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[Primary central nervous system vasculitis].

L A Kalashnikova1, L A Dobrynina1, M S Legenko1

  • 1Research Center of Neurology, Moscow, Russia.

Zhurnal Nevrologii I Psikhiatrii Imeni S.S. Korsakova
|October 19, 2019
PubMed
Summary
This summary is machine-generated.

Primary central nervous system vasculitis (PACNS) affects brain vessels without systemic disease. Diagnosis involves imaging and biopsy, with treatment including steroids and immunosuppressants.

Keywords:
MRIbiopsylarge/medium vesselsprimary vasculitis (angiitis) of the central nervous systemsmall vessels

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Area of Science:

  • Neurology
  • Immunology
  • Vascular Medicine

Background:

  • Primary vasculitis of the central nervous system (PACNS) is a rare condition affecting CNS blood vessels.
  • Its etiology remains unclear, necessitating a review of its characteristics.

Purpose of the Study:

  • To review the clinical, laboratory, and radiological features of PACNS.
  • To outline diagnostic approaches and treatment strategies for PACNS.

Main Methods:

  • Literature review of clinical presentations, diagnostic findings, and treatment outcomes for PACNS.
  • Analysis of diagnostic modalities including CSF analysis, MRI, angiography, and biopsy.

Main Results:

  • Clinical manifestations vary based on vessel size, ranging from encephalopathy to ischemic strokes.
  • CSF typically shows lymphocytic pleocytosis and elevated protein; MRI findings are non-specific.
  • Biopsy is gold standard for small vessels; angiography for medium/large vessels; MRI can show vessel wall enhancement.

Conclusions:

  • PACNS diagnosis requires a combination of clinical, imaging, and histopathological data.
  • Treatment involves corticosteroids and cyclophosphamide, with alternatives like rituximab for intolerance.