Primary Angiitis of the Central Nervous System in Children.

Lee,  Yun Jin; Kim,  Young Mi; Nam,  Sang Ook; Kim,  Hack Jin

Case Report

Journal of the Korean Child Neurology Society 2003;11(2):356-361.
Published online November 30, 2003.

Primary Angiitis of the Central Nervous System in Children.

Yun Jin Lee, Young Mi Kim, Sang Ook Nam, Hack Jin Kim

¹Department of Pediatrics, College of Medicine, Pusan National University, Busan, Korea. weareone@pusan.ac.kr
²Department of Radiology, College of Medicine, Pusan National University, Busan, Korea.

Abstract

Primary angiitis of the CNS(PACNS), also known as isolated angiitis of the CNS, is an idiopathic vasculitis confined to the CNS and it primarily occurrs to middle aged adults. Neurologic manifestations include headache, focal seizure, and progressive, behavioral or multifocal neurologic impairments. A 20-month-old boy was presented with recurrent right-sided hemiplegia and loss of consciousness. Brain MRI revealed focal T2 hyperintensely enhanced lesions involving left lentiform nucleus and caudate nucleus with no mass effects. Cerebral angiogram disclosed multifocal stenosis in both middle cerebral arteries and left posterior cerebral artery. The child was treated with prednisone alone since he was diagnosed as IACNS. He was asymptomatic with a normal neurologic examination and continued on prednisone therapy. Therefore, it is concluded that children who have frequent or severe headache or focal neurologic deficits should be carefully evaluated and those meeting criteria of IACNS should be treated aggressively.

Key Words: Primary angiitis of the central nervous system, Children