Publicación Oficial de la Sociedad Ecuatoriana de Neurología, de la Liga Ecuatoriana Contra la Epilepsia y de la Sociedad Iberoamericana de Enfermedad Cerebrovascular

Estatus epileptico no convulsivo

 

Encephalitis and rapidly progressive dementia due to probable Prion disease presenting with a non-convulsive status epilepticus. Case report and literature review. Encefalitis y demencia rápidamente progresiva por probable enfermedad priónica que se presenta con un estado epiléptico no convulsivo. Reporte de caso y revisión de la literatura.

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.

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