Creutzfeldt-Jakob disease (CJD) is a fatal rare disease which diagnosis is only confirmed by cerebral biopsy or demostrating prion transmissibility. Without these conditions, it is mandatory to rule of alternative diagnostics. Inside differential diagnosis acute herpes encephalitis must be considered, since its clinical presentation may resemble but its prognosis is considerably better. We present a 75 years old female with ataxia, cognitive impairments and seizures who had positive PCR for Herpes I in cerebrospinal fluid and positive Harrington's test too. We considered differential diagnosis between herpes encephalitis and CJD, despite Ig M for Herpes I was negative and neither MR nor electroencephalography showed characteristics of any of these diseases. With no response to antiviral treatment and demonstration of homocigousy for the methionin gen at 129 codon, the most probably diagnostic was CJD. Nevertheless, considering the different prognostic of both diseases brain biopsy was performed. It confirmed neuronal loss and spongiform degeneration with vacuolization with multiple PrP's deposits on immunohistochemistry. There were no signs of herpes encephalitis and Herpes virus PCR on brain biopsy was also negative. These results confirmed the diagnosis of CJD, sporadic variant. The patient died 3 months later.
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