Encephalitis and rapidly progressive dementia due to probable Prion disease presenting with a non-convulsive status epilepticus. Case report and literature review

Abstract

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.