A Family of Sporadic Creutzfeldt-Jakob Disease (sCJD).

Objective: sCJD is a rare prion disease with prevalence of 1 per 1,000,000 population per year. We present a case of sCJD whose family members & one friend died from sCJD recently. Background: NA Design/Methods: A 60-year-old right-handed male was evaluated in clinic for 6–8 weeks of behavioral issues. He lost a close friend & brother in past 6 months & father few years ago. All of them had rapidly progressive bizarre behavioral & memory issues. His friend had biopsy proven CJD. Interestingly, all of them were avid deer hunters. He had very prominent avolition with abulia with MoCA :16/30. Initial MRI brain was unremarkable. Patient was admitted a week later for worsening behavioral issues, staring episodes without loss of consciousness & left arm dystonic posturing. On exam, patient was not oriented, could not follow commands & speech was perseverative & easily distracted. He had hypertonia in left arm>leg. Labs-Serum T. Pallidum Ab: +ve but RPR 4000pg/ml. Histopathology confirmed the diagnosis of sCJD. Results: NA Conclusions: sCJD is a differential diagnosis even in the presence of family history, especially when there is a shared exposure. Disclosure: Dr. Nemade has nothing to disclose. Dr. Adams has nothing to disclose. Dr. Hanif has nothing to disclose. Dr. Nolte has nothing to disclose. Dr. Singh has nothing to disclose.