A48-06 Rapidly Progressive Dementia in a Lung Transplant Recipient: A Case of Sporadic Creutzfeldt-Jakob Disease

Introduction Altered mental status in lung transplant recipients is commonly attributed to infections, metabolic disturbances, or medication side effects. Sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive and uniformly fatal prion disease, is rarely considered in this population. Although cases have been reported in kidney and liver transplant recipients, sCJD remains exceedingly uncommon among lung transplant patients. Case Presentation A 69-year-old woman with COPD underwent bilateral lung transplantation in 2018 and was maintained on tacrolimus, mycophenolate, prednisone, and belatacept. She developed several months of progressive short-term memory loss and subtle behavioral changes, followed by a two-week period of worsening confusion, blank staring spells, and left-sided weakness. Head CT and chest imaging were unremarkable. EEG demonstrated frequent right frontotemporal lateralized periodic discharges. Brain MRI showed multifocal cortical ribboning with restricted diffusion, most pronounced in the right hemisphere. Empiric treatment with levetiracetam and valproate did not improve symptoms. Neurologic examination revealed increased tone, hyperreflexia, and minimal verbal output. Repeat MRI confirmed persistent right-predominant cortical abnormalities. CSF analysis demonstrated markedly elevated total tau (4381 pg/mL), 14-3-3 protein (25,550 AU/mL), and a positive RT-QuIC assay, establishing the diagnosis of sCJD. Infectious, metabolic, and autoimmune studies were negative. Despite brief periods of lucidity, she progressed to akinetic mutism, and care was transitioned to comfort measures. Discussion This case highlights a rare but important diagnostic consideration in transplant recipients presenting with neurologic decline. While metabolic derangements, infections, and medication-related causes are more frequent, characteristic MRI and EEG findings should prompt testing for prion disease. Early identification of sCJD enables timely prognostication and alignment of care with patient goals.Although sCJD has been reported in recipients of other solid organ transplants, this case adds to the limited literature in lung transplant patients. The potential role of chronic immunosuppression in modulating disease onset or accelerating progression remains unknown and warrants further study.