Differential diagnosis of intracranial vasculopathy
| Diseases | Clinical features | Diagnostic tests |
|---|---|---|
| Vasculopathy | ||
| Reversible cerebral vasoconstriction syndrome | History of ‘thunderclap’ headache, occurring postpartum or in association with vasoactive drugs, such as selective serotonin-reuptake inhibitors, amphetamines, marijuana and/or nasal decongestants | Usually non-inflammatory CSF (fewer than 5% have white cell count of >10/µL) |
| Intracranial atherosclerosis | Older age, presence of risk factors such as hypertension, diabetes mellitus, hypercholesterolaemia, coronary artery disease and other extracranial arterial atherosclerosis | |
| Intravascular lymphoma | Older age, presence of systemic symptoms (fever, weight loss or night sweats), skin rash | Elevation of serum lactate dehydrogenase and β2-microglobulin; random skin biopsy or brain biopsy |
| Radiation vasculopathy | History of cranial irradiation | |
| Antiphospholipid syndrome | History of deep vein thrombosis or spontaneous abortions | Testing for lupus anticoagulant, anticardiolipin IgG/IgM, anti-β2 glycoprotein I IgG/IgM |
| Secondary vasculitis | ||
| Varicella zoster virus vasculitis | History of shingles (not all cases have this) | Positive varicella CSF IgG (more sensitive than PCR). Note that CSF IgG will not be elevated solely due to prior history of chicken pox |
| Meningovascular syphilis | History of potential exposure or other evidence of tertiary syphilis, including tabes dorsalis or unexplained neuropsychiatric changes | Positive serum treponemal antibody with inflammatory CSF warrants treatment. CSF serology is specific but insensitive |
| Angioinvasive fungal infections | History of immuncompromised state (for symmetry with other entries) | Fungal cultures of blood and CSF. Systemic signs of sepsis. Blood or CSF β(1,3)-D-glucan |
| Systemic vasculitis | Renal insufficiency, pulmonary haemorrhage, unexplained sinusitis, abdominal pain | Elevated erythrocyte sedimentation rate or serum C reactive protein. Testing for antineutrophil cytoplasmic antibodies, antinuclear antibodies, cryoglobulins, hepatitis B/C serologies. Angiography of mesenteric vessels in polyarteritis nodosa |