Diagnostic and Therapeutic Insights Into Pediatric Neurosarcoidosis: Observations From French Pediatric Rheumatology Centers

IF 3.2 3区医学 Q2 CLINICAL NEUROLOGY

Pediatric neurology Pub Date : 2024-10-18 DOI:10.1016/j.pediatrneurol.2024.10.007

Racha Tohme MD , Anca Tanase MD , Cécile Dumaine MD , Perrine Dusser MD , Homa Adle-Biassette MD, PhD , Veronique Despert MD , Albert Faye MD, PhD , Inès Mannes MD , Isabelle Melki MD, PhD , Isabelle Kone-Paut MD, PhD , Ulrich Meinzer MD, PhD , SOFREMIP, French Society for Rheumatology and Pediatric Inflammatory Diseases

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引用次数: 0

Abstract

Background

The diagnosis and management of neurosarcoidosis (NS) in pediatric patients remain challenging, with limited case documentation to guide clinicians. Most existing reports focus on initial presentations. This study aimed to outline the clinical features, management, and medium-term outcomes of pediatric NS

Methods

In this retrospective, multicentric, observational study, we collected data from pediatric patients followed in French pediatric rheumatology centers with a diagnosis of NS between January 2001 and June 2023.

Results

We identified 11 patients diagnosed with NS, comprising eight girls and three boys. The mean age at diagnosis of sarcoidosis was 10 (5 to 15) years, and the mean age of diagnosis of NS was 11.5 (5 to 17) years. Predominant neurological symptoms included headache (nine of 11 patients), papilledema (6 of 11 patients), facial palsy (two patients), seizures (one patient), and motor deficit (two patients). Nine of 11 patients had eye involvement, which consisted of granulomatous and bilateral uveitis. All patients exhibited meningitis, with cerebrospinal fluid white blood cell counts ranging from 6 to 70 cells/mm³. Six individuals presented neurological abnormalities on imaging, detailed in this study. Treatment primarily involved corticosteroids, methotrexate, and tumor necrosis factor alpha (TNF-alpha) inhibitors. Biologics targeting TNF-alpha were necessary to achieve remission in eight of 11 patients. In two patients who did not receive this treatment initially, it was required later in the course of evolution.

Conclusions

This study enhances understanding of the clinical course of pediatric NS and supports the early use of TNF-alpha biologics for improved management in affected children.

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小儿神经肉芽肿病的诊断和治疗见解：法国小儿风湿病中心的观察。

背景：儿科神经肉芽肿病（NS）的诊断和治疗仍具有挑战性，指导临床医生的病例资料有限。现有的大多数报告都集中在最初的表现上。方法：在这项回顾性、多中心观察研究中，我们收集了2001年1月至2023年6月期间在法国儿科风湿病学中心随访的诊断为NS的儿科患者的数据：我们发现了11名确诊为NS的患者，其中包括8名女孩和3名男孩。确诊为肉样瘤病的平均年龄为10（5至15）岁，确诊为NS的平均年龄为11.5（5至17）岁。主要的神经系统症状包括头痛（11 名患者中的 9 人）、乳头水肿（11 名患者中的 6 人）、面瘫（2 名患者）、癫痫发作（1 名患者）和运动障碍（2 名患者）。11 名患者中有 9 名眼部受累，包括肉芽肿性和双侧葡萄膜炎。所有患者均表现为脑膜炎，脑脊液白细胞计数从6到70个/立方毫米不等。六名患者在影像学检查中出现神经系统异常，详见本研究。治疗主要包括皮质类固醇、甲氨蝶呤和肿瘤坏死因子α（TNF-α）抑制剂。在11名患者中，有8名患者需要使用针对TNF-α的生物制剂才能获得缓解。有两名患者最初没有接受这种治疗，但在后来的演变过程中需要接受这种治疗：这项研究加深了人们对小儿NS临床病程的了解，并支持尽早使用TNF-α生物制剂来改善患儿的治疗。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Pediatric neurology 医学-临床神经学

CiteScore

4.80

自引率

2.60%

发文量

176

审稿时长

78 days

期刊介绍： Pediatric Neurology publishes timely peer-reviewed clinical and research articles covering all aspects of the developing nervous system. Pediatric Neurology features up-to-the-minute publication of the latest advances in the diagnosis, management, and treatment of pediatric neurologic disorders. The journal''s editor, E. Steve Roach, in conjunction with the team of Associate Editors, heads an internationally recognized editorial board, ensuring the most authoritative and extensive coverage of the field. Among the topics covered are: epilepsy, mitochondrial diseases, congenital malformations, chromosomopathies, peripheral neuropathies, perinatal and childhood stroke, cerebral palsy, as well as other diseases affecting the developing nervous system.