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Primary angiitis of central nervous system (PACNS) is a rare idiopathic disorder affecting blood vessels of brain, spinal cord, and meninges, consequently leading to infarct and less frequently hemorrhage. CNS vasculitis can also occur as part of systemic vasculitis or secondary to autoimmune diseases or infections. The clinical manifestations of PACNS are non specific and no single laboratory investigation or neuroimaging finding can reliably diagnose this condition. Histopathological evidence of transmural inflammation of blood vessels of CNS is the gold standard, but is generally pursued subsequent to conventional angiogram (CA) because of its invasive nature. The differentials of PACNS are exhaustive and include systemic vasculitis, secondary vasculitis, non inflammatory intracranial vasculopathies, demyelination, and neoplasm. These alternative conditions can often be distinguished by history, examination, immunological testing, cerebrospinal fluid analysis, and neuroimaging. CA can detect vasculitic changes in the large to medium cerebral arteries but the specificity is low. Recent advancements in vessel wall imaging techniques have further enabled the distinction of various intracranial vasculopathies from CNS vasculitis. The disease has considerable morbidity and fatality unless timely treatment with immunosuppressive agents is initiated. Induction therapy with glucocorticoids and cyclophosphamide followed by azathioprine, mycophenolate mofetil, or methotrexate as maintenance therapy is the cornerstone of management. Biological agents such as rituximab and anti tumour necrosis factor alpha inhibitors (infliximab and etanercept) may be used in refractory cases. This review discusses the approach to the diagnosis, determinants of outcome, and management. [ABSTRACT FROM AUTHOR]

Purpose: To apply intracranial vessel wall imaging (VWI) to determine changes in vessel wall characteristics between North American moyamoya patients and controls, as well as with standard clinical measures of moyamoya disease severity., Methods: North American moyamoya patients and controls underwent intracranial 3.0 T VWI. Moyamoya patients also underwent digital subtraction angiography (DSA), from which modified Suzuki scores (mSS) were calculated. Lumen and outer vessel wall diameters of the supraclinoid internal carotid arteries (ICAs) and basilar artery on VWI were measured by two readers from which wall thickness was calculated. Controls and moyamoya patients were compared in logistic regression using disease category (moyamoya or none) as the dependent variable and wall thickness, age, gender, and side as the explanatory variables (significance: two-sided p < 0.05). In moyamoya patients, regression was performed with mSS as the dependent variable and wall thickness, age, gender, and side as the explanatory variables. Analyses were repeated for each lumen diameter and outer vessel wall diameter in place of wall thickness., Results: Patients with moyamoya (n = 23, gender = 3/20 male/female; age = 43 ± 12 years) and controls (n = 23, gender = 3/20 male/female, age = 43 ± 13 years) were included. Moyamoya patients showed a significantly smaller ICA lumen and outer vessel wall diameter compared to controls (p < 0.05) but no significant change in vessel wall thickness. Similarly, ICA lumen and outer vessel wall diameters decreased with increasing mSS (p < 0.05)., Conclusion: Findings suggest decreased ICA lumen and outer vessel wall diameters, but no significant difference in wall thickness, between patients and controls. Lumen and outer vessel wall diameters also decreased with disease severity.