Primary Angiitis of the Central Nervous System in Children.
- Author:
Yun Jin LEE
1
;
Young Mi KIM
;
Sang Ook NAM
;
Hack Jin KIM
Author Information
1. Department of Pediatrics, College of Medicine, Pusan National University, Busan, Korea. weareone@pusan.ac.kr
- Publication Type:Case Report
- Keywords:
Primary angiitis of the central nervous system;
Children
- MeSH:
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*
- From:
Journal of the Korean Child Neurology Society
2003;11(2):356-361
- CountryRepublic of Korea
- Language:Korean
-
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.
