A case of dura mater graft-associated Creutzfeldt-Jakob disease in South Africa

Toovey, S. ; Britz, M. ; Hewlett, R. H. (2006-07)

The original publication is available at http://www.samj.org.za


We report Africa’s first known graft-associated case of Creutzfeldt-Jakob disease (CJD), and compare it with other published cases. The patient presented 11 years postoperatively with ataxia, dysarthria, and cognitive impairment. Magnetic resonance imaging (MRI) (without diffusion weighting) and early electroencephalogram (EEG) were nonspecific, but triphasic waves appeared later, when cerebrospinal fluid (CSF) was positive for protein 14-3-3. Periodic synchronous discharges (PSDs) appeared 14 weeks before death. Delayed PSD may be a grave sign that forebodes death. CJD belongs to a group of neurodegenerative disorders known as prion diseases. In these conditions an abnormal isoform of the prion protein (PrP) may develop: (i) sporadically; (ii) through genetic mutations in the prion protein gene (familial CJD); or (iii) through infection, as in new-variant and iatrogenic CJD. Cadaver-derived human dura mater grafts from infected but asymptomatic donors can cause CJD in the implanted host.

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