engleski

Creutzfeldt-Jakob disease in a patient with a lyophilised dura mater graft- case reposrt and review of the literature

A 37-year-old patient with Creutzfeldt-Jakob disease (CJD) is presented, who had received a cadaveric dura mater graft 12 years before the onset of neurologic symptoms. Initial clinical presentation included cerebellar symptoms, with dementia and myoclonus developing in later stages of the disease. EEG showed diffuse slowing with sporadic triphasic periodic activity. CT was normal in the early stage but pronounced cerebral and cerebellar atrophy with widened sulci were seen on MRI in the late stage of the disease. The prion protein (PrP) gene was homozygous for valin at the polymorphic codon 129. Cerebrospinal fluid analysis for 14-3-3 protein was positive. We believe that this patient is the first Croatian to acquire CJD by dural implant. Based on this case and a review of 66 cases from the literature, it is manifest that the awareness of iatrogenig transmission of CJD and adoption of preventive measures are the only effective way to stop the spread of CJD among surgically treated patients.

Creutzfeldt-Jakob disease; cadaveric dura mater graft; prion protein

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