اسكولیت مغزی كودكان يك چالش روماتولوژیک جدید، گزارش سری موردی

Title

CHILDHOOD CNS VASCULITIS A NOVEL CHALLENGE IN PEDIATRIC RHEUMATOLOGY CLINIC, A CASE SERIES STUDY

English Abstract

Primary CNS vasculitis of childhood (cPACNS) is an inflammatory vascular process of CNS. It is considered a very novel and challenging clinical problem in pediatric rheumatology and neurology clinic. From 1994 to 2014 at pediatric rheumatology clinic, patients <18 years of age were included to this study as having cPACNS if they had: a clinical diagnosis of primary CNS vasculitis, and MRA findings demonstrating arterial stenosis and or aneurism not attributable to other causes. 22 patients were enrolled this study with mean age 10 years, 12 patients (54%) were female. The mean delayed time to diagnosis was 4 years. The most common neurologic symptoms were headache (88%) then seizure and mental disorder in (45%). Fever was in (54%) and positive ANA result in (31%) patients. 14 patients (63 %) showed abnormality in both MCA and ACA, whereas PCA showed this abnormity in 36% (8) patients. Most of patients 86 %( 19) had normal EEG findings. Five patients (22%) had severe and permanent neurological sequel. It seems cPACNS is unfamiliar disorder and we recommend that in any patient with unexplained headache, seizure and mental disorder CNS vasculitis should be considered.

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