J Korean Child Neurol Soc.  2003 Nov;11(2):356-361.

Primary Angiitis of the Central Nervous System in Children

• Lee YJ
• Kim YM
• Nam SO
• Kim HJ

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.

Keyword

Primary angiitis of the central nervous system; Children

MeSH Terms

Adult
Brain
Caudate Nucleus
Central Nervous System*
Child*
Constriction, Pathologic
Corpus Striatum
Headache
Hemiplegia
Humans
Infant
Magnetic Resonance Imaging
Male
Middle Aged
Middle Cerebral Artery
Neurologic Examination
Neurologic Manifestations
Posterior Cerebral Artery
Prednisone
Seizures
Unconsciousness
Vasculitis*
Prednisone