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Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS)—Does it Really Exist?

Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS) is a rare variant of the CLIPPERS spectrum with less than ten reports published so far. There is ongoing discussion regarding whether SLIPPERS is a disease entity on its own or just an...

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Autores principales: Freua, Fernando, Mahler, João Vitor, Lima, Pedro Lucas Grangeiro de Sá Barreto, Neville, Iuri Santana, Portella, Leonardo Barreira, Marussi, Victor Hugo Rocha, Lancellotti, Carmen Lucia Penteado, Nobrega, Paulo Ribeiro, Silva, Guilherme Diogo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10452368/
https://www.ncbi.nlm.nih.gov/pubmed/37626547
http://dx.doi.org/10.3390/brainsci13081191
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author Freua, Fernando
Mahler, João Vitor
Lima, Pedro Lucas Grangeiro de Sá Barreto
Neville, Iuri Santana
Portella, Leonardo Barreira
Marussi, Victor Hugo Rocha
Lancellotti, Carmen Lucia Penteado
Nobrega, Paulo Ribeiro
Silva, Guilherme Diogo
author_facet Freua, Fernando
Mahler, João Vitor
Lima, Pedro Lucas Grangeiro de Sá Barreto
Neville, Iuri Santana
Portella, Leonardo Barreira
Marussi, Victor Hugo Rocha
Lancellotti, Carmen Lucia Penteado
Nobrega, Paulo Ribeiro
Silva, Guilherme Diogo
author_sort Freua, Fernando
collection PubMed
description Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS) is a rare variant of the CLIPPERS spectrum with less than ten reports published so far. There is ongoing discussion regarding whether SLIPPERS is a disease entity on its own or just an acronym encompassing many underlying diagnoses, such as sarcoidosis, vasculitis and anti-glial fibrillary acidic protein (GFAP)-associated disease. A 40-year-old woman presented with episodes of language and attention impairment. Magnetic resonance imaging (MRI) revealed T2/FLAIR hyperintense lesions in the subcortical white matter associated with a micronodular, curvilinear perivascular contrast-enhancement. Alternative diagnoses were excluded. There was a remarkable response to steroids. A relapse occurred after six years, and the biopsy showed perivascular T-cell lymphocytic infiltrate, without granulomas, vasculitis, or neoplasia. There was complete resolution of the relapse after steroids. This case represents the longest reported follow-up of a patient diagnosed with SLIPPERS, and brain biopsy after 6 years did not suggest alternative diagnoses. This report contributes to the discussion regarding the possibility that exclusive supratentorial CLIPPERS-like pathology might be an isolated disease entity, but more biopsy-proven cases with a longer follow-up are needed to support this hypothesis. Recently, GFAP astrocytopathy has been characterized and might correspond to a significant number of cases previously diagnosed as CLIPPERS or SLIPPERS.
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spelling pubmed-104523682023-08-26 Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS)—Does it Really Exist? Freua, Fernando Mahler, João Vitor Lima, Pedro Lucas Grangeiro de Sá Barreto Neville, Iuri Santana Portella, Leonardo Barreira Marussi, Victor Hugo Rocha Lancellotti, Carmen Lucia Penteado Nobrega, Paulo Ribeiro Silva, Guilherme Diogo Brain Sci Case Report Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS) is a rare variant of the CLIPPERS spectrum with less than ten reports published so far. There is ongoing discussion regarding whether SLIPPERS is a disease entity on its own or just an acronym encompassing many underlying diagnoses, such as sarcoidosis, vasculitis and anti-glial fibrillary acidic protein (GFAP)-associated disease. A 40-year-old woman presented with episodes of language and attention impairment. Magnetic resonance imaging (MRI) revealed T2/FLAIR hyperintense lesions in the subcortical white matter associated with a micronodular, curvilinear perivascular contrast-enhancement. Alternative diagnoses were excluded. There was a remarkable response to steroids. A relapse occurred after six years, and the biopsy showed perivascular T-cell lymphocytic infiltrate, without granulomas, vasculitis, or neoplasia. There was complete resolution of the relapse after steroids. This case represents the longest reported follow-up of a patient diagnosed with SLIPPERS, and brain biopsy after 6 years did not suggest alternative diagnoses. This report contributes to the discussion regarding the possibility that exclusive supratentorial CLIPPERS-like pathology might be an isolated disease entity, but more biopsy-proven cases with a longer follow-up are needed to support this hypothesis. Recently, GFAP astrocytopathy has been characterized and might correspond to a significant number of cases previously diagnosed as CLIPPERS or SLIPPERS. MDPI 2023-08-11 /pmc/articles/PMC10452368/ /pubmed/37626547 http://dx.doi.org/10.3390/brainsci13081191 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Case Report
Freua, Fernando
Mahler, João Vitor
Lima, Pedro Lucas Grangeiro de Sá Barreto
Neville, Iuri Santana
Portella, Leonardo Barreira
Marussi, Victor Hugo Rocha
Lancellotti, Carmen Lucia Penteado
Nobrega, Paulo Ribeiro
Silva, Guilherme Diogo
Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS)—Does it Really Exist?
title Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS)—Does it Really Exist?
title_full Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS)—Does it Really Exist?
title_fullStr Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS)—Does it Really Exist?
title_full_unstemmed Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS)—Does it Really Exist?
title_short Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS)—Does it Really Exist?
title_sort supratentorial lymphocytic inflammation with parenchymal perivascular enhancement responsive to steroids (slippers)—does it really exist?
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10452368/
https://www.ncbi.nlm.nih.gov/pubmed/37626547
http://dx.doi.org/10.3390/brainsci13081191
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