Recurrent Fulminant Tumefactive Demyelination With Marburg-Like Features and Atypical Presentation: Therapeutic Dilemmas and Review of Literature

Atypical forms of demyelinating diseases with tumor-like lesions and aggressive course represent a diagnostic and therapeutic challenge for neurologists. Herein, we describe a 50-year-old woman presenting with subacute onset of left hemiparesis, memory difficulties and headache. Brain MRI revealed a...

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Autores principales: Vakrakou, Aigli G., Tzanetakos, Dimitrios, Argyrakos, Theodore, Koutsis, Georgios, Evangelopoulos, Maria-Eleptheria, Andreadou, Elisabeth, Anagnostouli, Maria, Breza, Marianthi, Tzartos, John S., Gialafos, Elias, Dimitrakopoulos, Antonios N., Velonakis, Georgios, Toulas, Panagiotis, Stefanis, Leonidas, Kilidireas, Constantinos
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7344179/
https://www.ncbi.nlm.nih.gov/pubmed/32714265
http://dx.doi.org/10.3389/fneur.2020.00536
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author Vakrakou, Aigli G.
Tzanetakos, Dimitrios
Argyrakos, Theodore
Koutsis, Georgios
Evangelopoulos, Maria-Eleptheria
Andreadou, Elisabeth
Anagnostouli, Maria
Breza, Marianthi
Tzartos, John S.
Gialafos, Elias
Dimitrakopoulos, Antonios N.
Velonakis, Georgios
Toulas, Panagiotis
Stefanis, Leonidas
Kilidireas, Constantinos
author_facet Vakrakou, Aigli G.
Tzanetakos, Dimitrios
Argyrakos, Theodore
Koutsis, Georgios
Evangelopoulos, Maria-Eleptheria
Andreadou, Elisabeth
Anagnostouli, Maria
Breza, Marianthi
Tzartos, John S.
Gialafos, Elias
Dimitrakopoulos, Antonios N.
Velonakis, Georgios
Toulas, Panagiotis
Stefanis, Leonidas
Kilidireas, Constantinos
author_sort Vakrakou, Aigli G.
collection PubMed
description Atypical forms of demyelinating diseases with tumor-like lesions and aggressive course represent a diagnostic and therapeutic challenge for neurologists. Herein, we describe a 50-year-old woman presenting with subacute onset of left hemiparesis, memory difficulties and headache. Brain MRI revealed a tumefactive right frontal-parietal lesion with perilesional edema, mass effect and homogenous post-contrast enhancement, along with other small atypical lesions in the white-matter. Brain biopsy of cerebral lesion ruled out lymphoma or any other neoplastic process and patient placed on corticosteroids with complete clinical/radiological remission. Two years after disease initiation, there was disease exacerbation with reappearance of the tumor-like mass. The patient initially responded to high doses of corticosteroids but soon became resistant. Plasma-exchange sessions were not able to limit disease burden. Resistance to therapeutic efforts led to a second biopsy that showed perivascular demyelination, predominantly consisting of macrophages, with a small number of T and B lymphocytes, and the presence of reactive astrocytes, typical of Creutzfeldt-Peters cells. The patient received high doses of cyclophosphamide with substantial clinical/radiological response but relapsed after 7-intensive cycles. She received 4-weekly doses of rituximab with disease exacerbation and brainstem involvement. She eventually died with complicated pneumonia. We present a very rare case of recurrent tumefactive demyelinating lesions, with atypical tumor-like characteristics, with initial response to corticosteroids and cyclophosphamide, but subsequent development of drug-resistance and unexpected exacerbation upon rituximab administration. Our clinical case raises therapeutic dilemmas and points to the need for immediate and appropriate immunosuppression in difficult to treat tumefactive CNS lesions with Marburg-like features.
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spelling pubmed-73441792020-07-25 Recurrent Fulminant Tumefactive Demyelination With Marburg-Like Features and Atypical Presentation: Therapeutic Dilemmas and Review of Literature Vakrakou, Aigli G. Tzanetakos, Dimitrios Argyrakos, Theodore Koutsis, Georgios Evangelopoulos, Maria-Eleptheria Andreadou, Elisabeth Anagnostouli, Maria Breza, Marianthi Tzartos, John S. Gialafos, Elias Dimitrakopoulos, Antonios N. Velonakis, Georgios Toulas, Panagiotis Stefanis, Leonidas Kilidireas, Constantinos Front Neurol Neurology Atypical forms of demyelinating diseases with tumor-like lesions and aggressive course represent a diagnostic and therapeutic challenge for neurologists. Herein, we describe a 50-year-old woman presenting with subacute onset of left hemiparesis, memory difficulties and headache. Brain MRI revealed a tumefactive right frontal-parietal lesion with perilesional edema, mass effect and homogenous post-contrast enhancement, along with other small atypical lesions in the white-matter. Brain biopsy of cerebral lesion ruled out lymphoma or any other neoplastic process and patient placed on corticosteroids with complete clinical/radiological remission. Two years after disease initiation, there was disease exacerbation with reappearance of the tumor-like mass. The patient initially responded to high doses of corticosteroids but soon became resistant. Plasma-exchange sessions were not able to limit disease burden. Resistance to therapeutic efforts led to a second biopsy that showed perivascular demyelination, predominantly consisting of macrophages, with a small number of T and B lymphocytes, and the presence of reactive astrocytes, typical of Creutzfeldt-Peters cells. The patient received high doses of cyclophosphamide with substantial clinical/radiological response but relapsed after 7-intensive cycles. She received 4-weekly doses of rituximab with disease exacerbation and brainstem involvement. She eventually died with complicated pneumonia. We present a very rare case of recurrent tumefactive demyelinating lesions, with atypical tumor-like characteristics, with initial response to corticosteroids and cyclophosphamide, but subsequent development of drug-resistance and unexpected exacerbation upon rituximab administration. Our clinical case raises therapeutic dilemmas and points to the need for immediate and appropriate immunosuppression in difficult to treat tumefactive CNS lesions with Marburg-like features. Frontiers Media S.A. 2020-06-30 /pmc/articles/PMC7344179/ /pubmed/32714265 http://dx.doi.org/10.3389/fneur.2020.00536 Text en Copyright © 2020 Vakrakou, Tzanetakos, Argyrakos, Koutsis, Evangelopoulos, Andreadou, Anagnostouli, Breza, Tzartos, Gialafos, Dimitrakopoulos, Velonakis, Toulas, Stefanis and Kilidireas. 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) and the copyright owner(s) 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 Neurology
Vakrakou, Aigli G.
Tzanetakos, Dimitrios
Argyrakos, Theodore
Koutsis, Georgios
Evangelopoulos, Maria-Eleptheria
Andreadou, Elisabeth
Anagnostouli, Maria
Breza, Marianthi
Tzartos, John S.
Gialafos, Elias
Dimitrakopoulos, Antonios N.
Velonakis, Georgios
Toulas, Panagiotis
Stefanis, Leonidas
Kilidireas, Constantinos
Recurrent Fulminant Tumefactive Demyelination With Marburg-Like Features and Atypical Presentation: Therapeutic Dilemmas and Review of Literature
title Recurrent Fulminant Tumefactive Demyelination With Marburg-Like Features and Atypical Presentation: Therapeutic Dilemmas and Review of Literature
title_full Recurrent Fulminant Tumefactive Demyelination With Marburg-Like Features and Atypical Presentation: Therapeutic Dilemmas and Review of Literature
title_fullStr Recurrent Fulminant Tumefactive Demyelination With Marburg-Like Features and Atypical Presentation: Therapeutic Dilemmas and Review of Literature
title_full_unstemmed Recurrent Fulminant Tumefactive Demyelination With Marburg-Like Features and Atypical Presentation: Therapeutic Dilemmas and Review of Literature
title_short Recurrent Fulminant Tumefactive Demyelination With Marburg-Like Features and Atypical Presentation: Therapeutic Dilemmas and Review of Literature
title_sort recurrent fulminant tumefactive demyelination with marburg-like features and atypical presentation: therapeutic dilemmas and review of literature
topic Neurology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7344179/
https://www.ncbi.nlm.nih.gov/pubmed/32714265
http://dx.doi.org/10.3389/fneur.2020.00536
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