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Lymphomatosis cerebri Presenting as a Recurrent Leukoencephalopathy

A 29-year-old immunocompetent woman was admitted in 2006 with ataxia, limb weakness, generalized dystonia, and vertical diplopia that developed after a febrile episode. Brain magnetic resonance imaging (MRI) revealed the presence of extensive periventricular white matter lesions that did not enhance...

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Autores principales: Courtois, Floriane, Gille, Michel, Haven, Frédéric, Hantson, Philippe
Formato: Online Artículo Texto
Lenguaje:English
Publicado: S. Karger AG 2012
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3506038/
https://www.ncbi.nlm.nih.gov/pubmed/23185172
http://dx.doi.org/10.1159/000343947
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author Courtois, Floriane
Gille, Michel
Haven, Frédéric
Hantson, Philippe
author_facet Courtois, Floriane
Gille, Michel
Haven, Frédéric
Hantson, Philippe
author_sort Courtois, Floriane
collection PubMed
description A 29-year-old immunocompetent woman was admitted in 2006 with ataxia, limb weakness, generalized dystonia, and vertical diplopia that developed after a febrile episode. Brain magnetic resonance imaging (MRI) revealed the presence of extensive periventricular white matter lesions that did not enhance after gadolinium injection. As low titers of cytomegalovirus-IgM antibodies were found in the serum, a presumed diagnosis of postviral acute disseminated encephalomyelitis (ADEM) was made, and the patient received a 5-day course of 1 g methylprednisolone. The clinical and radiological outcome was very rapidly favorable, and subsequent brain MRIs in 2007 and 2008 were normal. In March 2011, the patient was readmitted with the complaints of abnormal fatigue, imbalance, and speech disorder. The neurological examination showed fluctuating spatiotemporal disorientation with dyscalculia, verbal deafness, gait ataxia, right hemianopia, and pyramidal signs in the four limbs. The brain MRI demonstrated extensive T(2) hyperintense white matter lesions predominating in the left temporal and parieto-occipital lobes, with a pseudotumoral aspect enhancing with gadolinium contrast. A clinical improvement was transiently noted after pulse steroid therapy, but after relapse and radiological worsening, the diagnosis of recurrent ADEM was challenged. The brain biopsy confirmed the presence of primary central nervous system lymphoma (PCNSL) under the variant form of lymphomatosis cerebri. Despite a partial response to chemotherapy, the patient died 8 months after the diagnosis. We discuss the role of sentinel lesions that may precede PCNSL for several years and insist on the importance to consider early brain biopsy in the presence of extensive, non-enhancing white matter lesions, even in a young and immunocompetent patient.
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spelling pubmed-35060382012-11-26 Lymphomatosis cerebri Presenting as a Recurrent Leukoencephalopathy Courtois, Floriane Gille, Michel Haven, Frédéric Hantson, Philippe Case Rep Neurol Published online: November, 2012 A 29-year-old immunocompetent woman was admitted in 2006 with ataxia, limb weakness, generalized dystonia, and vertical diplopia that developed after a febrile episode. Brain magnetic resonance imaging (MRI) revealed the presence of extensive periventricular white matter lesions that did not enhance after gadolinium injection. As low titers of cytomegalovirus-IgM antibodies were found in the serum, a presumed diagnosis of postviral acute disseminated encephalomyelitis (ADEM) was made, and the patient received a 5-day course of 1 g methylprednisolone. The clinical and radiological outcome was very rapidly favorable, and subsequent brain MRIs in 2007 and 2008 were normal. In March 2011, the patient was readmitted with the complaints of abnormal fatigue, imbalance, and speech disorder. The neurological examination showed fluctuating spatiotemporal disorientation with dyscalculia, verbal deafness, gait ataxia, right hemianopia, and pyramidal signs in the four limbs. The brain MRI demonstrated extensive T(2) hyperintense white matter lesions predominating in the left temporal and parieto-occipital lobes, with a pseudotumoral aspect enhancing with gadolinium contrast. A clinical improvement was transiently noted after pulse steroid therapy, but after relapse and radiological worsening, the diagnosis of recurrent ADEM was challenged. The brain biopsy confirmed the presence of primary central nervous system lymphoma (PCNSL) under the variant form of lymphomatosis cerebri. Despite a partial response to chemotherapy, the patient died 8 months after the diagnosis. We discuss the role of sentinel lesions that may precede PCNSL for several years and insist on the importance to consider early brain biopsy in the presence of extensive, non-enhancing white matter lesions, even in a young and immunocompetent patient. S. Karger AG 2012-11-06 /pmc/articles/PMC3506038/ /pubmed/23185172 http://dx.doi.org/10.1159/000343947 Text en Copyright © 2012 by S. Karger AG, Basel http://creativecommons.org/licenses/by-nc-nd/3.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution-Noncommercial-No-Derivative-Works License (http://creativecommons.org/licenses/by-nc-nd/3.0/). Users may download, print and share this work on the Internet for noncommercial purposes only, provided the original work is properly cited, and a link to the original work on http://www.karger.com and the terms of this license are included in any shared versions.
spellingShingle Published online: November, 2012
Courtois, Floriane
Gille, Michel
Haven, Frédéric
Hantson, Philippe
Lymphomatosis cerebri Presenting as a Recurrent Leukoencephalopathy
title Lymphomatosis cerebri Presenting as a Recurrent Leukoencephalopathy
title_full Lymphomatosis cerebri Presenting as a Recurrent Leukoencephalopathy
title_fullStr Lymphomatosis cerebri Presenting as a Recurrent Leukoencephalopathy
title_full_unstemmed Lymphomatosis cerebri Presenting as a Recurrent Leukoencephalopathy
title_short Lymphomatosis cerebri Presenting as a Recurrent Leukoencephalopathy
title_sort lymphomatosis cerebri presenting as a recurrent leukoencephalopathy
topic Published online: November, 2012
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3506038/
https://www.ncbi.nlm.nih.gov/pubmed/23185172
http://dx.doi.org/10.1159/000343947
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