Plaque-type dura mater graft-associated Creutzfeldt-Jakob disease: an autopsied case report.
Daisuke Tahara, Daichi Yokoi, Nao Tahara et al.
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Case in brief
A 41-year-old man developed right-hand paraesthesia 27 years after receiving a right parietal dural graft following traumatic subdural haematoma. His illness progressed slowly, with loss of independent walking, cognitive decline, myoclonus and akinetic mutism; EEG never showed periodic sharp-wave complexes. Genetic testing found no prion protein gene mutation, with methionine homozygosity at codon 129. Autopsy showed spongiform change, neuronal loss and astrogliosis, with broadly distributed abnormal prion protein deposits, including plaques and prominent staining in spinal grey matter. Intermediate-type prion protein was detected in brain and cervical cord, but not systemic organs.
What made this case unusual
The combination of a slowly progressive course without periodic sharp-wave complexes and intense abnormal prion protein staining throughout spinal grey matter informed the authors’ suggestion that transmission may have occurred indirectly via a peripheral route rather than directly from the graft to the central nervous system.
Diagnostic / clinical pearl
Dura mater graft-associated CJD may have a slowly progressive presentation without periodic sharp-wave complexes; their absence did not exclude the diagnosis in this case.