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From: TSS ()
In Reply to: Abnormal PrP in the retina of the most commonly subtype sCJD posted by TSS on August 20, 2005 at 6:48 am:
[Article in French] Fauquembergue M, Tilikete C, Perret-Liaudet A, Kopp N, Krolak-Salmon P, Vighetto A. Unite de Neuro-ophtalmologie et service de Neurologie D, Hospices Civils de Lyon, Hopital Neurologique Pierre-Wertheimer, Bron. INTRODUCTION: Creutzfeldt-Jakob's disease has various anatomoclinical presentations including a rare form with preponderant visual signs described by Heidenhain. 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 increased over a period of two months. Addition of neurological symptoms, abnormalities 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 neuroophthalmologic signs. The diagnosis was established by post-mortem examination and immuno-electrophoretic demonstration of type 1 prion protein. CONCLUSION: Heidenhain's form of Creutzfeldt-Jakob's disease highlights the importance of general rules for prevention of iatrogenic hazard during ophthalmological examinations. PMID: 16106810 [PubMed - in process]
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