Diagnostic approach for children with suspected childhood angiitis of the central nervous system presenting with stroke-type phenotype

cPACNS: childhood angiitis of the central nervous system; MRI: magnetic resonance imaging; MRA: magnetic resonance angiography; CBC: complete blood count; ESR: erythrocyte sedimentation rate; CRP: C-reactive protein; PTT: partial thromboplastin time; INR: international normalized ratio; CSF: cerebrospinal fluid; DSA: digital subtraction angiography.

* All patients with AP-cPACNS have an abnormal brain MRI and may have abnormal findings on MRA. Typical findings include acute and/or chronic stroke, enhancement, and multifocal vessel narrowing. Refer to UpToDate topic for additional details.

¶ Blood cultures and antibody testing for bacterial, viral, and fungal causes varies by geography and potential exposure. Refer to UpToDate topics for additional details.

Δ Rheumatologic and other autoimmune testing is performed for patients with clinical features suggestive of a systemic inflammatory disease such as Takayasu arteritis or polyarteritis nodosa. Testing typically includes antinuclear antibodies, antineutrophil cytoplasmic antibodies, extractable nuclear antigens, anti-double strand DNA (deoxyribonucleic acid), and quantitative immunoglobulin levels.

◊ Thrombophilia testing includes antiphospholipid antibodies, protein C and S activity levels, and antithrombin levels, as well as testing for genetic variants in Factor V Leiden and prothrombin genes.