"The aim of this article will be to discuss primary angiitis of the CNS (PACNS), also known as primary CNS vasculitis (PCNSV), since the other vasculitides are already discussed in specific articles. Please refer to the article on vasculitis for a general discussion of that entity."
"The aim of this article will be to discuss primary angiitis of the CNS (PACNS), also known as primary CNS vasculitis (PCNSV), since the other vasculitides are already discussed in specific articles. Please refer to the article on vasculitis for a general discussion of that entity."
"IgA arteritis"
"Clinical features of primary angiitis of the CNS are non-specific. There is often a long prodromal period with some patients presenting with acutely 15. The diagnosis is made based on Calabrese’s criteria 4, including:"
"Common clinical features include headache (thunderclap headaches are rare), cognitive impairment, focal neurological deficits (e.g. weakness, visual deficit, aphasia) from stroke (ischaemic or haemorrhagic), and seizures 11,12."
"MRI is nearly always abnormal 11,12, and is more specific in demonstrating multiple infarctions, which are typically bilateral, affecting different vascular territories, are of variable size, and are in various stages of healing. Unilateral cases have only been very rarely reported 9."
"MR angiography may show multifocal intracranial arterial narrowing and/or beading of both small and medium-sized blood vessels 8,11. Vessel wall MRI (VW-MRI) may be a useful adjunct to conventional MRI, allowing differentiation between vasculitis, where there is contrast enhancement of the affected arterial wall with concentric wall thickening 17, and other causes of vascular narrowing, such as intracranial atherosclerotic disease (ICAD) plaque, reversible cerebral vasoconstriction syndrome, dissection, and moyamoya disease 7."
"Primary angiitis of the CNS is managed with immunosuppression 15. In induction, this includes high-dose corticosteroids with or without cyclophosphamide or mycophenolate mofetil 15. Maintenance immunosuppression is recommended, and most commonly includes azathioprine, mycophenolate mofetil, methotrexate, or rituximab 3,12,15."
"drug-induced (e.g. amphetamines)"
"History and etymology"