Cargando…
A Case Series of Adult-Onset Rasmussen’s Encephalitis: Diagnostic and Therapeutic Challenges
Rasmussen’s encephalitis (RE) is a rare neurologic disorder characterized by progressive cerebral hemiatrophy and medically refractory epilepsy. The majority of current literature on this topic is focused on the pediatric population. In this case series, we will review three cases of adult-onset RE,...
Autores principales: | , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2017
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5660978/ https://www.ncbi.nlm.nih.gov/pubmed/29118737 http://dx.doi.org/10.3389/fneur.2017.00564 |
_version_ | 1783274390061842432 |
---|---|
author | Castellano, James Francis Meyer, Jenny A Lado, Fred Alexander |
author_facet | Castellano, James Francis Meyer, Jenny A Lado, Fred Alexander |
author_sort | Castellano, James Francis |
collection | PubMed |
description | Rasmussen’s encephalitis (RE) is a rare neurologic disorder characterized by progressive cerebral hemiatrophy and medically refractory epilepsy. The majority of current literature on this topic is focused on the pediatric population. In this case series, we will review three cases of adult-onset RE, as defined by fulfillment of the 2005 Bien criteria. The diagnostic challenge of characterizing this rare disease will be highlighted by the extensive serum, CSF, and pathologic sampling in all three patients. MR imaging and EEG data will be examined over time to characterize hallmark findings as well as progression. In addition, we will review the various forms of therapy attempted in these three patients, namely anti-epileptic drug therapy and immunomodulatory therapy. We will also utilize this case series to critically evaluate the broader context of atypical presentations of this disease and the value of current diagnostic criteria. |
format | Online Article Text |
id | pubmed-5660978 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-56609782017-11-08 A Case Series of Adult-Onset Rasmussen’s Encephalitis: Diagnostic and Therapeutic Challenges Castellano, James Francis Meyer, Jenny A Lado, Fred Alexander Front Neurol Neuroscience Rasmussen’s encephalitis (RE) is a rare neurologic disorder characterized by progressive cerebral hemiatrophy and medically refractory epilepsy. The majority of current literature on this topic is focused on the pediatric population. In this case series, we will review three cases of adult-onset RE, as defined by fulfillment of the 2005 Bien criteria. The diagnostic challenge of characterizing this rare disease will be highlighted by the extensive serum, CSF, and pathologic sampling in all three patients. MR imaging and EEG data will be examined over time to characterize hallmark findings as well as progression. In addition, we will review the various forms of therapy attempted in these three patients, namely anti-epileptic drug therapy and immunomodulatory therapy. We will also utilize this case series to critically evaluate the broader context of atypical presentations of this disease and the value of current diagnostic criteria. Frontiers Media S.A. 2017-10-25 /pmc/articles/PMC5660978/ /pubmed/29118737 http://dx.doi.org/10.3389/fneur.2017.00564 Text en Copyright © 2017 Castellano, Meyer and Lado. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Neuroscience Castellano, James Francis Meyer, Jenny A Lado, Fred Alexander A Case Series of Adult-Onset Rasmussen’s Encephalitis: Diagnostic and Therapeutic Challenges |
title | A Case Series of Adult-Onset Rasmussen’s Encephalitis: Diagnostic and Therapeutic Challenges |
title_full | A Case Series of Adult-Onset Rasmussen’s Encephalitis: Diagnostic and Therapeutic Challenges |
title_fullStr | A Case Series of Adult-Onset Rasmussen’s Encephalitis: Diagnostic and Therapeutic Challenges |
title_full_unstemmed | A Case Series of Adult-Onset Rasmussen’s Encephalitis: Diagnostic and Therapeutic Challenges |
title_short | A Case Series of Adult-Onset Rasmussen’s Encephalitis: Diagnostic and Therapeutic Challenges |
title_sort | case series of adult-onset rasmussen’s encephalitis: diagnostic and therapeutic challenges |
topic | Neuroscience |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5660978/ https://www.ncbi.nlm.nih.gov/pubmed/29118737 http://dx.doi.org/10.3389/fneur.2017.00564 |
work_keys_str_mv | AT castellanojamesfrancis acaseseriesofadultonsetrasmussensencephalitisdiagnosticandtherapeuticchallenges AT meyerjennya acaseseriesofadultonsetrasmussensencephalitisdiagnosticandtherapeuticchallenges AT ladofredalexander acaseseriesofadultonsetrasmussensencephalitisdiagnosticandtherapeuticchallenges AT castellanojamesfrancis caseseriesofadultonsetrasmussensencephalitisdiagnosticandtherapeuticchallenges AT meyerjennya caseseriesofadultonsetrasmussensencephalitisdiagnosticandtherapeuticchallenges AT ladofredalexander caseseriesofadultonsetrasmussensencephalitisdiagnosticandtherapeuticchallenges |