title: Vessel wall enhancement in the diagnosis and management of primary angiitis of the central nervous system in children.
abstract: We describe two cases of primary angiitis of the central nervous system in children (cPACNS) diagnosed by vessel wall contrast enhancement on magnetic resonance imaging (MRI). Both patients developed acute cerebral infarction after fever and malaise. In patient 1, a 7-month-old boy, MRI revealed extensive cerebral infarction in the right middle cerebral artery (MCA) area and stenosis at the M1 portion of the right MCA. Oral glucocorticoid therapy was initiated. Vessel wall enhancement was ameliorated 3months after onset, and stenosis was mostly restored. Patient 2, a 5-year-old boy, suffered from cerebral infarction in the left MCA area, and stenosis was identified in the left internal carotid artery, left MCA, and left posterior cerebral artery. Although vessel wall enhancement was reduced after glucocorticoid therapy, vessel wall enhancement of left MCA re-emerged, accompanied by increased erythrocyte sedimentation rate (ESR) and, decreased cerebral blood flow (CBF) in the affected hemisphere. Intravenous methylprednisolone therapy followed by oral glucocorticoid and mycophenolate mofetil resulted in resolution of these findings. Vessel wall enhancement is a promising finding in the diagnosis of cPACNS. Disease flares occur rarely in medium-to-large vessel cPACNS during dose tapering. Vessel wall enhancement, ESR, and CBF may be useful for the assessment of the activity of angiitis.
authors: Division of Child Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, Yonago, Japan. Electronic address: oonok@med.tottori-u.ac.jp.
authors: Koyo
authors: K
authors: Ohno
authors: Division of Child Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, Yonago, Japan.
authors: Yoshiaki
authors: Y
authors: Saito
authors: Division of Child Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, Yonago, Japan.
authors: Hirofumi
authors: H
authors: Kurata
authors: Division of Child Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, Yonago, Japan.
authors: Yusuke
authors: Y
authors: Saiki
authors: Department of Pediatrics, Yasugi Municipal Hospital, Yasugi, Japan.
authors: Hiroko
authors: H
authors: Ohtahara
authors: Division of Neurosurgery, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, Yonago, Japan.
authors: Hiroki
authors: H
authors: Yoshioka
authors: Division of Radiology, Faculty of Medicine, Tottori University, Yonago, Japan.
authors: Eijiro
authors: E
authors: Yamashita
authors: Division of Radiology, Faculty of Medicine, Tottori University, Yonago, Japan.
authors: Shinya
authors: S
authors: Fujii
authors: Division of Child Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, Yonago, Japan.
authors: Yoshihiro
authors: Y
authors: Maegaki
mesh_terms: D002533:Cerebral Angiography / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D006801:Humans; D018810:Magnetic Resonance Angiography / Q000379:methods*; D008297:Male; D020293:Vasculitis, Central Nervous System / Q000000981:diagnostic imaging* / Q000188:drug therapy
keywords: Medium-to-large vessel angiitis of the CNS; Primary angiitis of the central nervous system (PACNS); Vessel wall enhancement