Primary Angiitis of the Central Nervous System with Pathological Findings of Eosinophilic Granulomatosis with Polyangiitis

• Published in: Internal Medicine Vol. 63; no. 13; pp. 1939 - 1943
• Main Authors: Yamashita, Kimika, Miyazawa, Koichi, Murakami, Kazuhiro, Nakashima, Ichiro
• Format: Journal Article
• Language: English
• Published: Japan The Japanese Society of Internal Medicine 01-07-2024; Japan Science and Technology Agency
• Subjects: Aged; Biopsy; Brain - diagnostic imaging; Brain - pathology; brain biopsy; Central nervous system; Churg-Strauss syndrome; Churg-Strauss Syndrome - complications; Churg-Strauss Syndrome - diagnosis; Churg-Strauss Syndrome - pathology; EGPA; Eosinophilia; Female; Granulomatosis with Polyangiitis - complications; Granulomatosis with Polyangiitis - diagnosis; Granulomatosis with Polyangiitis - pathology; Humans; Leukocytes (eosinophilic); Magnetic Resonance Imaging; Neuroimaging; PACNS; Peripheral blood; Steroid hormones; Substantia alba; Vasculitis; Vasculitis, Central Nervous System - diagnosis; Vasculitis, Central Nervous System - diagnostic imaging; Vasculitis, Central Nervous System - pathology; brain biopsy; PACNS; EGPA
• ISSN: 0918-2918; 1349-7235; 1349-7235
• DOI: 10.2169/internalmedicine.2793-23

A 79-year-old woman presented with difficulty walking and disturbance of consciousness. Magnetic resonance imaging revealed diffuse white matter lesions and abnormal signals along the surface of the brain and sulci. A brain biopsy revealed granulomatous vasculitis with eosinophil infiltration. There was no peripheral blood eosinophilia or evidence of angiitis in other organs, and primary angiitis of the central nervous system (PACNS) with pathological findings of eosinophilic granulomatosis with polyangiitis (EGPA) was diagnosed. Steroids and other immunosuppressant therapies showed only limited effects. PACNS with pathological findings of EGPA is extremely rare, and a prompt brain biopsy is necessary for a diagnosis.