Amélie Dos santos, Edouard Courtin, Aurélie Ruet, Pierre Duffau, Guillaume Mathey, Ines Bekkour, Jonathan Ciron, Laure Michel, François Xavier Blanc, Jésus Aguilar, Pascal Lejeune, Guillaume Marc, David Laplaud, Armelle Magot, Mohamed Hamidou, Sandrine Wiertlewski
{"title":"神经肉芽肿病:全国多中心队列中中枢神经系统疾病的临床、生物学和磁共振成像表现","authors":"Amélie Dos santos, Edouard Courtin, Aurélie Ruet, Pierre Duffau, Guillaume Mathey, Ines Bekkour, Jonathan Ciron, Laure Michel, François Xavier Blanc, Jésus Aguilar, Pascal Lejeune, Guillaume Marc, David Laplaud, Armelle Magot, Mohamed Hamidou, Sandrine Wiertlewski","doi":"10.1002/brb3.3443","DOIUrl":null,"url":null,"abstract":"<div>\n \n \n <section>\n \n <h3> Introduction</h3>\n \n <p>Neurosarcoidosis (NS) is a systemic inflammatory granulomatous disease affecting of patients with sarcoidosis. Its diagnosis is difficult as there is no specific test for it. Because of its rarity, the management of NS has so far only been described in case series and short retrospective cohorts. The objective of this study is description of the clinical, paraclinical presentation and the therapeutic management of central nervous system (CNS) involvement in NS patients in France.</p>\n </section>\n \n <section>\n \n <h3> Methods</h3>\n \n <p>This multicenter, retrospective, observational study involved patients hospitalized between 2010 and 2019 with a diagnosis of sarcoidosis and CNS involvement.</p>\n </section>\n \n <section>\n \n <h3> Results</h3>\n \n <p>We included 118 patients (38 with isolated NS, 80 with NS associated with systemic sarcoidosis). NS was the initial presentation in 78% of patients, with cranial nerve involvement (36%), medullary symptoms (23%), and seizures (21%). Twenty-one percent of the patients had already been diagnosed with systemic sarcoidosis. The most frequent biological abnormality was lymphopenia (62.5%), while angiotensin-converting enzyme was increased in 21%. Meningitis was present in 45% and hyperproteinorachia in 69.5% of cases. MRI mainly revealed white matter abnormalities and leptomeningeal enhancement (34%). Corticosteroids were the most useful treatment, and immunosuppressive agents were used in steroid-resistant patients and to limit side effects. Methotrexate, cyclophosphamide, and anti-TNFα were also used, exhibiting good efficacy.</p>\n </section>\n \n <section>\n \n <h3> Conclusions</h3>\n \n <p>This cohort contributes to a better understanding of the clinical phenotype and associated imaging and biological abnormalities. Sharing of clinical, biological, and imaging data, as well as the therapeutic responses, of patients with NS helps to better understand and manage this disease that affects a small number of patients per center. A database project could be implemented in the future to enable this.</p>\n </section>\n </div>","PeriodicalId":9081,"journal":{"name":"Brain and Behavior","volume":null,"pages":null},"PeriodicalIF":2.6000,"publicationDate":"2024-09-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/brb3.3443","citationCount":"0","resultStr":"{\"title\":\"Neurosarcoidosis: Clinical, biological, and MRI presentation of central nervous system disease in a national multicenter cohort\",\"authors\":\"Amélie Dos santos, Edouard Courtin, Aurélie Ruet, Pierre Duffau, Guillaume Mathey, Ines Bekkour, Jonathan Ciron, Laure Michel, François Xavier Blanc, Jésus Aguilar, Pascal Lejeune, Guillaume Marc, David Laplaud, Armelle Magot, Mohamed Hamidou, Sandrine Wiertlewski\",\"doi\":\"10.1002/brb3.3443\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div>\\n \\n \\n <section>\\n \\n <h3> Introduction</h3>\\n \\n <p>Neurosarcoidosis (NS) is a systemic inflammatory granulomatous disease affecting of patients with sarcoidosis. 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Neurosarcoidosis: Clinical, biological, and MRI presentation of central nervous system disease in a national multicenter cohort
Introduction
Neurosarcoidosis (NS) is a systemic inflammatory granulomatous disease affecting of patients with sarcoidosis. Its diagnosis is difficult as there is no specific test for it. Because of its rarity, the management of NS has so far only been described in case series and short retrospective cohorts. The objective of this study is description of the clinical, paraclinical presentation and the therapeutic management of central nervous system (CNS) involvement in NS patients in France.
Methods
This multicenter, retrospective, observational study involved patients hospitalized between 2010 and 2019 with a diagnosis of sarcoidosis and CNS involvement.
Results
We included 118 patients (38 with isolated NS, 80 with NS associated with systemic sarcoidosis). NS was the initial presentation in 78% of patients, with cranial nerve involvement (36%), medullary symptoms (23%), and seizures (21%). Twenty-one percent of the patients had already been diagnosed with systemic sarcoidosis. The most frequent biological abnormality was lymphopenia (62.5%), while angiotensin-converting enzyme was increased in 21%. Meningitis was present in 45% and hyperproteinorachia in 69.5% of cases. MRI mainly revealed white matter abnormalities and leptomeningeal enhancement (34%). Corticosteroids were the most useful treatment, and immunosuppressive agents were used in steroid-resistant patients and to limit side effects. Methotrexate, cyclophosphamide, and anti-TNFα were also used, exhibiting good efficacy.
Conclusions
This cohort contributes to a better understanding of the clinical phenotype and associated imaging and biological abnormalities. Sharing of clinical, biological, and imaging data, as well as the therapeutic responses, of patients with NS helps to better understand and manage this disease that affects a small number of patients per center. A database project could be implemented in the future to enable this.
期刊介绍:
Brain and Behavior is supported by other journals published by Wiley, including a number of society-owned journals. The journals listed below support Brain and Behavior and participate in the Manuscript Transfer Program by referring articles of suitable quality and offering authors the option to have their paper, with any peer review reports, automatically transferred to Brain and Behavior.
* [Acta Psychiatrica Scandinavica](https://publons.com/journal/1366/acta-psychiatrica-scandinavica)
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* [Brain Pathology](https://publons.com/journal/1787/brain-pathology)
* [Child: Care, Health and Development](https://publons.com/journal/6111/child-care-health-and-development)
* [Criminal Behaviour and Mental Health](https://publons.com/journal/3839/criminal-behaviour-and-mental-health)
* [Depression and Anxiety](https://publons.com/journal/1528/depression-and-anxiety)
* Developmental Neurobiology
* [Developmental Science](https://publons.com/journal/1069/developmental-science)
* [European Journal of Neuroscience](https://publons.com/journal/1441/european-journal-of-neuroscience)
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* [GLIA](https://publons.com/journal/1287/glia)
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