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Background <p>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.</p> Aims <p>to summarize the frequency of neurologic manifestations reported among FMF patients.</p> Methods <p>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<sup>2</sup> was greater than 50%, and a fixed model was used otherwise. R version 4.3.2 for performing the analysis.</p> Results <p>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.</p> Conclusion <p>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.</p>

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Prevalence of neurological manifestation in familial mediterranean fever patients: systematic review & meta-analysis

  • 期刊论文

  • 发表日期：

    2025年7月11日

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  • Mohammad Hamad,
  • Tala Mohammad,
  • Mira Bishtawi,
  • Randa Shahwan,
  • Jaber H. Jaradat,
  • Wafa’ A. Hazaymeh,
  • Omar F. Jbarah,
  • Ethar Hazaimeh

摘要

Background

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.

Aims

to summarize the frequency of neurologic manifestations reported among FMF patients.

Methods

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 I2 was greater than 50%, and a fixed model was used otherwise. R version 4.3.2 for performing the analysis.

Results

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.

Conclusion

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.