Primary angiitis of the central nervous system: response to mycophenolate mofetil. Issue 10 (17th September 2009)

• Record Type: Journal Article
• Authors: Chenevier, F; Renoux, C; Marignier, R; Durand-Dubief, F; Hermier, M; Streichenberger, N; Vukusic, S; Confavreux, C
• Is Part Of: Journal of neurology, neurosurgery and psychiatry. Volume 80:Issue 10(2009)
• Page Start: 1159
• Page End: 1161
• Publication Date: 2009-09-17
• Subjects: Neurology -- Periodicals; Nervous system -- Surgery -- Periodicals; Psychiatry -- Periodicals; 616.8
• DOI: 10.1136/jnnp.2008.154567
• Languages: English
• ISSNs: 0022-3050

Abstract

Abstract : Primary angiitis of the central nervous system (PACNS) is a rare inflammatory disease restricted to the CNS of unknown cause. Clinical presentation and evolution are highly variable with potentially fluctuating signs and symptoms. Brain imaging often shows supratentorial ischaemic lesions. Definite diagnosis is established by brain biopsy. Treatment usually combines glucocorticosteroids and cyclophosphamide. A case of PACNS is reported here, which was proved by a brain biopsy and characterised by unusually prominent involvement of the posterior cerebral fossa. Successful treatment with mycophenolate mofetil in combination with steroids is described.