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Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS Syndrome): A Case Report and Literature Review
CLIPPERS is a rare, chronic inflammatory neurological syndrome affecting multiple regions of the brain including the brainstem, cerebellum, and spinal cord. More than 100 cases have been documented globally since its initial description in 2010. This article reports the first case of the CLIPPERS sy...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Hindawi
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10382238/ https://www.ncbi.nlm.nih.gov/pubmed/37520771 http://dx.doi.org/10.1155/2023/5811243 |
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author | Shrestha, Nripesh Man Acharya, Niranjan Desar, Rabindra |
author_facet | Shrestha, Nripesh Man Acharya, Niranjan Desar, Rabindra |
author_sort | Shrestha, Nripesh Man |
collection | PubMed |
description | CLIPPERS is a rare, chronic inflammatory neurological syndrome affecting multiple regions of the brain including the brainstem, cerebellum, and spinal cord. More than 100 cases have been documented globally since its initial description in 2010. This article reports the first case of the CLIPPERS syndrome in Nepal. Clinical and radiological evidences of the patient lead to the diagnosis of this disease. Brain MRI reveals punctate and curvilinear gadolinium enhancement in the pons and cerebellum, which is diagnostic for the disease. Steroid therapy has been reported to be effective in treating CLIPPERS syndrome. Although its pathophysiology indicates an immune-mediated process, the etiology is yet unknown. The treatment and prognosis of this illness depend on an early and accurate diagnosis. |
format | Online Article Text |
id | pubmed-10382238 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Hindawi |
record_format | MEDLINE/PubMed |
spelling | pubmed-103822382023-07-29 Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS Syndrome): A Case Report and Literature Review Shrestha, Nripesh Man Acharya, Niranjan Desar, Rabindra Case Rep Neurol Med Case Report CLIPPERS is a rare, chronic inflammatory neurological syndrome affecting multiple regions of the brain including the brainstem, cerebellum, and spinal cord. More than 100 cases have been documented globally since its initial description in 2010. This article reports the first case of the CLIPPERS syndrome in Nepal. Clinical and radiological evidences of the patient lead to the diagnosis of this disease. Brain MRI reveals punctate and curvilinear gadolinium enhancement in the pons and cerebellum, which is diagnostic for the disease. Steroid therapy has been reported to be effective in treating CLIPPERS syndrome. Although its pathophysiology indicates an immune-mediated process, the etiology is yet unknown. The treatment and prognosis of this illness depend on an early and accurate diagnosis. Hindawi 2023-07-21 /pmc/articles/PMC10382238/ /pubmed/37520771 http://dx.doi.org/10.1155/2023/5811243 Text en Copyright © 2023 Nripesh Man Shrestha et al. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Shrestha, Nripesh Man Acharya, Niranjan Desar, Rabindra Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS Syndrome): A Case Report and Literature Review |
title | Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS Syndrome): A Case Report and Literature Review |
title_full | Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS Syndrome): A Case Report and Literature Review |
title_fullStr | Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS Syndrome): A Case Report and Literature Review |
title_full_unstemmed | Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS Syndrome): A Case Report and Literature Review |
title_short | Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS Syndrome): A Case Report and Literature Review |
title_sort | chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (clippers syndrome): a case report and literature review |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10382238/ https://www.ncbi.nlm.nih.gov/pubmed/37520771 http://dx.doi.org/10.1155/2023/5811243 |
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