Angiitis of the CNS, Primary

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