MRI and clinicopathological correlation of childhood primary central nervous system angiitis.
AIM To review the long term clinical and imaging follow up of eight children with histopathologically proven childhood primary central nervous system angiitis (cPACNS) MATERIALS AND METHODS Eight children (5 17 years, five female and three male patients) with biopsy proven cPACNS vasculitis were reviewed retrospectively. All children were followed at the Children's Hospital of Eastern Ontario. Magnetic resonance imaging (MRI) images of the brain parenchyma and vessel pattern of the arteries of the circle of Willis were reviewed at baseline and follow up(s) All MRI images were correlated with clinical and histopathological findings. RESULTS Seven (87.5% children demonstrated parenchymal abnormalities: five of the seven (71.4% presented with non haemorrhagic lesions, one (14.3% presented with a parenchymal bleed, and one (14.3% showed diffuse oedema. One child remained negative for brain parenchymal abnormality at baseline and follow up. Of the seven children with a parenchymal abnormality, parenchymal enhancement was seen in two (28.5% and leptomeningeal enhancement was seen in one child. Only two children had positive diffusion weighted imaging (DWI) restriction at baseline. Five of the seven children (71.4% showed no magnetic resonance angiography (MRA) abnormalities at baseline. In one, MRA detected complete absence of flow. Follow up MRA (24 74 months) demonstrated that five of the seven (71.4% children had progressive paucity of the peripheral vascularity. Two children (28.5% with shorter term follow up (7 and 13 months) did not demonstrate MRA changes. CONCLUSION Long term follow up MRA demonstrated progressive paucity of peripheral vessels, which could be secondary to the inflammation affecting the peripheral vasculature causing reduction in vessel lumen size beyond the resolution of the maximum intensity projection (MIP) reconstruction.
Clinical radiology