Investigation of childhood central nervous system vasculitis: magnetic resonance angiography versus catheter cerebral angiography

AIM We compared the clinical utility of magnetic resonance angiography (MRA) to catheter cerebral angiography (CA) in the investigation of children with suspected central nervous system (CNS) vasculitis. METHOD Single-centre retrospective review of children with a suspected diagnosis of CNS vasculitis studied with both MRA and CA. MRA and CA-detected abnormalities (location, multiplicity, and morphology) were compared; sensitivity and specificity were calculated on a per lesion and per patient basis for MRA, with CA as the reference standard. RESULTS Findings in fourteen patients (median age at presentation of 5y 10mo [range 1y 5mo-14y 5mo]; eight males, six females) relating to sixteen paired studies of MRA and CA were reviewed. CA-detected lesions were commonly bilateral (13/16 studies, p<0.05), and likely to be proximally distributed (15/16 studies, p<0.05). The sensitivity and specificity of MRA for CA lesion detection was 63% (95% confidence interval [CI] 48-78) and 89% (95% CI 81-93), respectively with moderate agreement between the two modalities (kappa=0.51, 95% CI 0.37-0.66). The majority of the false negative observations involved the posterior circulation (9/14). The overall sensitivity for MRA diagnosis of vasculitis per patient was 94% (95% CI 67-99). INTERPRETATION MRA failed to identify all lesions detected on CA, particularly those in the posterior circulation. MRA is a reasonable initial modality in the investigation of suspected CNS vasculitis but in cases of abnormal parenchymal MRI and normal MRA, CA should be considered.