Primary angiitis of the CNS

Original article Primary angiitis of the central nervous system: a study of histopathological patterns and review of the literature

“…Although the disease was earlier thought to be rare, recent studies have shown that childhood PACNS could be an important cause of vascular stroke in children [3]. Diagnostic features are based on rare autopsy find-ings and, more recently, on angiographic findings [4,14,32]. Distinct phenotypes in children include progressive, angiography-positive, medium-to-large vessel CNS vasculitis; non-progressive, angiography-positive, medium to-large vessel CNS vasculitis; angiography-negative, small-vessel CNS vasculitis, confirmed by brain biopsy [4,32].…”

Rituximab for Primary Angiitis of the Central Nervous System: Report of 2 Patients from the French COVAC Cohort and Review of the Literature

“…Only a positive biopsy can ascertain the diagnosis, and all appropriate staining, immunohistochemistry studies, and immunophenotyping are needed to rule out related conditions such as amyloid-β-related angiitis or different mimicking diseases such as CNS lymphoma. However, in practice and for more than two-thirds of patients in previously referenced series, biopsy was not performed and diagnosis relied on the combination of vascular changes on CCA and the exclusion, after thorough clinical and laboratory evaluation, of other conditions that can involve brain vessels 1,5,6,7,8 . Our 2 patients underwent extensive investigations to rule out differential diagnoses.…”

“…Although the optimal treatment for PACNS has not been determined, most patients achieve good outcomes with corticosteroids, with or without CYC 2,3,8 . Refractory and/or relapsing cases are rare, and require, after having reconsidered the diagnosis, alternative and/or rescue therapeutic options.…”

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

“…The annual incidence is approximately 2 per million of the population, and it occurs more commonly in men, with the onset usually in the fifth decade of life [113,114]. Patients generally present with headache and encephalopathy; strokes may also occur.…”