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Neurosarcoidosis: Clinical, biological, and MRI presentation of central nervous system disease in a national multicenter cohort

dc.rights.licenseopenen_US
dc.contributor.authorDOS SANTOS, Amélie
dc.contributor.authorCOURTIN, Edouard
hal.structure.identifierNeurocentre Magendie : Physiopathologie de la Plasticité Neuronale [U1215 Inserm - UB]
dc.contributor.authorRUET, Aurélie
dc.contributor.authorDUFFAU, Pierre
dc.contributor.authorMATHEY, Guillaume
dc.contributor.authorBEKKOUR, Ines
dc.contributor.authorCIRON, Jonathan
dc.contributor.authorMICHEL, Laure
dc.contributor.authorBLANC, François-Xavier
dc.contributor.authorAGUILAR, Jésus
dc.contributor.authorLEJEUNE, Pascal
dc.contributor.authorMARC, Guillaume
dc.contributor.authorLAPLAUD, David
dc.contributor.authorMAGOT, Armelle
dc.contributor.authorHAMIDOU, Mohamed
dc.contributor.authorWIERTLEWSKI, Sandrine
dc.date.accessioned2025-04-30T09:10:25Z
dc.date.available2025-04-30T09:10:25Z
dc.date.issued2024-09
dc.identifier.urihttps://oskar-bordeaux.fr/handle/20.500.12278/206496
dc.description.abstractEnIntroduction: 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.
dc.language.isoENen_US
dc.rightsAttribution 3.0 United States*
dc.rights.urihttp://creativecommons.org/licenses/by/3.0/us/*
dc.subject.enNeurosarcoidosis
dc.subject.enSarcoidosis
dc.subject.enCentral Nervous System
dc.subject.enGranulomatous
dc.title.enNeurosarcoidosis: Clinical, biological, and MRI presentation of central nervous system disease in a national multicenter cohort
dc.title.alternativeBrain Behaven_US
dc.typeArticle de revueen_US
dc.identifier.doi10.1002/brb3.3443en_US
dc.subject.halSciences du Vivant [q-bio]/Neurosciences [q-bio.NC]en_US
dc.identifier.pubmed39279263en_US
bordeaux.journalBrain and Behavioren_US
bordeaux.page3443en_US
bordeaux.volume14en_US
bordeaux.hal.laboratoriesNeurocentre Magendie - U1215en_US
bordeaux.issue9en_US
bordeaux.institutionUniversité de Bordeauxen_US
bordeaux.institutionINSERMen_US
bordeaux.teamRelations glie-neuroneen_US
bordeaux.peerReviewedouien_US
bordeaux.inpressnonen_US
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hal.popularnonen_US
hal.audienceInternationaleen_US
hal.exporttrue
dc.rights.ccCC BYen_US
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