[Heidenhain's variant of Creutzfeldt-Jakob's disease].
BACKGROUND
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.


METHODS
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.


CONCLUSIONS
Heidenhain's form of Creutzfeldt-Jakob's disease highlights the importance of general rules for prevention of iatrogenic hazard during ophthalmological examinations.
Fauquembergue
M
M
Unite de Neuro-ophtalmologie et service de Neurologie D, Hospices Civils de Lyon, Hôpital Neurologique Pierre-Wertheimer, Bron.
Tilikete
C
C
Perret-Liaudet
A
A
Kopp
N
N
Krolak-Salmon
P
P
Vighetto
A
A
D048948:14-3-3 Proteins / Q000378:metabolism; D000368:Aged; D007562:Creutzfeldt-Jakob Syndrome / Q000150:complications* / Q000175:diagnosis / Q000235:genetics; D004569:Electroencephalography; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007122:Immunoelectrophoresis; D008279:Magnetic Resonance Imaging; D009154:Mutation / Q000502:physiology; D014786:Vision Disorders / Q000175:diagnosis / Q000209:etiology* / Q000235:genetics
D048948:14-3-3 Proteins