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Updated: Jul 15, 2026
10:50
Visualizing Impairment of the Endothelial and Glial Barriers of the Neurovascular Unit during Experimental Autoimmune Encephalomyelitis In Vivo
Published on: March 26, 2019
Primary angiitis of the central nervous system
Eamonn S Molloy1, Rula A Hajj-Ali
1Rula A. Hajj-Ali, MD Center for Vasculitis Care and Research, Department of Rheumatic and Immunologic Diseases, Cleveland Clinic Foundation, 9500 Euclid Avenue, Desk A50, Cleveland, OH 44195, USA. hajjalr@ccf.org.
Current Treatment Options in Neurology
|April 21, 2007
Summary
Primary angiitis of the central nervous system (PACNS) requires careful diagnosis. Treatment varies by subtype, with GACNS and atypical PACNS needing immunosuppression, while BACNS is treated as RCVS.
Area of Science:
- Neurology
- Immunology
- Vascular Medicine
Background:
- Primary angiitis of the central nervous system (PACNS) is rare and lacks controlled trials.
- Differentiating PACNS from secondary CNS vasculitis and RCVS is crucial.
- PACNS is categorized into granulomatous (GACNS), benign (BACNS), and atypical subtypes.
Purpose of the Study:
- To outline diagnostic considerations and therapeutic strategies for PACNS subtypes.
- To differentiate PACNS from other cerebrovascular conditions.
- To guide treatment based on PACNS classification and disease severity.
Main Methods:
- Review of clinical, laboratory, angiographic, and pathological findings for PACNS classification.
- Analysis of treatment outcomes for different PACNS subsets.
- Comparison of PACNS with reversible cerebral vasoconstriction syndromes (RCVS).
Main Results:
- GACNS and atypical PACNS necessitate high-dose glucocorticoids and cyclophosphamide for GACNS, and initial glucocorticoids for atypical PACNS.
- PACNS mass lesions may resolve with immunosuppressive therapy, avoiding surgery.
- BACNS, now considered RCVS, is treated with calcium channel blockers and possibly short-term glucocorticoids, avoiding further immunosuppression.
Conclusions:
- Treatment for PACNS is subtype-dependent, emphasizing the distinction between GACNS, atypical PACNS, and BACNS/RCVS.
- Aggressive immunosuppression is vital for GACNS and atypical PACNS, while BACNS requires a different approach.
- Accurate diagnosis and classification are essential for effective management of PACNS and related disorders.
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