Skip to Main Content

KEY FEATURES

  • Rare syndrome that produces small and medium-sized vasculitis limited to the brain and spinal cord

CLINICAL FINDINGS

  • Biopsy-proved cases have predominated in men who present with a history of weeks to months of headaches, encephalopathy, and multifocal strokes

  • Systemic symptoms and signs are absent

DIAGNOSIS

  • MRI of the brain is almost always abnormal

  • Spinal fluid often reveals a mild lymphocytosis and a modest increase in protein level

  • Angiograms classically reveal a "string of beads" pattern produced by alternating segments of arterial narrowing and dilation

  • However, neither MRI nor angiogram appearance is specific for vasculitis

  • Definitive diagnosis requires

    • A compatible clinical picture

    • Exclusion of infection (including subacute bacterial endocarditis), neoplasm (especially intravascular lymphoma), or drug exposure (eg, cocaine) that can mimic primary angiitis of the central nervous system (CNS) and

    • A positive brain biopsy

  • Many patients who fit the clinical profile of stroke, headache, but no encephalopathy may have reversible cerebral vasoconstriction rather than true vasculitis

  • Routine laboratory tests, including erythrocyte sedimentation rate and C-reactive protein, are usually normal

TREATMENT

  • Biopsy-proven cases usually improve with prednisone therapy

  • Often require cyclophosphamide

  • Vasculitis of small cortical and leptomeningeal vessels is associated with a better response and outcome than vasculitis of larger arteries

  • Cases of CNS vasculitis associated with cerebral amyloid angiopathy often respond well to corticosteroidsReversible cerebral vasoconstriction may be best treated with calcium channel blockers (eg, nimodipine or verapamil) and possibly a short course of corticosteroids

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.

  • Create a Free Profile