" /> Primary angiitis of the central nervous system - CISMeF





ICD-11 code : 4A44.7;

Preferred Label : Primary angiitis of the central nervous system;

ICD-11 definition : 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.;

ICD-11 synonym : PACNS - [primary angiitis of the central nervous system]; Primary vasculitis of the central nervous system; Isolated angiitis of the central nervous system;

ICD-11 acronym : PACNS;

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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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14/06/2025


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