Meet the Challenge of Primary CNS Vasculitis

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Table 4: Treatment and Outcome of Different PCNSV Subsets

There is emerging evidence that therapy of PCNSV can differ among patients and that a uniform approach is not indicated (see Table 4). Relapses or recurrences were recorded in only 26% of the Mayo Clinic series.¹ Patients with relapsing disease required longer therapy but otherwise had outcomes similar to those without relapses.

Therapy appears to be associated with a favorable outcome in most patients. In the Mayo Clinic series, most patients who had low disability at the time of diagnosis continued to have low disability at last follow-up; in contrast, most of the 22 patients with severe disability at diagnosis had less disability at follow-up.¹

Serial MRI and MRA (four to six weeks after the beginning of treatment, then every three to four months during the first year of treatment, or in case of new neurological deficit), as well as repeat careful neurological examinations are useful to monitor the disease course. In patients with stable imaging but worsening clinical symptoms, a repeat spinal fluid exam and repeat angiography may be indicated.

Our understanding of PCNSV and the delineation of its spectrum and subsets has advanced, but further study is needed to clarify methods of diagnosis and optimal management.

Acknowledgments

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Table 5: Discriminating Features of PCNSV and RVCS

We thank Drs. John Huston III, Teresa J.H. Christianson, Kenneth T. Calamia, James F. Meschia, Caterina Giannini, and Dylan Miller for their collaboration in clinical studies on primary central nervous system vasculitis; Dr. Huston for providing angiographic and MRI documentation, and Drs. Giannini and Miller for providing histopathological documentation.

Dr. Salvarani is director of the Unit of Rheumatology at Arcispedale S. Maria Nuova in Reggio Emilia, Italy. Dr. Brown is chair of neurology at the Mayo Clinic in Rochester, Minn. Dr. Hunder is professor emeritus at the Mayo Clinic College of Medicine in Rochester, Minn.

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