Introduction: Creutzfeldt-Jakob disease (CJD) is a rapidly progressive, fatal neurodegenerative disorder affecting one individual per million per year, worldwide. Variant (vCJD) accounts for less than 1% of CJD cases. Person-to-person transmission of vCJD has occurred through blood transfusion and theoretically could occur via organ or tissue transplantation. We present a case of a 54-year-old gentleman who was diagnosed with CJD two years after liver transplant.
Case Description/Methods: A 54-year-old man with history of Hepatitis C/ Alcoholic Cirrhosis, hepatocellular carcinoma and orthotopic liver transplant was admitted for obstructive jaundice. He underwent ERCP with stone extraction. Postoperative course was complicated by intermittent confusion, headaches, insomnia and jerky limb movements. Family reported similar symptoms since transplant with worsening after the procedure. On exam, he was afebrile, intermittently confused, dysphoric with non focal neurological exam. He had episodes of agitation, belligerence, falls, loss of consciousness, auditory and visual hallucinations during hospital stay. Medications potentially contributing to delirium were held. Laboratory work-up, lumbar puncture, CT and MRI brain were unremarkable. EEG revealed extensive muscle artifact and mild diffuse encephalopathy. He was treated for acute neuropsychiatric decompensation and was discharged home after clinical improvement. Patient was seen in Transplant Clinic four months later with recurrent symptoms. A repeat MRI brain with contrast revealed interval development of hyperintense signal within the thalami. A repeat lumbar puncture revealed normal glucose, high protein, Tau protein in cerebrospinal fluid and positive RT-QuIC. These CSF findings combined with symptoms and MRI lesions led to the diagnosis of CJD. Patient died within a few months after his diagnosis.
Discussion: CJD via organ transplant is controversial and rare with very few cases reported in the literature. It has never been unequivocally related to inoculation of tissue other than the nervous tissue. Livers of patients with subacute spongiform encephalopathy (SSE) have transmitted disease to primates pointing to their infective potential. Our case highlights the importance of continuing to seek to identify individuals who might be at risk of CJD by this route so that appropriate public health measures can be implemented. The role of immunosuppressants in the acceleration of the disease course has also never been established and needs further research.
Figure: Magnetic Resonance Images with contrast: a) Symmetric hyperintensity of the pulvinar (posterior) thalamic nuclei on DWI (Pulvinar sign of vCJD) b) FLAIR image shows symmetric pulvinar and dorsomedial thalamic nuclear hyperintensity (Hockey-stick sign of vCJD)
Disclosures: Aimen Farooq indicated no relevant financial relationships. Baha Aldeen Bani Fawwaz indicated no relevant financial relationships. Ahmed Ramadan indicated no relevant financial relationships. Jian Guan indicated no relevant financial relationships. Aymen Koteish indicated no relevant financial relationships.
Aimen Farooq, MD, Baha Aldeen Bani Fawwaz, MD, Ahmed Ramadan, MD, Jian Guan, MD, PhD, Aymen Koteish, MD. P2926 - A Rare Case of Creutzfeldt-Jakob Disease in a Liver Transplant Patient, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.