دورية أكاديمية
أعرض في EDS

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

التفاصيل البيبلوغرافية
العنوان: Neurosarcoidosis: Clinical, biological, and MRI presentation of central nervous system disease in a national multicenter cohort.
المؤلفون: Dos Santos A; Service de Neurologie, CRC-SEP, CHU Poitiers, Poitiers Université, POITIERS, France., Courtin E; Service de Neurologie, CHU Bordeaux, Bordeaux Université, Bordeaux, France., Ruet A; Service de Neurologie, CHU Bordeaux, Bordeaux Université, Bordeaux, France., Duffau P; Service de Médecine interne, CHU Bordeaux, Bordeaux Université, Bordeaux, France., Mathey G; Service de Neurologie, CHU Nancy, Nancy Université, Nancy, France., Bekkour I; Service de Neurologie, CHU Nancy, Nancy Université, Nancy, France., Ciron J; Service de Neurologie, CHU Toulouse, Toulouse Université, Toulouse, France., Michel L; Service de Neurologie, CHU Rennes, Rennes Université, Rennes, France., Blanc FX; Service de Pneumologie, L'institut du Thorax, CHU Nantes, Nantes Université, Nantes, France., Aguilar J; Service de Neuroradiologie, CHU Nantes, Nantes Université, Nantes, France., Lejeune P; CHD La Roche sur Yon, Service de Neurologie, La Roche sur Yon, France., Marc G; CHD de St Nazaire, Service de Neurologie, St. Nazaire, France., Laplaud D; Service de Neurologie, Institut du Thorax et du Système Nerveux, CRC-SEP, CHU Nantes, Nantes Université, Nantes, France.; CR2TI, INSERM U1064, Nantes, France., Magot A; CHU Nantes, Service des explorations fonctionnelles neurologiques, Nantes Université, Nantes, France., Hamidou M; CHU Nantes, Service de Médecine Interne, Nantes Université, Nantes, France., Wiertlewski S; Service de Neurologie, Institut du Thorax et du Système Nerveux, CRC-SEP, CHU Nantes, Nantes Université, Nantes, France.; CR2TI, INSERM U1064, Nantes, France.
المصدر: Brain and behavior [Brain Behav] 2024 Sep; Vol. 14 (9), pp. e3443.
نوع المنشور: Journal Article; Multicenter Study; Observational Study
اللغة: English
بيانات الدورية: Publisher: John Wiley & Sons Country of Publication: United States NLM ID: 101570837 Publication Model: Print Cited Medium: Internet ISSN: 2162-3279 (Electronic) NLM ISO Abbreviation: Brain Behav Subsets: MEDLINE
أسماء مطبوعة: Original Publication: Hoboken, NJ : John Wiley & Sons
مواضيع طبية MeSH: Sarcoidosis*/diagnostic imaging , Sarcoidosis*/drug therapy , Central Nervous System Diseases*/diagnostic imaging , Central Nervous System Diseases*/drug therapy , Magnetic Resonance Imaging*, Humans ; Male ; Female ; Middle Aged ; Adult ; Retrospective Studies ; France ; Aged ; Immunosuppressive Agents/therapeutic use ; Adrenal Cortex Hormones/therapeutic use
مستخلص: 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.
(© 2024 The Authors. Brain and Behavior published by Wiley Periodicals LLC.)
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فهرسة مساهمة: Keywords: central nervous system; granulomatous; neurosarcoidosis; sarcoidosis
المشرفين على المادة: 0 (Immunosuppressive Agents)
0 (Adrenal Cortex Hormones)
SCR Disease Name: Neurosarcoidosis
تواريخ الأحداث: Date Created: 20240916 Date Completed: 20240916 Latest Revision: 20240924
رمز التحديث: 20240924
مُعرف محوري في PubMed: PMC11403122
DOI: 10.1002/brb3.3443
PMID: 39279263
قاعدة البيانات: MEDLINE
الوصف
تدمد:2162-3279
DOI:10.1002/brb3.3443