Diagnosis of Sporadic Creutzfeldt-Jakob Disease in Both Kidney Recipients From the Same Donor: Was It Graft Transmission?

Recognizing rare causes of neuropsychiatric symptoms in transplant recipients can be challenging, as neurological complications occur in many patients and are usually due to infections, comorbidities, or drug toxicity. Once these factors have been ruled out, prion diseases such as Creutzfeldt-Jakob disease (CJD) should be considered. CJD can spread through medical interventions. Current diagnostic criteria for iatrogenic CJD take into account the possibility of transmission through corneal or dural transplants, contaminated neurosurgical tools, as well as administration of cadaveric pituitary hormones. They do not recognize solid organ transplantation as a viable transmission pathway. We present a unique case of concurrent rapidly progressive dementia in both kidney recipients from the same donor, in one of whom the diagnosis of CJD was confirmed. The clinical course, CSF findings, and neuropathology suggested possible prion transmission from their common donor. This case raises awareness of the possibility of transplant-derived prion infections and illustrates the complexity of the diagnostic workup. We advocate for wider adoption of appropriate diagnostic tools.