J Korean Soc Emerg Med.
2003 Aug;14(3):329-332.
A Case of Primary Angiitis of the Central Nervous System: A Case Presentation
- Affiliations
-
- 1Department of Emergency Medicine, University of Ulsan College of Medicine, Asan Medical Center, Hanil Clinic, Seoul, Korea. wkim@amc.seoul.kr
Abstract
- Primary angiitis of the central nervous system (PACNS) is a rare idiopathic disorder with recurrent vasculitis restricted to the central nervous system (CNS) and involves small-sized (and at time medium-sized) blood vessels. The typical clinical manifestations include multifocal strokes or encephalopathy, often associated with severe headaches. There are very few pediatric cases with PACNS reported in the literature. The diagnosis of PACNS is mainly based on its clinical features, demonstration of angiitis on angiography or biopsy, and exclusion of secondary causes of angiitis. A high index of suspicion is necessary for the diagnosis. Though the prognosis is generally poor, use of steroids and immunosuppressive therapy has been useful in achieving successful remission in many patients. We report the case of a patient with PACNS presenting recurrent headaches for 5 days.
Keyword
- Full Text Links
-
- Actions
-
Cited
- CITED
-
- Close
- Share
-
- Similar articles
-
- Childhood Primary Angiitis of the Central Nervous System Presenting as Isolated Middle Cerebral Artery Dissection
- Primary Angiitis of the Central Nervous System Presenting Tumefactive Lesions and Small Arteriolar Ectasias
- Necrotizing Primary Angiitis of the Central Nervous System Mimicking Brain Abscess: A Case Report and Literature Review
- Isolated Angiitis of Cntral Nervous System: A case Report
- Primary Angiitis of the Central Nervous System: Exclusion of Differentials and Long-Term Follow-Up
