Hamad Mohammad, Mohammad Tala, Bishtawi Mira, Shahwan Randa, Jaradat Jaber H, Hazaymeh Wafa' A, Jbarah Omar F, Hazaimeh Ethar
Faculty of Medicine, University of Jordan, Amman, Jordan.
Medical researcher, Neuropedia for Training and Scientific Research, Amman, Jordan.
Neurol Sci. 2025 Jul 11. doi: 10.1007/s10072-025-08351-z.
Familial Mediterranean Fever (FMF) is the most common autoinflammatory disease worldwide, characterized by recurrent attacks of fever and polyserositis. Several studies have reported FMF cases with neurological conditions. As well as multiple neurological diseases have been reported in multiple studies in patients with FMF.
to summarize the frequency of neurologic manifestations reported among FMF patients.
PubMed, Web of Science, and SCOPUS were searched for studies that reported any neurological manifestation in FMF patients. Studies were screened first by abstract and then by full text, any conflict was resolved by a discussion. The JBI tool was used for assessing the risk of bias in studies. A meta-analysis of the proportions was performed for neurological manifestations in patients with FMF. A random effects model was indicated when I was greater than 50%, and a fixed model was used otherwise. R version 4.3.2 for performing the analysis.
Out of 787 citations, 36 studies (14 case reports, 22 others) were included with a total of 906 FMF patients (14 case reports, 892 others) with neurological manifestations. For case-report studies, the common manifestation was headache (42.9%) followed by Muscle weakness, diplopia (35.7%), Sensory disorders (35.7%) and facial nerve palsy (14.3%), The most common comorbid neurological condition was Multiple sclerosis (28.6%). Regarding Other study types, the Pooled prevalence of the most common manifestation was Neuropathy (0.45 [0.00; 0.99]), then Myalgia (0.27, 95%CI: [0.11; 0.47]), Headache (0.23, 95%CI: [0.12; 0.36]), and visual disorders (0.13 [0.01; 0.37]). The most common comorbid was ADHD (0.31, 95%CI: [0.25; 0.37]). Most studies were evaluated at a low risk of bias.
Nearly half of the patients in our review had Neuropathy in this review. ADHD was co-comorbid in 31% of patients. These findings underscore the importance of increased awareness and surveillance for these manifestations in patients with FMF. Further research is needed to better understand the pathogenesis of this manifestation.
家族性地中海热（FMF）是全球最常见的自身炎症性疾病，其特征为发热和多浆膜炎反复发作。多项研究报告了FMF患者合并神经系统疾病的病例。同时，在多项针对FMF患者的研究中也报告了多种神经系统疾病。
总结FMF患者中报告的神经学表现的频率。
在PubMed、科学网和Scopus数据库中检索报告FMF患者任何神经学表现的研究。首先通过摘要筛选研究，然后进行全文筛选，如有任何争议则通过讨论解决。使用JBI工具评估研究中的偏倚风险。对FMF患者的神经学表现进行比例的荟萃分析。当I大于50%时采用随机效应模型，否则使用固定效应模型。使用R版本4.3.2进行分析。
在787篇文献中，纳入了36项研究（14篇病例报告，22篇其他研究），共906例有神经学表现的FMF患者（14篇病例报告，892例其他研究）。对于病例报告研究，常见表现为头痛（42.9%），其次是肌肉无力、复视（35.7%）、感觉障碍（35.7%）和面神经麻痹（14.3%），最常见的合并神经疾病是多发性硬化（28.6%）。对于其他研究类型，最常见表现的合并患病率为神经病变（0.45 [0.00; 0.99]），其次是肌痛（0.27，95%置信区间：[0.11; 0.47]）、头痛（0.23，95%置信区间：[0.12; 0.36]）和视觉障碍（0.13 [0.01; 0.37]）。最常见的合并症是注意力缺陷多动障碍（ADHD）（0.31，95%置信区间：[0.25; 0.37]）。大多数研究的偏倚风险评估为低风险。
在本综述中，近一半的患者有神经病变。31%的患者合并ADHD。这些发现强调了提高对FMF患者这些表现的认识和监测的重要性。需要进一步研究以更好地理解这种表现的发病机制。
