Differential Diagnosis of Primary Angiitis of the Central Nervous System
What other diseases can mimic CNS vasculitis and must be excluded prior to giving a patient the diagnosis of Primary Angiitis of the Central Nervous System/PCNSV?
• Infections: hepatitis B/C, herpes, HIV, varicella-zoster, syphilis, Lyme disease, progressive multifocal leukoencephalopathy, Bartonella, Mycobacterium tuberculosis, fungal, others.
• Malignancy-associated vasculitis: CNS lymphoma, lymphomatoid granulomatosis, angiocentric lymphoma.
• Drugs: amphetamines, cocaine, heroin, ephedrine, phenylpropanolamine. May cause vasospasm and not vasculitis.
• Connective tissue diseases: SLE, Sjögren’s, Behcet’s, PAN, Churg–Strauss, ANCA-associated vasculitis, antiphospholipid antibody syndrome.
• Susac’s syndrome: endotheliopathy presenting with sensorineural hearing loss, encephalopathy, and retinal artery occlusion.
• Others: sarcoidosis, autoimmune meningoencephalitis, CADASIL, MELAS, fibromuscular dysplasia, cerebral artery dissection, Moyamoya, cardiac emboli.
