Immunomodulating Biologics and sCJD

Objective: NA Background: A 69-year-old male with history of ulcerative colitis presented with cognitive decline. For colitis he was treated with infliximab, but developed anaphylaxis with it and was switched to adalimumab. After a year, he was transitioned to vedolizumab for recurrent symptoms. Design/Methods: NA Results: Over 6 months, he experienced progressive behavioral symptoms and worsening memory. Initial MOCA score 23/30. Neurological examination and brain MRI were normal. Blood work including TSH, RPR, CBC, CMP, and B12 were unrevealing. Formal neurocognitive assessment revealed deficiencies in executive functioning and memory. He had progressive cognitive decline and developed gait instability. After multiple falls, he was admitted to the hospital to expedite work-up. His examination demonstrated truncal and appendicular ataxia. Repeat brain MRI was unremarkable. EEG showed generalized slowing. Lumbar puncture was performed with normal WBC, RBC, protein, and glucose. JC virus antibody in blood and CSF were negative. Paraneoplastic antibodies and anti-thyroid antibodies were negative. CSF 14-3-3 protein and RT-QuIC were positive. After 2 months, the patient was readmitted for progressive neurologic deterioration. Examination revealed encephalopathy, dysarthria, hyperreflexia with clonus, and prominent ataxia; MOCA was 16. He developed aspiration pneumonia and was transitioned to hospice care. He died few weeks later. Brain autopsy was diagnostic of CJD. Conclusions: Immunomodulating biologics are associated with an increased incidence of opportunistic infections. Our patient received multiple biologics for his ulcerative colitis. Infliximab and adalimumab are TNF alpha inhibitors, while vedolizumab binds to α4β7 integrin in the gut. There are no reports of CJD with biologics. There has been a single report of CJD in a patient with MS on glatiramer. The development of this fatal diagnosis in our patient on biologics may be coincidental. However, as the use of biologics becomes more common we need to be vigilant and monitor patients closely for rare but serious complications. Disclosure: Dr. Lee has nothing to disclose. Dr. Ridha has nothing to disclose. Dr. Lin has nothing to disclose. Dr. Anderson has nothing to disclose. Dr. Srinivasan has nothing to disclose.