Cargando…

Bi-insular cortical involvement in anti-NMDA-receptor encephalitis – a case report

BACKGROUND: Anti-NMDA-receptor encephalitis is an immune-mediated inflammatory disorder of the central nervous system. Brain MRI is unremarkable in at least 50 % of patients and highly variable in the remaining patients with signal abnormalities in different brain regions. Only scarce reports exist...

Descripción completa

Detalles Bibliográficos
Autores principales: Hegen, Harald, Uprimny, Christian, Grams, Astrid, Virgolini, Irene, Ramberger, Melanie, Beer, Ronny, Helbok, Raimund, Pfausler, Bettina, Schmutzhard, Erich
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4977643/
https://www.ncbi.nlm.nih.gov/pubmed/27502387
http://dx.doi.org/10.1186/s12883-016-0653-9
_version_ 1782447067330772992
author Hegen, Harald
Uprimny, Christian
Grams, Astrid
Virgolini, Irene
Ramberger, Melanie
Beer, Ronny
Helbok, Raimund
Pfausler, Bettina
Schmutzhard, Erich
author_facet Hegen, Harald
Uprimny, Christian
Grams, Astrid
Virgolini, Irene
Ramberger, Melanie
Beer, Ronny
Helbok, Raimund
Pfausler, Bettina
Schmutzhard, Erich
author_sort Hegen, Harald
collection PubMed
description BACKGROUND: Anti-NMDA-receptor encephalitis is an immune-mediated inflammatory disorder of the central nervous system. Brain MRI is unremarkable in at least 50 % of patients and highly variable in the remaining patients with signal abnormalities in different brain regions. Only scarce reports exist on other imaging modalities. CASE PRESENTATION: A 31-year-old woman sub-acutely developed psychosis, behavioural changes, amnesia, alternating states of agitation and mutism, fever and epileptic seizures. Clinically suspected diagnosis of anti-NMDA-receptor encephalitis was confirmed by the detection of anti-NMDA receptor antibodies in CSF and serum. During the acute phase, brain MRI abnormalities were found in both insular cortices and hippocampi, whereas F(18)-FDG-PET showed hypermetabolism bilaterally in insular and prefrontal cortex. After resection of the underlying ovarian teratoma and with multimodal immunotherapy the patient substantially improved reaching a modified Rankin Scale score of 2 after 3 months. At follow-up, both hippocampi were still affected on MRI, whereas insular cortex appeared normal; however, both regions showed prominent glucose hypometabolism. CONCLUSIONS: Here, we report bi-insular cortical abnormalities on MRI and F(18)-FDG-PET in a patient with anti-NMDA-receptor encephalitis during the acute phase and after clinical improvement.
format Online
Article
Text
id pubmed-4977643
institution National Center for Biotechnology Information
language English
publishDate 2016
publisher BioMed Central
record_format MEDLINE/PubMed
spelling pubmed-49776432016-08-10 Bi-insular cortical involvement in anti-NMDA-receptor encephalitis – a case report Hegen, Harald Uprimny, Christian Grams, Astrid Virgolini, Irene Ramberger, Melanie Beer, Ronny Helbok, Raimund Pfausler, Bettina Schmutzhard, Erich BMC Neurol Case Report BACKGROUND: Anti-NMDA-receptor encephalitis is an immune-mediated inflammatory disorder of the central nervous system. Brain MRI is unremarkable in at least 50 % of patients and highly variable in the remaining patients with signal abnormalities in different brain regions. Only scarce reports exist on other imaging modalities. CASE PRESENTATION: A 31-year-old woman sub-acutely developed psychosis, behavioural changes, amnesia, alternating states of agitation and mutism, fever and epileptic seizures. Clinically suspected diagnosis of anti-NMDA-receptor encephalitis was confirmed by the detection of anti-NMDA receptor antibodies in CSF and serum. During the acute phase, brain MRI abnormalities were found in both insular cortices and hippocampi, whereas F(18)-FDG-PET showed hypermetabolism bilaterally in insular and prefrontal cortex. After resection of the underlying ovarian teratoma and with multimodal immunotherapy the patient substantially improved reaching a modified Rankin Scale score of 2 after 3 months. At follow-up, both hippocampi were still affected on MRI, whereas insular cortex appeared normal; however, both regions showed prominent glucose hypometabolism. CONCLUSIONS: Here, we report bi-insular cortical abnormalities on MRI and F(18)-FDG-PET in a patient with anti-NMDA-receptor encephalitis during the acute phase and after clinical improvement. BioMed Central 2016-08-08 /pmc/articles/PMC4977643/ /pubmed/27502387 http://dx.doi.org/10.1186/s12883-016-0653-9 Text en © The Author(s). 2016 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
Hegen, Harald
Uprimny, Christian
Grams, Astrid
Virgolini, Irene
Ramberger, Melanie
Beer, Ronny
Helbok, Raimund
Pfausler, Bettina
Schmutzhard, Erich
Bi-insular cortical involvement in anti-NMDA-receptor encephalitis – a case report
title Bi-insular cortical involvement in anti-NMDA-receptor encephalitis – a case report
title_full Bi-insular cortical involvement in anti-NMDA-receptor encephalitis – a case report
title_fullStr Bi-insular cortical involvement in anti-NMDA-receptor encephalitis – a case report
title_full_unstemmed Bi-insular cortical involvement in anti-NMDA-receptor encephalitis – a case report
title_short Bi-insular cortical involvement in anti-NMDA-receptor encephalitis – a case report
title_sort bi-insular cortical involvement in anti-nmda-receptor encephalitis – a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4977643/
https://www.ncbi.nlm.nih.gov/pubmed/27502387
http://dx.doi.org/10.1186/s12883-016-0653-9
work_keys_str_mv AT hegenharald biinsularcorticalinvolvementinantinmdareceptorencephalitisacasereport
AT uprimnychristian biinsularcorticalinvolvementinantinmdareceptorencephalitisacasereport
AT gramsastrid biinsularcorticalinvolvementinantinmdareceptorencephalitisacasereport
AT virgoliniirene biinsularcorticalinvolvementinantinmdareceptorencephalitisacasereport
AT rambergermelanie biinsularcorticalinvolvementinantinmdareceptorencephalitisacasereport
AT beerronny biinsularcorticalinvolvementinantinmdareceptorencephalitisacasereport
AT helbokraimund biinsularcorticalinvolvementinantinmdareceptorencephalitisacasereport
AT pfauslerbettina biinsularcorticalinvolvementinantinmdareceptorencephalitisacasereport
AT schmutzharderich biinsularcorticalinvolvementinantinmdareceptorencephalitisacasereport