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Neurological Face of Familial Mediterranean Fever
Polat Cengiz Bektaş1, Aslı Kavas Tufan2, Nuran Çetin2
1Department of Pediatrics, Eskişehir Osmangazi University, Faculty of Medicine, Eskişehir, Turkey.
Objective:
Familial Mediterranean fever is a systemic inflammatory disease characterized by recurrent attacks in the form of fever and inflammation of serous membranes. We aimed to search for neurological signs and symptoms of children with familial Mediterranean fever.
Materials And Methods:
Medical records database from 2010 to 2020 was screened retrospectively. In total, 625 children with familial Mediterranean fever were included in the study. Neurological symptoms and associated factors were searched.
Results:
The mean age at onset of familial Mediterranean fever symptoms and time to diagnosis was calculated as 5.12 ± 3.51 years and 7.27 ± 3.9 years, respectively. The neurological symptoms were present in 142 (23.5%) patients. Headache was the most common symptom. During follow-up, different neurologic diseases were diagnosed in 40 familial Mediterranean fever patients and epilepsy was the most frequent disease. The coexistent disease was present in 49.9% of children with familial Mediterranean fever. Juvenile idiopathic arthritis was found to be a risk factor for the neurologic symptom (P < .05). The frequency of neurological symptoms was higher in patients with E148Q mutation (P < .012).
Conclusion:
The results of the present study revealed that patients with familial Mediterranean fever can present with various central nervous system manifestations. A multidisciplinary approach must be considered in the treatment of these children.
Insights
Familial Mediterranean Fever (FMF) can cause neurological symptoms in children, with headache being most common. Early diagnosis and a multidisciplinary approach are crucial for managing these central nervous system manifestations.
Area of Science:
- Pediatric Rheumatology
- Pediatric Neurology
- Systemic Inflammatory Diseases
Background:
- Familial Mediterranean Fever (FMF) is a genetic autoinflammatory disorder.
- FMF is characterized by recurrent fever and serosal inflammation.
- Neurological manifestations in pediatric FMF are not well-documented.
Purpose of the Study:
- To investigate the prevalence and types of neurological signs and symptoms in children diagnosed with FMF.
- To identify factors associated with neurological involvement in pediatric FMF patients.
Main Methods:
- Retrospective analysis of medical records from 2010-2020.
- Inclusion of 625 pediatric patients diagnosed with FMF.
- Systematic search for neurological symptoms and coexisting conditions.
Main Results:
- Neurological symptoms were observed in 23.5% of pediatric FMF patients, with headache being the most frequent.
- Epilepsy was the most common neurological disease diagnosed during follow-up.
- Juvenile idiopathic arthritis and the E148Q mutation were identified as risk factors for neurological symptoms.
Conclusions:
- Pediatric patients with FMF can exhibit diverse central nervous system manifestations.
- A comprehensive, multidisciplinary approach is recommended for managing neurological complications in pediatric FMF.
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