4A44.7 Primary angiitis of the central nervous system

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01


In primary angiitis of the central nervous system, vasculitis is limited to the central nervous system. Primary angiitis of the central nervous system is a very rare disease, and its manifestation can be mimicked by many other diseases. Patients with primary angiitis commonly show headache, waxing and waning altered mental status, and transient ischemic attack-like events. Diagnosis is often based on angiography, although brain biopsy remains the only definitive diagnostic test.

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