PReS-FINAL-2362: The spectrum of childhood inflammatory brain diseases; an increasingly recognised field

Abstract

Background/Purpose: Childhood Inflammatory Brain diseases are potentially life-threatening diseases leading to severe neurological deficits if not treated. The spectrum of childhood inflammatory brain diseases is rapidly expanding, and recent evidence shows the presence of inflammatory markers in previously thought to be vasculopathies, such as Moyamoya. We reviewed all pediatric patients enrolled in the international BrainWorks database. Methods: All pediatric patients enrolled through a BrainWorks center were identified in the BrainWorks database. Patients were eligible for inclusion if predetermined information at the baseline visit was available. This included, information on demographic information, such as age at diagnosis and gender, diagnosis at enrollment, clinical, laboratory, neuroradiology and if applicable brain biopsy. Results: In total 370 (190 boys, 180 girls) children were enrolled in BrainWorks with complete dataset at baseline. The mean age at diagnosis was 9.33 years (boys 9.67 years, girls 9.07 years). The diagnosis included were; Non-progressive large vessel CNS vasculitis n=108 (68 boys, 40 girls, mean age 8.15 years), Small vessel CNS vasculitis n = 76 (26 boys, 50 girls, mean age 11 years), Progressive large vessel CNS vasculitis n=25 (17 boys, 8 girls, mean age 10.3 years), NMDAR-encephalitis n=42 (15 boys, 27 girls, mean age 9.9 years), secondary CNS vasculitis due to underlying systemic disease n=33 (16 boys, 17 girls), and Moyamoya n=33 (18 boys, 15 girls). Other diagnosis included CNS vasculitis due to infection n=20, ADEM n=5, MS n=2, Rasmussen n=2, and other vasculopathies/vasculitis (n-21). Focal deficits at presentation were more commonly seen in patients with large vessel CNS vasculitis and infantile Moyamoya, while diffuse deficits were seen more in children with small vessel CNS vasculitis and NMDAR encephalitis. Seizures were seen in all inflammatory brain diseases, but were more frequently present in NMDAR encephalitis and small vessel CNS vasculitis (80% and 61%) compared to the large vessel CNS vasculitis subtypes and Moyamoya. Conclusion: Childhood inflammatory Brain diseases encompasses many different diagnosis. The most frequent diagnosis are the different subtypes of childhood CNS vasculitis, however NMDAR encephalitis and Moyamoya are increasingly recognized and diagnosed.