Background/Purpose:

Childhood primary angiitis of the CNS (cPACNS) is an increasingly recognized, reversible cause of severe neurological and psychiatric deficit such as stroke, refractory seizures and hallucination in previously healthy children. Aggressive immunosuppression has significantly decreased the mortality, however little is known about the long-term neurocognitive burden of cPACNS. Objectibes: To describe the neuropsychological outcome of children with cPACNS, compare the cognitive burden between distinct disease subtypes and identify risk factors for poor neurocognitive outcome.

Methods:

A single-centre cohort study of children with cPACNS based on Calabrese criteria was performed. Children had to have completed a standard neurocognitive evaluation. Demographic characteristics, disease subtypes, clinical features, laboratory markers, neuroimaging studies, brain biopsy findings and treatment regimens were captured. Neurocognitive function was evaluated with a standardized battery of neuropsychological tests including domains of cognitive, social, emotional and adaptive function. The primary study outcome was the Full Scale IQ (FSIQ). Secondary outcomes included neurological function as defined by the Paediatric Stroke Outcome Measure PSOM, Health-related Quality of Life (PedsQL), disease activity and disease damage (VAS). Analysis: Univariate analysis compared variables between distinct disease subtypes; a multivariate prediction model for adverse cognitive outcome was used

Results:

A total of 104 children were diagnosed with primary CNS vasculitis in the study interval, of whom 63 (61%) completed the neuropsychological assessment. Of these 19 had small vessel CNS vasculitis (SVcPACNS) and 44 angiography-positive CNS vasculitis (APcPACNS). The cohort included 28 girls (16 SVcPACNS, 12 APcPACNS) and 35 boys (3 SVcPACNS, 32 APcPACNS), median age at diagnosis was 8.1 years; SVcPACNS patients were older at diagnosis (9.8 vs. 7.5 years). At diagnosis, SVcPACNS patients present significantly more commonly with seizures(79%, p<0.05) and acute behaviour change (47% p<0.05), while hemiparesis was more frequently seen in APcPACNS (91%,p<0.05). Outcome: Overall, the mean FSIQ was 93 (52-132). The mean FSIQ in SVcPACNS was 82 (54-119) and was significantly lower than in APcPACNS, 97 (52-132) (p<0.05). Abnormal FSIQ scores (<85) were seen in 53% of the SVcPACNS and 27% of APcPACNS patients. Children with SVcPACNS had lower scores than APcPACNS in the subdomains of verbal comprehension, working memory and processing speed (p<0.05). Prediction model: Children with SVcPACNS and with seizures were at the highest risk of cognitive deficits.

Conclusion:

Children with cPACNS carry significant disease burden. The inflammation causes drastically impaired cognitive functioning including comprehension, processing speed and memory deficits. Children with SVcPACNS and in particular those presenting with seizures are at the highest risk for adverse cognitive outcome. Early rehabilitation interventions have to be tailored to this high risk group.

---

« Back to 2012 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/burden-of-childhood-central-nervous-system-vasculitis-identifying-high-risk-factors-for-poor-cognitive-outcome/