Non convulsive status epilepticus

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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Palabras clave: demencia, encefalitis, Enfermedad por priones, Estatus epileptico no convulsivo, Síndrome de Creutzfeldt-Jakob,

Keywords: Creutzfeldt-Jakob syndrome, dementia, encephalitis, Non convulsive status epilepticus, Prion Disease,

Evaluación de la Utilidad de las Escalas Pronósticas del Status Epiléptico Convulsivo en el Status Epiléptico no Convulsivo. Usefulness Of Status Convulsive Score In Prognosis Of Non-Convulsive Status Epilepticus

Introduction: The Non-Convulsive Status Epilepticus (NCSE) is a rare clinical entity. As of today, a predictive mortality scale has not been developed. Moreover, the risk factors that contribute to the prognosis have not been clearly defined. The aim of this study was to evaluate the usefulness of the prognostic scores for status epilepticus (SE): the epileptic status severity score (STESS), the modified RANKIN scale STESS (mRSTESS) and the epileptic status mortality score based on epidemiology (EMSE-EACEG) applied in patients diagnosed with NCSE.

Results: The sample analyzed for this study consisted of 66 patients with an average age of 59 years (SD 18.5).

The STESS and mRSTESS were significantly associated with higher odds for poor prognosis at the time of discharge in patients with NCSE (p<0.05 and p<0.05, respectively). However, none of the three was associated with mortality.

Conclusion: Considering that the scores used to predict prognosis in patients with SE have not shown equal diagnostic accuracy in the NCSE, we believe that a validated predictive scale of morbidity and mortality would be of great help for these patients.