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Tumefactive demyelinating CNS lesion in a 60-year-old woman with familial Mediterranean fever

We here report on a 60-year-old woman with familial Mediterranean fever (FMF) who developed cognitive impairment 16 years after initial diagnosis. On MRI, a new extensive white matter lesion in the right frontal lobe with mild local mass effect but without contrast enhancement was detectable and cla...

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Autores principales: Trostel, Constanze, Laichinger, Kornelia, Hauser, Till-Karsten, Saur, Sebastian, Krumbholz, Markus, Henes, Jörg, Ziemann, Ulf, Kowarik, Markus C.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Vienna 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9606047/
https://www.ncbi.nlm.nih.gov/pubmed/34731365
http://dx.doi.org/10.1007/s10354-021-00893-z
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author Trostel, Constanze
Laichinger, Kornelia
Hauser, Till-Karsten
Saur, Sebastian
Krumbholz, Markus
Henes, Jörg
Ziemann, Ulf
Kowarik, Markus C.
author_facet Trostel, Constanze
Laichinger, Kornelia
Hauser, Till-Karsten
Saur, Sebastian
Krumbholz, Markus
Henes, Jörg
Ziemann, Ulf
Kowarik, Markus C.
author_sort Trostel, Constanze
collection PubMed
description We here report on a 60-year-old woman with familial Mediterranean fever (FMF) who developed cognitive impairment 16 years after initial diagnosis. On MRI, a new extensive white matter lesion in the right frontal lobe with mild local mass effect but without contrast enhancement was detectable and classified as a tumefactive lesion. Additional MR spectroscopy showed markedly increased choline levels accompanied by a significant lactate peak, highly suggestive of a low-florid demyelinating process. Although diffuse central nervous system (CNS) lesions have been described in single FMF cases, tumefactive lesions have not been observed in FMF patients without concomitant multiple sclerosis. In summary, this case highlights rare differential diagnoses of atypical, inflammatory CNS lesions and the clinical utility of MR spectroscopy.
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spelling pubmed-96060472022-10-28 Tumefactive demyelinating CNS lesion in a 60-year-old woman with familial Mediterranean fever Trostel, Constanze Laichinger, Kornelia Hauser, Till-Karsten Saur, Sebastian Krumbholz, Markus Henes, Jörg Ziemann, Ulf Kowarik, Markus C. Wien Med Wochenschr Main Topic We here report on a 60-year-old woman with familial Mediterranean fever (FMF) who developed cognitive impairment 16 years after initial diagnosis. On MRI, a new extensive white matter lesion in the right frontal lobe with mild local mass effect but without contrast enhancement was detectable and classified as a tumefactive lesion. Additional MR spectroscopy showed markedly increased choline levels accompanied by a significant lactate peak, highly suggestive of a low-florid demyelinating process. Although diffuse central nervous system (CNS) lesions have been described in single FMF cases, tumefactive lesions have not been observed in FMF patients without concomitant multiple sclerosis. In summary, this case highlights rare differential diagnoses of atypical, inflammatory CNS lesions and the clinical utility of MR spectroscopy. Springer Vienna 2021-11-03 2022 /pmc/articles/PMC9606047/ /pubmed/34731365 http://dx.doi.org/10.1007/s10354-021-00893-z Text en © The Author(s) 2021 https://creativecommons.org/licenses/by/4.0/Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) .
spellingShingle Main Topic
Trostel, Constanze
Laichinger, Kornelia
Hauser, Till-Karsten
Saur, Sebastian
Krumbholz, Markus
Henes, Jörg
Ziemann, Ulf
Kowarik, Markus C.
Tumefactive demyelinating CNS lesion in a 60-year-old woman with familial Mediterranean fever
title Tumefactive demyelinating CNS lesion in a 60-year-old woman with familial Mediterranean fever
title_full Tumefactive demyelinating CNS lesion in a 60-year-old woman with familial Mediterranean fever
title_fullStr Tumefactive demyelinating CNS lesion in a 60-year-old woman with familial Mediterranean fever
title_full_unstemmed Tumefactive demyelinating CNS lesion in a 60-year-old woman with familial Mediterranean fever
title_short Tumefactive demyelinating CNS lesion in a 60-year-old woman with familial Mediterranean fever
title_sort tumefactive demyelinating cns lesion in a 60-year-old woman with familial mediterranean fever
topic Main Topic
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9606047/
https://www.ncbi.nlm.nih.gov/pubmed/34731365
http://dx.doi.org/10.1007/s10354-021-00893-z
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