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Related Experiment Video

Updated: Nov 1, 2025

Visualizing Impairment of the Endothelial and Glial Barriers of the Neurovascular Unit during Experimental Autoimmune Encephalomyelitis In Vivo
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[Primary vasculitis of central nervous system].

Mathieu Zuber1

  • 1Service de neurologie et neurovasculaire, Groupe hospitalier Paris Saint-Joseph, Paris, France - Université de Paris, France.

La Revue Du Praticien
|June 23, 2021
PubMed
Summary

Diagnosing primary central nervous system vasculitis (PCNS) is challenging. Advanced imaging is key, and treatment strategies, including immunosuppressants, have improved outcomes with reduced mortality.

Area of Science:

  • Neurology
  • Immunology
  • Vascular Medicine

Background:

  • Primary central nervous system vasculitis (PVCNS) is a rare condition characterized by inflammation of cerebral vessels without systemic disease.
  • Accurate diagnosis remains challenging despite recent large cohort studies.
  • Headache, stroke, and encephalopathy are common presenting symptoms.

Purpose of the Study:

  • To review the diagnostic challenges and therapeutic strategies for primary central nervous system vasculitis.
  • To highlight recent advancements in diagnostic imaging and treatment protocols.
  • To discuss prognostic factors and outcomes in PVCNS.

Main Methods:

  • Review of recent large cohort studies from the USA and France.
  • Emphasis on neuroimaging techniques such as MRI, MRA, CT angiography, and conventional angiography.
Keywords:
Central Nervous System DiseasesVasculitis

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  • Discussion of treatment protocols involving corticosteroids and immunosuppressants.
  • Main Results:

    • Neuroimaging, particularly MRI and vascular imaging, is the cornerstone for diagnosis, often replacing cerebral biopsy.
    • Favorable prognostic indicators include distal vessel involvement, minimal ischemic events, and meningeal enhancement.
    • Treatment with prednisone alone or in combination with cyclophosphamide has improved outcomes.

    Conclusions:

    • Modern diagnostic approaches, primarily advanced neuroimaging, have improved the certainty of PVCNS diagnosis.
    • Identification of favorable prognostic factors guides treatment decisions.
    • Current medical care has significantly reduced PVCNS mortality rates to 5-10% at 5 years.