Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + Download Section PDF Listen ++ For further information, see CMDT Part 20-28: Primary Angiitis of the Central Nervous System + Key Features Download Section PDF Listen +++ ++ Small and medium-sized vasculitis limited to the brain and spinal cord + Clinical Findings Download Section PDF Listen +++ ++ 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 Download Section PDF Listen +++ ++ 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 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, may be normal + Treatment Download Section PDF Listen +++ ++ 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