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Neurologic manifestations in familial Mediterranean fever
1Pediatric Rheumatology Unit, Soroka Medical Center, Ben Gurion University of the Negev Beer Sheva, Israel.
Insights
Neurologic complications in children with familial Mediterranean fever (FMF) are uncommon but can occur. Headaches are most frequent, while meningitis and convulsions are rare but serious manifestations of FMF.
Area of Science:
- Pediatrics
- Neurology
- Rheumatology
Background:
- Familial Mediterranean fever (FMF) is a genetic autoinflammatory disorder.
- Neurologic manifestations in pediatric FMF are infrequently documented.
Observation:
- A study reviewed 101 children diagnosed with FMF.
- Thirteen patients (13%) exhibited neurologic symptoms.
Findings:
- Headaches were the most common neurologic issue, affecting 10 of the 13 patients during FMF flares.
- Two patients experienced febrile seizures before age 5, recurring later.
- One patient presented with aseptic meningitis and drug-resistant convulsions, which resolved with colchicine treatment.
Implications:
- The study highlights the importance of considering neurologic involvement in children with FMF.
- Early recognition and management of these rare complications are crucial for patient outcomes.
- This underscores the systemic nature of FMF beyond its typical gastrointestinal and rheumatologic symptoms.
Abstract:
Neurologic involvement in children with familial Mediterranean fever is relatively uncommon and rarely described in the pediatric literature. Although headaches occur frequently, meningitis and convulsions are rare. Thirteen of 101 children with familial Mediterranean fever developed neurologic manifestations. Of these 13 patients, 10 had headaches during acute episodes of the fever. Two patients had convulsions with fever before the age of 5 years; the convulsions and acute episodes recurred at ages 9 and 10 years. Another patient had two episodes of aseptic meningitis followed by convulsive disorder before the diagnosis of familial Mediterranean fever was made; his convulsions were resistant to antiepileptic drugs alone and subsided only when colchicine was added. The possibility of neurologic involvement should be considered in patients with familial Mediterranean fever.