Resumo

CUBIDES-DIAZ, Diego Alejandro et al. Encephalitis And Rapidly Progressive Dementia Due To Probable Prion Disease Presenting With A Non-Convulsive Status Epilepticus. Case Report And Literature Review. Rev Ecuat Neurol [online]. 2024, vol.33, n.1, pp.115-121. ISSN 2631-2581.  https://doi.org/10.46997/revecuatneurol33100115.

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.

Palavras-chave : Encephalitis; Dementia; Prion Disease; Non-convulsive status epilepticus; Creutzfeldt-Jakob syndrome.

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