Creutzfeldt-Jacob disease (CJD)

Record number: 
Adverse Occurrence type: 
MPHO Type: 
Time to detection: 
15 months – 30 years
Alerting signals, symptoms, evidence of occurrence: 
First symptoms are the development of lethargy, nausea and ataxia 15 months – 30 years after corneal transplant. Early neurologic manifestations are difficulty swallowing, akinetic mutism, and difficulty walking. Neurologic deterioration with bilateral myoclonic jerks, ataxia, dysarthria, hyperactive deep tendon reflexes, cerebellar syndrome, progressive dementia, unresponsiveness, coma, and death. Cerebrospinal fluid analysis showing a very high neuron specific enolase concentration and abnormal MRI and EEG studies may suggest the diagnosis of CJD.
Demonstration of imputability or root cause: 
Autopsy confirmation of CJD in both donor and recipient. The presence of prion protein PrPSc in corneal tissue or the optic nerve.