and medical sciences; Brain - pathology; Brain Chemistry; Creutzfeldt-Jakob Syndrome - diagnosis; Creutzfeldt-Jakob Syndrome - physiopathology; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Electroretinography; Female; Humans; Magnetic Resonance Imaging; Medical sciences; Neurology; Prions - analysis; Retina - physiopathology; Retinal Diseases - diagnosis; Retinal Diseases - physiopathology; Vision Disorders - diagnosis; Vision Disorders - physiopathology; Electroretinography; Human; Nervous system diseases; B wave; Cerebral disorder; Infection; Case study; Eye; Electrodiagnosis; Central nervous system disease; Complication; Female; Degenerative disease; Prion; Diagnosis; Creutzfeldt Jakob disease
• ISSN: 1070-8022; 1536-5166
• DOI: 10.1097/00041327-200020020-00011

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.