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Primary CNS vasculitis (PCNSV) is an uncommon disorder of unknown cause that is restricted to brain and spinal cord. Glucocorticoids alone or in combination with cyclophosphamide achieve a favorable response in most patients.1,2 However, some patients are intolerant or respond poorly to cyclophosphamide; therefore, there is the need for new treatment options. We report a patient with PCNSV who appeared to respond to treatment with corticosteroids and rituximab. This study was approved by the Mayo Clinic Institutional Review Board and written informed patient consent to perform the study was obtained.
Footnotes
Author contributions: Dr. Carlo Salvarani: acquisition and interpretation of the data, drafting/revising the manuscript for content. Dr. Robert D. Brown, Jr: drafting/revising the manuscript for content. Dr. Jonathan M. Morris: acquisition of the images, drafting/revising the manuscript for content. Dr. John Huston III: acquisition of the images, drafting/revising the manuscript for content. Dr. Gene G. Hunder: drafting/revising the manuscript for content.
Study funding: No targeted funding reported.
Disclosure: The authors report no disclosures relevant to the manuscript. Go to Neurology.org for full disclosures.
- Received September 22, 2013.
- Accepted in final form December 18, 2013.
- © 2014 American Academy of Neurology
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