Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.

Early Predictors of Neurological Long-Term Deficits in Children with Primary CNS Vasculitis.

S. Soon,  Gordon, Yau,  Ivanna, Deveber,  Gabrielle, Armstrong,  Derek, Tyrrell,  Pascal, Templeton,  Jeffrey, Laughlin,  Suzanne


Childhood non-progressive primary CNS vasculitis (NPcPACNS) accounts for the majority of strokes in children. The aims of the study were 1) to report the neurological long-term outcome of NPcPACNS and 2) to determine early risk factors of long-term deficits in childhood vasculitis and stroke.


A single-centre cohort study of consecutive children diagnosed with cPACNS based on Calabrese criteria between January 1990 and December 2009 was performed. Patients were included if they 1) had non-progressive cPACNS (unilateral proximal stenoses and no evidence of progression >3 months) and 2) had serial clinical assessments and neuroimaging. The study excluded progressive cPACNS and angiography negative cPACNS. Data collection: 1) Demographics, preceding chickenpox; 2) Clinical characteristics, diffuse and focal neurological deficits recorded by two independent investigators, recurrence of ischemic events, 3) laboratory tests including inflammatory and prothrombotic markers and CSF analysis; 3) neuroimaging: serial Magnetic Resonance Imaging (MRI), MR angiography (MRA) and conventional angiography were blindly reviewed by two independent neuroradiologists following a previous developed protocol; 5) treatment with initial anticoagulation, antiplatelet and adjunctive immunosuppressive therapy. Outcome: Neurological deficit impacting on function as determined by Pediatric Stroke Outcome Measure (PSOM) at last follow-up. Analysis: putative early predictors of long-term deficits were tested in univariate and multivariable regression analyses.


A total of 44 children with NP-cPACNS were included; were 32 were boys and 12 were girls; median age at diagnosis was 5.5 years (range 0.5–16.4 years) and median follow-up was 6.4 years; 25 (57%) had preceding chickenpox <12 months. All children presented with arterial ischemic stroke, 28 (64%) had right-sided symptoms. Headache was seen in 17 (39%), 16 (36%) had diffuse deficits and only 6 (14%) seizures. Recurrent ischemic events were observed in 13 (30%). Inflammatory markers were elevated 23 (52%). All patient had unilateral ischemic MRI lesions, including 21 (48%) with isolated basal ganglia lesions. The majority (58%) had vessel stenoses of >50%. Initial therapy: Anticoagulation in 61%, antiplatelet in 31%, both in 6% and adjunctive corticosteroids in 13 (30%). Outcome: Neurological long-term deficits were seen in 20 (45%). Children presenting with seizures (p=0.006), evidence of MRI beyond the basal ganglia (p=0.001) and/or high degree of vascular stenosis (p=0.04) were at highest risk for long-term deficits.


The long-term outcome of childhood vascular stroke is devastating: Neurological deficits impacting on function can be found in 45% of children with non-progressive primary CNS vasculitis, the most common cause of childhood stroke. Early predictors including seizures at diagnosis, evidence of extensive MRI lesions and high degree of vascular stenosis can identify children at highest risk for long-term deficits. Innovative, tailored treatment strategies have to target high risk patients to prevent long-term neurological damage.

To cite this abstract, please use the following information:
S. Soon, Gordon, Yau, Ivanna, Deveber, Gabrielle, Armstrong, Derek, Tyrrell, Pascal, Templeton, Jeffrey, et al; Early Predictors of Neurological Long-Term Deficits in Children with Primary CNS Vasculitis. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :2019
DOI: 10.1002/art.29784

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