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.

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 <1:1. Video EEG showed GPDs with numerous bursts of rhythmic sharp waves and PLD in F4, F8 region. Solumedrol was added for possible auto-immune encephalitis but had to be discontinued due to agitation. Quetiapine 50mg QHS & IVIG x 5 days was then added. In the 1^st week, worsening electrographic seizures led to intubation & escalation in antiepileptic therapy & higher Midazolam doses. He later developed spontaneous & startle myoclonus. EEG showed 0.5-1 Hz biphasics & triphasics. MRI brain 2 weeks later showed cortical ribboning in right hemisphere & hockey stick sign. PRNP gene, Mayo Paraneoplastic & autoimmune epilepsy panel were negative. After discussion with wife 2 weeks later, patient was made comfort care & died on the same day. Lab report later showed positive RT-QuIC & Protein 14-3-3, CSF tau protein > 4000pg/ml. Histopathology confirmed the diagnosis of sCJD.