Prevalence of Neurological Manifestation in Familial Mediterranean Fever Patients: Systematic Review & Meta-Analysis
Mohammad Hamad1, Tala Mohammad1, Mira Bishtawi2, Randa Shahwan2, Jaber H. Jaradat3, Abdul Jaber Tayem4, Omar F. Jbarah5, Ethar Hazaimeh5
1Faculty of Medicine, University of Jordan, 2Faculty of Medicine, Al Balqa Applied University, 3Faculty of Medicine, Mutah University, 4Cedars Sinai Medical Center, 5Neuropedia for Training and Scientific Research
Objective:

summarize the frequency of neurologic manifestations reported among FMF patients


Background:

Familial Mediterranean Fever(FMF) is the most common hereditary autoinflammatory disease worldwide, characterised 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.


Design/Methods:

Databases were searched for studies that reported neurological manifestation in FMF patients. Studies were screened by abstract and then by full text, any conflict was resolved by a discussion. 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. Random effects model was indicated when I2 was greater than 50%, otherwise fixed model was used. R version 4.3.2 for performing the analysis


Results:

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%), 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, 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.


Conclusions:

2 out of 50 patients with FMF had at least 1 neurological manifestation. Nearly half of the patients in our review had Neuropathy. 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 understand the pathogenesis of this manifestation better.

10.1212/WNL.0000000000208372
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