Article Text

PDF
Primary angiitis of the central nervous system: response to mycophenolate mofetil
  1. F Chenevier1,2,
  2. C Renoux1,2,
  3. R Marignier1,2,3,
  4. F Durand-Dubief1,2,
  5. M Hermier2,4,
  6. N Streichenberger2,5,
  7. S Vukusic1,2,3,
  8. C Confavreux1,2,3
  1. 1
    Service de Neurologie A and EDMUS Coordinating Center, Hôpital Neurologique Pierre Wertheimer, Hospices Civils de Lyon, Lyon, France
  2. 2
    Université de Lyon, Lyon, France
  3. 3
    Inserm U 842, Lyon, France
  4. 4
    Service de Neuroradiologie, Hôpital Neurologique Pierre Wertheimer, Hospices Civils de Lyon, Lyon, France
  5. 5
    Service de Neuropathologie, Hôpital Neurologique Pierre Wertheimer, Hospices Civils de Lyon, Lyon, France
  1. 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

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.

Statistics from Altmetric.com

Footnotes

  • Competing interests None.

  • Patient consent Obtained.

  • Provenance and Peer review Not commissioned; externally peer reviewed.

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.