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An Evidence-Based Approach to Diagnosis and Prognosis in a Young Woman with New-Onset Super-Refractory Status Epilepticus: A Case Report

New-onset refractory status epilepticus (NORSE) is a rare clinical diagnosis. Autoimmune encephalitis and paraneoplastic encephalitis are the most common identifiable etiologies of NORSE; of the known autoimmune encephalitides, <200 cases of GAD65-related epilepsy have been reported in the litera...

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Autores principales: Juneja, Pallavi, Swor, Dionne
Formato: Online Artículo Texto
Lenguaje:English
Publicado: S. Karger AG 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8832188/
https://www.ncbi.nlm.nih.gov/pubmed/35221970
http://dx.doi.org/10.1159/000519947
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author Juneja, Pallavi
Swor, Dionne
author_facet Juneja, Pallavi
Swor, Dionne
author_sort Juneja, Pallavi
collection PubMed
description New-onset refractory status epilepticus (NORSE) is a rare clinical diagnosis. Autoimmune encephalitis and paraneoplastic encephalitis are the most common identifiable etiologies of NORSE; of the known autoimmune encephalitides, <200 cases of GAD65-related epilepsy have been reported in the literature. We describe a case of a 24-year-old female who presented with confusion and myoclonus. Electroencephalogram revealed NORSE that evolved into super-refractory status epilepticus. Her CSF showed a mild lymphocytic pleocytosis, and her MRI demonstrated changes in the bilateral temporal lobes. She was ultimately diagnosed with GAD65 autoimmune encephalitis and treated with immune-modulating therapy. This case highlights the complex diagnostic workup of patients with NORSE and the utility of validated prediction models for diagnosis and prognosis. While maintaining a broad differential, the patient's Antibody Prevalence in Epilepsy (APE) score was a helpful clinical tool to support the pursuit of an autoimmune etiology. We also underline the need to concomitantly treat patients quickly during diagnostic workup because earlier treatment increases the Response to Immunotherapy in Epilepsy (RITE) score, indicating a more favorable prognosis.
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spelling pubmed-88321882022-02-25 An Evidence-Based Approach to Diagnosis and Prognosis in a Young Woman with New-Onset Super-Refractory Status Epilepticus: A Case Report Juneja, Pallavi Swor, Dionne Case Rep Neurol Single Case − General Neurology New-onset refractory status epilepticus (NORSE) is a rare clinical diagnosis. Autoimmune encephalitis and paraneoplastic encephalitis are the most common identifiable etiologies of NORSE; of the known autoimmune encephalitides, <200 cases of GAD65-related epilepsy have been reported in the literature. We describe a case of a 24-year-old female who presented with confusion and myoclonus. Electroencephalogram revealed NORSE that evolved into super-refractory status epilepticus. Her CSF showed a mild lymphocytic pleocytosis, and her MRI demonstrated changes in the bilateral temporal lobes. She was ultimately diagnosed with GAD65 autoimmune encephalitis and treated with immune-modulating therapy. This case highlights the complex diagnostic workup of patients with NORSE and the utility of validated prediction models for diagnosis and prognosis. While maintaining a broad differential, the patient's Antibody Prevalence in Epilepsy (APE) score was a helpful clinical tool to support the pursuit of an autoimmune etiology. We also underline the need to concomitantly treat patients quickly during diagnostic workup because earlier treatment increases the Response to Immunotherapy in Epilepsy (RITE) score, indicating a more favorable prognosis. S. Karger AG 2022-01-17 /pmc/articles/PMC8832188/ /pubmed/35221970 http://dx.doi.org/10.1159/000519947 Text en Copyright © 2022 by S. Karger AG, Basel https://creativecommons.org/licenses/by-nc/4.0/This article is licensed under the Creative Commons Attribution-NonCommercial-4.0 International License (CC BY-NC) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission.
spellingShingle Single Case − General Neurology
Juneja, Pallavi
Swor, Dionne
An Evidence-Based Approach to Diagnosis and Prognosis in a Young Woman with New-Onset Super-Refractory Status Epilepticus: A Case Report
title An Evidence-Based Approach to Diagnosis and Prognosis in a Young Woman with New-Onset Super-Refractory Status Epilepticus: A Case Report
title_full An Evidence-Based Approach to Diagnosis and Prognosis in a Young Woman with New-Onset Super-Refractory Status Epilepticus: A Case Report
title_fullStr An Evidence-Based Approach to Diagnosis and Prognosis in a Young Woman with New-Onset Super-Refractory Status Epilepticus: A Case Report
title_full_unstemmed An Evidence-Based Approach to Diagnosis and Prognosis in a Young Woman with New-Onset Super-Refractory Status Epilepticus: A Case Report
title_short An Evidence-Based Approach to Diagnosis and Prognosis in a Young Woman with New-Onset Super-Refractory Status Epilepticus: A Case Report
title_sort evidence-based approach to diagnosis and prognosis in a young woman with new-onset super-refractory status epilepticus: a case report
topic Single Case − General Neurology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8832188/
https://www.ncbi.nlm.nih.gov/pubmed/35221970
http://dx.doi.org/10.1159/000519947
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