Prion diseases are rare and rapidly progressive fatal neurological disorders characterized by abnormal folding of neuronal proteins.
The diagnosis is often challenging and relies on a high clinical suspicion, imagenological findings, electroencephalographic (EEG) patterns and cerebrospinal fluid (CSF) analysis.
We present a case of probable prion disease with an accelerated neurological decline and a non-convulsive status epilepticus (NCSE), which has only been described in a few cases worldwide, and seems to be associated with worse neurological outcomes and shorter survival time.
Clinical manifestations, treatment, and outcomes are shown below.
