Primary angiitis of the central nervous system: response to mycophenolate mofetil
- F Chenevier1,2,
- C Renoux1,2,
- R Marignier1,2,3,
- F Durand-Dubief1,2,
- M Hermier2,4,
- N Streichenberger2,5,
- S Vukusic1,2,3,
- C Confavreux1,2,3
- 1Service de Neurologie A and EDMUS Coordinating Center, Hôpital Neurologique Pierre Wertheimer, Hospices Civils de Lyon, Lyon, France
- 2Université de Lyon, Lyon, France
- 3Inserm U 842, Lyon, France
- 4Service de Neuroradiologie, Hôpital Neurologique Pierre Wertheimer, Hospices Civils de Lyon, Lyon, France
- 5Service de Neuropathologie, Hôpital Neurologique Pierre Wertheimer, Hospices Civils de Lyon, Lyon, France
- Correspondence to Professor C Confavreux, Service de Neurologie A and the European Database for Multiple Sclerosis Coordinating Centre, Hôpital Neurologique Pierre Wertheimer, 59 Boulevard Pinel, 69677 Lyon-Bron cedex, France; christian.confavreux{at}chu-lyon.fr
- Received 4 June 2008
- Revised 29 October 2008
- Accepted 30 October 2008
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.
Footnotes
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Competing interests None.
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Patient consent Obtained.
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Provenance and Peer review Not commissioned; externally peer reviewed.
