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Key Features

  • 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, or metabolic disorder or drug exposure (eg, cocaine) that can mimic primary angiitis of the CNS

    • 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

  • Usually improve with prednisone therapy

  • May require cyclophosphamide

  • Reversible cerebral vasoconstriction may be best treated with calcium channel blockers (eg, nimodipine or verapamil) and possibly a short course of corticosteroids

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