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Primary CNS Vasculitides

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White Matter Diseases

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Abstract

The definitions primary central nervous system (CNS) vasculitides or primary angiitis of the CNS (PACNS) refer to a group of rare disorders, characterized by variable degree of heterogeneous inflammatory infiltrates (granulomatous, lymphocytic, or necrotizing), which cause vessel-wall weakening and necrosis and determine a wide spectrum of clinical manifestations, including headache, encephalopathy, ischemic or hemorrhagic strokes, and seizures.

Although a diagnosis of probable PACNS can rely on a combination of clinical presentation, laboratory, cerebrospinal fluid, and imaging findings (magnetic resonance imaging and cerebral angiography), only meningeal and brain biopsy is the gold standard to confirm the presence of typical histopathological patterns and to reach a definite diagnosis of PACNS.

A prompt and accurate diagnosis is essential to start an early induction treatment, based on a combination of high doses of corticosteroids and immunosuppressants, with the main goal of achieving a faster remission and to prevent the progression of the vasculitic process. After induction therapy, maintenance therapy with corticosteroid-sparing oral immunosuppressants should be used to maintain remission, limit the risk of relapses, and prevent long-term disabilities.

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Author information

Authors and Affiliations

  1. Neurology Unit, IRCCS San Raffaele Scientific Institute, Milano, Italy

    Massimo Filippi

  2. Neuroimaging Research Unit, IRCCS San Raffaele Scientific Institute, Milano, Italy

    Massimo Filippi & Maria A. Rocca

  3. Neurophysiology Unit, IRCCS San Raffaele Scientific Institute, Milano, Italy

    Massimo Filippi

  4. Vita-Salute San Raffaele University, Milano, Italy

    Massimo Filippi

Authors
  1. Massimo Filippi
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  2. Maria A. Rocca
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Filippi, M., Rocca, M.A. (2020). Primary CNS Vasculitides. In: White Matter Diseases . Springer, Cham. https://doi.org/10.1007/978-3-030-38621-4_6

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  • DOI: https://doi.org/10.1007/978-3-030-38621-4_6

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