| Mimics of angiography-positive CNS vasculitis in children |
- Noninflammatory CNS vasculopathies: Dissection, thrombembolic disease, hemoglobin disorders, aPL syndrome, fibromuscular dyplasia, collagen vascular disorders, focal cerebral angiopathy (Marfan syndrome, Ehlers-Danlos syndrome, among others), Moyamoya disease (idiopathic)
- Conditions associated with cerebral vasospasms: Channelopathies including familiar hemiplegic migraine and calcium channelopathy, reversible vasoconstrictive syndrome
- Genetic syndromes with associated vasculopathy: Neurofibromatosis type 1, Down syndrome, PHACES, CADASIL, Fabry disease, homocysteinuria
- Other syndromes with associated cerebral vasculopathy: Cogan syndrome (vasculopathy plus inflammatory eye disease [interstitial keratitis] and vestibularauditory dysfunction), Susac syndrome (noninflammatory vasculopathy resulting in retinopathy, hearing loss and encephalopathy)
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| Mimics of angiography-negative CNS vasculitis in children |
- Nonvasculitic inflammatory brain diseases: Demyelinating diseases (acute demyelinating encephalomyelitis, MS, optic neuritis), antibody-mediated inflammatory brain diseases (anti-NMDAR encephalitis, antibody-mediated limbic encephalitis, neuromyelitis optica, Hashimoto encephalitis, postmycoplasma encephalitis, celiac disease-associated encephalitis, PANDAS), T cell-mediated inflammatory brain diseases (Rasmussen encephalitis), granulomatous inflammatory brain diseases (neurosarcoidosis, ANCA-associated vasculitis)
- Infections: Tuberculosis, JC virus
- Metabolic diseases with associated inflammatory or ischemic brain lesions: MELAS, ROME, polymerase gamma deficiency
- Malignancies: Angiocentric lymphoma
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| Mimics of angiography-positive CNS vasculitis in adults |
- Arteriosclerosis
- Vasospastic diseases: Reversible vasoconstriction syndrome; migraines; vasospasms secondary to hypertension, drug exposure, subarachnoid hemorrhage, eclapsia, pheochromocytoma
- Thrombembolic disease, bacterial endocarditis
- Hemoglobin disorders
- Antiphospholipid antibody syndrome
- Fibromuscular dyplasia
- Genetic diseases associated with obliterating cerebral vasulopathies: Degos disease, retinocerebral vasculopathy with cerebral leucodystrophy
- Other syndromes with associated cerebral vasculopathy: Cogan syndrome, Susac syndrome, Sneddon syndrome (progressive noninflammatory cerebral arteriopathy and livedo)
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| Mimics of angiography-negative CNS vasculitis in adults |
- Demyelinating diseases (MS, optic neuritis)
- Antibody-mediated inflammatory brain diseases: Anti-NMDAR encephalitis, antibody-mediated limbic encephalitis, neuromyelitis optica, Hashimoto encephalitis, postmycoplasma encephalitis, celiac disease-associated encephalitis
- T cell-mediated inflammatory brain diseases: Rasmussen encephalitis
- Granulomatous inflammatory brain diseases: Neurosarcoidosis, ANCA-associated vasculitis
- Infections: Tuberculosis; JC virus, syphilis
- Autoinflammatory syndromes: FMF
- Malignancies
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