Primary angiitis of the central nervous system

Molloy, Eamonn S.; Hajj-Ali, Rula A.

doi:10.1007/bf02938406

Cited by 39Vasculitis and related conditionsNeurological Complications and SyndromesIgG4-Related and Inflammatory Diseases

Primary angiitis of the central nervous system

Molloy, Eamonn S., Hajj-Ali, Rula A.·2007·Current Treatment Options in Neurology

DOI 10.1007/bf02938406

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Abstract

Primary angiitis of the central nervous system (PACNS) is a rare disorder that must be differentiated from secondary CNS vasculitides, reversible cerebral vasoconstriction syndromes, and other vascular disorders. Because of the rarity of PACNS, no controlled trials have been performed, thereby precluding an evidence-based approach to therapy. PACNS has been considered to have three major subsets, as defined by clinical, laboratory, angiographic, and pathologic findings: granulomatous angiitis of the central nervous system (GACNS), benign angiopathy of the central nervous system (BACNS), and atypical PACNS. Approximately 5% of cases may present as a tumor-like mass lesion. Because the subset of patients with GACNS has a more guarded prognosis, treatment with high-dose glucocorticoids and cyclophosphamide is necessary. All patients with atypical PACNS should be treated with high-dose glucocorticoids initially. The use of additional immunosuppressive agents depends on the severity of the initial presentation, the ability to achieve remission with glucocorticoid monotherapy, and the occurrence of relapse upon glucocorticoid tapering. PACNS mass lesions can regress completely with immunosuppressive therapy, and therefore, surgical excision may be avoided. Patients with BACNS should be treated with a calcium channel blocker (eg, verapamil) with or without a short course of glucocorticoids. These patients should not receive additional immunosuppressive therapies such as cyclophosphamide. This reflects the fact that BACNS is now more correctly considered as a reversible cerebral vasoconstriction syndrome (RCVS) and not as a true vasculitis. However, a patient initially diagnosed with RCVS that demonstrates incomplete resolution of angiographic findings after 3 to 4 months should be re-evaluated for alternative diagnoses, including PACNS.

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Authors: Molloy, E. S., & Hajj-Ali, R. A.
Affiliations: Cleveland Clinic (US)
Venue: Current Treatment Options in Neurology
Publisher: Springer Science and Business Media LLC
Published: 2007-05
Volume: 9
Issue: 3
Pages: 169-75
ISSN: 1092-8480 (ISSN-L)
1534-3138
DOI: 10.1007/bf02938406
OpenAlex: W2087914957
Cited by: 39
References: 60
Citations by year: 2025: 2 · 2024: 2 · 2023: 1 · 2021: 2 · 2019: 3 · 2017: 2 · 2016: 4 · 2015: 2 · 2014: 5 · 2013: 5 · 2012: 3
FWCI: 1.43
Type: journal article
Language: en
Open access: closed
Licence: www.springer.com/tdm (tdm)
www.springer.com/tdm (vor)
Locations: Current Treatment Options in Neurology (publishedVersion)
PubMed (publishedVersion)
Topics: Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Vasculitis and related conditions
Health Sciences › Medicine › Psychiatry and Mental health › Neurological Complications and Syndromes
Health Sciences › Medicine › Rheumatology › IgG4-Related and Inflammatory Diseases
Keywords: primary angiitis of the central nervous system, reversible cerebral vasoconstriction syndrome, glucocorticoids, cyclophosphamide, calcium channel blockers, verapamil, immunosuppressive therapy, differential diagnosis, prognosis

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