Creutzfeldt-Jacob disease (CJD)

Record number: 
Adverse Occurrence type: 
MPHO Type: 
Estimated frequency: 
Time to detection: 
30 years
Alerting signals, symptoms, evidence of occurrence: 
45 year old female developed cerebellar syndrome, slight slurred speech, truncal ataxia, dysmetria, wide based gait 30 years after cornea transplantations . She deteriorated with severe dysarthria, immobility. dementia, myoclonic jerks, flexion rigidity, coma and died 8 months after onset of symptoms. No autopsy. She had corneal transplants in 1965 and 1982. The first donor , a 63 year old female, had incoordination, memory loss, involuntary movements, myoclonic jerks and died one month later. An autopsy showed spongiform encephalopathy and CJD.
Demonstration of imputability or root cause: 
Probable transmission. The patient had typical CJD but had no autopsy to confirm the diagnosis. The donor had proven CJD 30 years earlier, a long incubation period. Others had incubation periods of up to 25 years after dura transplants but usually CJD from cornea has a shorter incubation period
Imputability grade: 
2 Probable
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