واسکولیت اولیه مغزی، تجربه ده ساله در کلینیک روماتولوژی کودکان

Title

Childhood Primary Angeitis of CNS, Ten Years’ Experience in a Pediatric Rheumatology Clinic

English Abstract

Background: CNS vasculitis in children as a primary ascular inflammatory process is considered a novel clinical problem in pediatric rheumatology clinics. Methods: From 1996 to 2016 at pediatric rheumatology clinic, patients < 18 years of age were included to this study as having childhood primary angeitis of CNS (cPACNS). Including riteria were: clinical diagnosis of primary CNS vasculitis, and MRA findings demonstrating aneurism and or arterial stenosis not attributable to other causes. Results: 22 patients were enrolled with mean age 10 years, (54%) were female. Headache was in (88%) then mental disorder and seizure in (45%) were the most common neurologic symptoms. The mean delayed to diagnosis was 4 years. Fever was in (54%) and ANA in (31%) was positive. 63 %had abnormality in both MCA and ACA (14 patients), whereas PCA showed abnormity in 36% (8 patients). 86 % had normal EEG results. 22% suffered from severe and permanent neurological damage. Conclusion: Since cPACNS is unfamiliar and rare disorder, e recommend that in any patient with unexplained neurologic symptoms such as headache, seizure and mental disorder, CNS vasculitis should be considered as a probable background.

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