Central nervous system vasculitis in adults: An update.

Area of Science:

• Neurology
• Immunology
• Radiology

Background:

• Primary central nervous system vasculitis (PCNSV) presents diverse symptoms, complicating diagnosis.
• Diagnostic tools for PCNSV have variable sensitivity and specificity.
• Differential diagnoses include reversible cerebral vasoconstriction syndrome (RCVS) and noninflammatory vasculopathies.

Purpose of the Study:

• To review the diagnostic challenges and management strategies for PCNSV.
• To differentiate PCNSV from secondary vasculitis and RCVS.
• To highlight the prognostic implications of large-vessel versus small-vessel PCNSV.

Main Methods:

• Literature review of diagnostic and management approaches for PCNSV, secondary CNS vasculitis, and RCVS.
• Analysis of recent data comparing prognosis in large-vessel versus small-vessel PCNSV.
• Emphasis on the role of brain biopsy in definitive diagnosis.

Main Results:

• PCNSV diagnosis is challenging due to varied clinical presentations and imperfect diagnostic tests.
• Brain biopsy remains crucial for accurate diagnosis and excluding mimicking conditions.
• Large-vessel PCNSV is associated with a poorer prognosis compared to small-vessel PCNSV.

Conclusions:

• Accurate diagnosis of PCNSV requires careful consideration of differential diagnoses like RCVS.
• Management strategies should be tailored based on disease characteristics and vessel size.
• Further research is needed to improve diagnostic accuracy and therapeutic outcomes for CNS vasculitis.