Abstract:
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.
