Cargando…
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...
Autores principales: | , , , , , , , , |
---|---|
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 |
_version_ | 1785095652489822208 |
---|---|
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. |
format | Online Article Text |
id | pubmed-10452368 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
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 |
work_keys_str_mv | AT freuafernando supratentoriallymphocyticinflammationwithparenchymalperivascularenhancementresponsivetosteroidsslippersdoesitreallyexist AT mahlerjoaovitor supratentoriallymphocyticinflammationwithparenchymalperivascularenhancementresponsivetosteroidsslippersdoesitreallyexist AT limapedrolucasgrangeirodesabarreto supratentoriallymphocyticinflammationwithparenchymalperivascularenhancementresponsivetosteroidsslippersdoesitreallyexist AT nevilleiurisantana supratentoriallymphocyticinflammationwithparenchymalperivascularenhancementresponsivetosteroidsslippersdoesitreallyexist AT portellaleonardobarreira supratentoriallymphocyticinflammationwithparenchymalperivascularenhancementresponsivetosteroidsslippersdoesitreallyexist AT marussivictorhugorocha supratentoriallymphocyticinflammationwithparenchymalperivascularenhancementresponsivetosteroidsslippersdoesitreallyexist AT lancellotticarmenluciapenteado supratentoriallymphocyticinflammationwithparenchymalperivascularenhancementresponsivetosteroidsslippersdoesitreallyexist AT nobregapauloribeiro supratentoriallymphocyticinflammationwithparenchymalperivascularenhancementresponsivetosteroidsslippersdoesitreallyexist AT silvaguilhermediogo supratentoriallymphocyticinflammationwithparenchymalperivascularenhancementresponsivetosteroidsslippersdoesitreallyexist |