Neurology Department, Hospital General Universitario Gregorio Marañón, 46 Doctor Esquerdo Street, 28007, Madrid, Spain.
Rheumatology Department, Hospital General Universitario Gregorio Marañón, 46 Doctor Esquerdo Street, 28007, Madrid, Spain.
Neurol Sci. 2024 Dec;45(12):5875-5887. doi: 10.1007/s10072-024-07681-8. Epub 2024 Jul 16.
Rheumatoid meningitis (RM) is an extremely rare extra-articular complication of rheumatoid arthritis (RA), with approximately 165 cases reported world-wide. RM exhibits a broad range of symptoms, with stroke-like episodes and seizures being the most common manifestations. The primary differential diagnoses include vascular and infectious diseases. The influence of immunomodulatory medications on the pathophysiology of RM remains unclear. There are no consensus guidelines on therapeutic regimen.
We present four patients with prior history of RA that developed different neurological syndromes in correlation to radiological leptomeningitis. Clinical presentations, comorbid conditions, supplementary diagnostic assessments, treatments, and prognosis are provided. A literature review of recent immunosuppressive management in RM patients was performed.
Three patients presented to hospital with recurrent focal seizures. Only two suffered meningism, reporting headache and fever. Magnetic resonance imaging (MRI) showed different grades of leptomeningitis across all cases. Notably, three cases demonstrated bilateral involvement extending to the pachymeninges. Two patients exhibited pronounced CSF mononuclear inflammation while extended microbiological evaluations yielded negative results. Two patients required biopsy for confirmation. The initiation of immunosuppressive therapy marked a turning point for three patients who previously exhibited progressive deterioration. Mortality was absent in all cases.
Our experience remarks the elusive nature of RM. Rigorous exclusionary diagnostics are imperative to differentiate RM from mimicking conditions. Clinical manifestations oscillate between transient episodes and progressive neurological impairments, punctuated by frequent epileptic seizures. In scenarios where clinical worsening persists or where clinical and radiological evaluations are inconclusive, aggressive immunosuppressive therapy is recommended.
类风湿性脑膜炎（RM）是类风湿关节炎（RA）的一种极罕见的关节外并发症，全球约有 165 例报告。RM 表现出广泛的症状，以中风样发作和癫痫发作最为常见。主要的鉴别诊断包括血管和感染性疾病。免疫调节药物对 RM 病理生理学的影响尚不清楚。对于治疗方案，目前没有共识指南。
我们介绍了 4 例有 RA 病史的患者，他们出现了不同的与放射学脑膜脑炎相关的神经综合征。提供了临床表现、合并症、补充诊断评估、治疗和预后。对 RM 患者最近免疫抑制治疗进行了文献复习。
3 例患者因反复发作性局灶性癫痫发作就诊。仅有 2 例出现脑膜炎体征，表现为头痛和发热。磁共振成像（MRI）显示所有病例均存在不同程度的脑膜脑炎。值得注意的是，3 例表现为双侧受累，延伸至硬脑膜。2 例患者表现出明显的 CSF 单核细胞炎症，而广泛的微生物学评估结果为阴性。为了确诊，有 2 例患者需要进行活检。免疫抑制治疗的开始对 3 例先前表现出进行性恶化的患者来说是一个转折点。所有病例均无死亡。
我们的经验表明 RM 具有隐匿性。为了将 RM 与类似疾病区分开来，严格的排他性诊断是必不可少的。临床表现在短暂发作和进行性神经损伤之间波动，常伴有癫痫发作。在临床恶化持续或临床和影像学评估不确定的情况下，建议积极进行免疫抑制治疗。
