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Inflammatory Disorders of the Central Nervous System Vessels: Narrative Review

Aleksandra Ekkert1, Marta Šaulytė2, Dalius Jatužis1

  • 1Centre of Neurology, Vilnius University, Santariskiu 2, LT-08661 Vilnius, Lithuania.

Medicina (Kaunas, Lithuania)
|October 27, 2022
PubMed

Insights

Central nervous system (CNS) vasculitides, or inflammation of CNS vessels, can lead to severe disability or death. Early diagnosis and treatment of CNS vasculitis are crucial to prevent complications like stroke.

Area of Science:

  • Neurology
  • Immunology
  • Vascular Medicine

Background:

  • Inflammatory disorders of central nervous system (CNS) vessels, known as CNS vasculitides, pose significant risks, including disability and mortality.
  • Causes include primary angiitis of the CNS (PACNS), inflammatory cerebral amyloid angiopathy, and systemic inflammatory disorders.
  • Symptoms are often non-specific, presenting as encephalopathy, cognitive/affective changes, headache, and focal neurological deficits.

Purpose of the Study:

  • To review primary and secondary non-infectious CNS vasculitides.
  • To detail diagnostic workup peculiarities and current treatment recommendations for CNS vasculitis.

Main Methods:

  • Review of literature on CNS vasculitis.
  • Discussion of diagnostic criteria and therapeutic strategies.

Main Results:

  • Non-specific symptoms complicate early diagnosis of CNS vasculitis.
  • Diagnostic tools include neuropsychiatric exams, blood/CSF analysis, and MRI.
  • Brain biopsy is the gold standard for diagnosis.

Conclusions:

  • Timely diagnosis and treatment of CNS vasculitis are vital to prevent severe outcomes like stroke.
  • Understanding specific vasculitis characteristics aids diagnosis.
  • Current treatment guidelines aim to mitigate complications.

Keywords:
PACNSamyloid-related brain inflammationcentral nervous system vasculitissystemic vasculitis neurologic manifestations

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