57-year-old female with past medical history of anxiety and hypertension presented with two weeks of progressive speech impairment and difficulty with ADLs. She was evaluated by her primary care physician and found to have a urinary tract infection. Due to worsening symptoms, she required hospitalization. Neurological examination demonstrated abnormal movements concerning for myoclonic jerks in addition to speech impairment. Magnetic resonance imaging of her brain revealed bilateral caudate and lentiform hyperintensity along with cortical hyperintensity along the left frontal cortex. Electroencephalogram showed left frontal sharp wave discharges concerning for nonconvulsive status epilepticus. Lumbar puncture was obtained. CSF protein was elevated at 46 mg/dL with a normal CSF white blood cell count.  Due to initial concern for limbic encephalitis, she was treated with steroids and intravenous immunoglobulin without improvement. CSF final autoimmune, infectious, and paraneoplastic testing were all negative. The patient continued to seize despite burst suppression and anti-epileptic medications including Levetiracetam, Clobazam, Valproic Acid, Topiramate and Lacosamide. Upon further questioning, the patient’s mother died 15 years prior and had a rapidly progressive decline of cognitive function proceeding death. A CSF RT-QuiC had been sent and later resulted positive along with T-tau greater than 20,000 pg/ml and 14-3-3 proteins 157702 ng/ml. Family made the decision to progress to comfort directed care.

Seizures are an uncommon early symptom of sporadic CJD (roughly only 3%) and can mimic other disease presentations and are even rarer in genetic forms of CJD. This case report emphasizes the importance of performing appropriate testing given the phenotypic variability seen in this case.