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New-Onset Refractory Status Epilepticus: More Investigations, More Questions

A 27-year-old previously healthy woman was admitted to the hospital with recurrent seizures. Status epilepticus developed that became refractory to third-line therapy with propofol and barbiturates. The patient had a very extensive diagnostic workup including autoimmune, viral and genetic investigat...

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Autores principales: Dillien, Philippe, Ferrao Santos, Susana, van Pesch, Vincent, Suin, Vanessa, Lamoral, Sophie, Hantson, Philippe
Formato: Online Artículo Texto
Lenguaje:English
Publicado: S. Karger AG 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4939680/
https://www.ncbi.nlm.nih.gov/pubmed/27462243
http://dx.doi.org/10.1159/000447295
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author Dillien, Philippe
Ferrao Santos, Susana
van Pesch, Vincent
Suin, Vanessa
Lamoral, Sophie
Hantson, Philippe
author_facet Dillien, Philippe
Ferrao Santos, Susana
van Pesch, Vincent
Suin, Vanessa
Lamoral, Sophie
Hantson, Philippe
author_sort Dillien, Philippe
collection PubMed
description A 27-year-old previously healthy woman was admitted to the hospital with recurrent seizures. Status epilepticus developed that became refractory to third-line therapy with propofol and barbiturates. The patient had a very extensive diagnostic workup including autoimmune, viral and genetic investigations. A tentative immune therapy was proposed with high doses of steroids and plasma exchanges. Our patient had an inherited heterozygous single nucleotide variant in the sequence c.1280A>G [p.Lys427Arg] of the SMC3 gene that was insufficient to explain the seizures. Surprisingly, IgM antibodies against Japanese encephalitis virus were positive on the serum drawn 11 days after symptom onset, as detected by ELISA and the immunofluorescence antibody (IFA) technique. IgG antibodies were also positive using the IFA technique, but not with ELISA. The same investigations as well as the detection of the viral genome by the q-RT-PCR technique were negative on cerebrospinal fluid. Despite the suspicion of a viral infection, we concluded that our patient had a new-onset refractory status epilepticus of cryptogenic origin. Termination of the status epilepticus was obtained after 47 days, with a possible benefit from the introduction of ketamine.
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spelling pubmed-49396802016-07-26 New-Onset Refractory Status Epilepticus: More Investigations, More Questions Dillien, Philippe Ferrao Santos, Susana van Pesch, Vincent Suin, Vanessa Lamoral, Sophie Hantson, Philippe Case Rep Neurol Case Report A 27-year-old previously healthy woman was admitted to the hospital with recurrent seizures. Status epilepticus developed that became refractory to third-line therapy with propofol and barbiturates. The patient had a very extensive diagnostic workup including autoimmune, viral and genetic investigations. A tentative immune therapy was proposed with high doses of steroids and plasma exchanges. Our patient had an inherited heterozygous single nucleotide variant in the sequence c.1280A>G [p.Lys427Arg] of the SMC3 gene that was insufficient to explain the seizures. Surprisingly, IgM antibodies against Japanese encephalitis virus were positive on the serum drawn 11 days after symptom onset, as detected by ELISA and the immunofluorescence antibody (IFA) technique. IgG antibodies were also positive using the IFA technique, but not with ELISA. The same investigations as well as the detection of the viral genome by the q-RT-PCR technique were negative on cerebrospinal fluid. Despite the suspicion of a viral infection, we concluded that our patient had a new-onset refractory status epilepticus of cryptogenic origin. Termination of the status epilepticus was obtained after 47 days, with a possible benefit from the introduction of ketamine. S. Karger AG 2016-06-14 /pmc/articles/PMC4939680/ /pubmed/27462243 http://dx.doi.org/10.1159/000447295 Text en Copyright © 2016 by S. Karger AG, Basel http://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 Case Report
Dillien, Philippe
Ferrao Santos, Susana
van Pesch, Vincent
Suin, Vanessa
Lamoral, Sophie
Hantson, Philippe
New-Onset Refractory Status Epilepticus: More Investigations, More Questions
title New-Onset Refractory Status Epilepticus: More Investigations, More Questions
title_full New-Onset Refractory Status Epilepticus: More Investigations, More Questions
title_fullStr New-Onset Refractory Status Epilepticus: More Investigations, More Questions
title_full_unstemmed New-Onset Refractory Status Epilepticus: More Investigations, More Questions
title_short New-Onset Refractory Status Epilepticus: More Investigations, More Questions
title_sort new-onset refractory status epilepticus: more investigations, more questions
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4939680/
https://www.ncbi.nlm.nih.gov/pubmed/27462243
http://dx.doi.org/10.1159/000447295
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