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Adult Primary Central Nervous System Vasculitis

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Systemic Vasculitides: Current Status and Perspectives

Abstract

Primary central nervous system vasculitis is an uncommon disorder of unknown cause that is limited to the brain and spinal cord. The median age of onset is 50 years. The neurological manifestations are diverse, but commonly include headache, altered cognition, focal weakness, or stroke. Serological markers of inflammation are usually normal. Cerebrospinal fluid is abnormal in approximately 80–90 % of the cases. The diagnosis is unlikely in the presence of a normal magnetic resonance imaging (MRI) of the brain. Biopsy of central nervous system tissue showing vasculitis is the only definitive test, however angiography has often been used for diagnosis even though it has only moderate sensitivity and specificity. The size of the vessels involved is varied and influences outcomes and response to treatment. Early recognition is important because treatment with corticosteroids, with or without cytotoxic drugs, may prevent serious outcomes. The differential diagnosis includes reversible cerebral vasoconstriction syndromes and secondary cerebral vasculitis.

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Authors and Affiliations

  1. Unit of Rheumatology, Arcispedale S.Maria Nuova, V.le Risorgimento N80, 42100, Reggio Emilia, Italy

    Carlo Salvarani

  2. Department of Neurology, Mayo Clinic, Rochester, MN, USA

    Robert D. Brown Jr.

  3. Division of Anatomic Pathology, Mayo Clinic, Rochester, MN, USA

    Robert D. Brown Jr.

  4. Mayo Clinic College of Medicine, Rochester, MN, USA

    Caterina Giannini & Gene G. Hunder

Authors
  1. Carlo Salvarani
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  2. Robert D. Brown Jr.
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  3. Caterina Giannini
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  4. Gene G. Hunder
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Corresponding author

Correspondence to Carlo Salvarani .

Editor information

Editors and Affiliations

  1. University of Bari Medical School, Bari, Italy

    Franco Dammacco

  2. University of Bari Medical School, Bari, Italy

    Domenico Ribatti

  3. University of Bari Medical School, Bari, Italy

    Angelo Vacca

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Salvarani, C., Brown, R.D., Giannini, C., Hunder, G.G. (2016). Adult Primary Central Nervous System Vasculitis. In: Dammacco, F., Ribatti, D., Vacca, A. (eds) Systemic Vasculitides: Current Status and Perspectives. Springer, Cham. https://doi.org/10.1007/978-3-319-40136-2_22

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