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,...

Descripción completa

Detalles Bibliográficos
Autores principales: Castellano, James Francis, Meyer, Jenny A, Lado, Fred Alexander
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