Introduction.Creutzfeldt-Jakob’s disease has various anatomoclinical present ations including a rare form with preponderant visual signs described by Heidenh ain. In this form, the visual symptoms may be isolated for...Introduction.Creutzfeldt-Jakob’s disease has various anatomoclinical present ations including a rare form with preponderant visual signs described by Heidenh ain. In this form, the visual symptoms may be isolated for a few weeks, leading to multiple ophthalmological examinations. Observation. We report the case of a 75-year-old woman who developed isolated visual disorders which rapidly increa sed over a period of two months. Addition of neurological symptoms, abnormalitie s of EEG and positivity of 14-3-3 protein led to the diagnosis of Creutzfeldt -Jakob’s disease. The patient died 14 months after the first neuroophthalmolog ic signs. The diagnosis was established by post-mortem examination and immuno- electrophoretic demonstration of type 1 prion protein. Conclusion. Heidenhain’s form of Creut zfeldt Jakob’s disease highlights the importance of general rules for preventio n of iatrogenic hazard during ophthalmological examinations.展开更多
文摘Introduction.Creutzfeldt-Jakob’s disease has various anatomoclinical present ations including a rare form with preponderant visual signs described by Heidenh ain. In this form, the visual symptoms may be isolated for a few weeks, leading to multiple ophthalmological examinations. Observation. We report the case of a 75-year-old woman who developed isolated visual disorders which rapidly increa sed over a period of two months. Addition of neurological symptoms, abnormalitie s of EEG and positivity of 14-3-3 protein led to the diagnosis of Creutzfeldt -Jakob’s disease. The patient died 14 months after the first neuroophthalmolog ic signs. The diagnosis was established by post-mortem examination and immuno- electrophoretic demonstration of type 1 prion protein. Conclusion. Heidenhain’s form of Creut zfeldt Jakob’s disease highlights the importance of general rules for preventio n of iatrogenic hazard during ophthalmological examinations.