ArticleJournal of neurology, neurosurgery, and psychiatry2025
Biopsy-proven PACNS: results from the large, multicentre cohort of cerebral vasculitis patients.
Article in Journal of neurology, neurosurgery, and psychiatry, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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Who cites it
2 citing papers in PubMed.
- Ischemic stroke and transient ischemic attack associated with neuroborreliosis: a Danish retrospective cohort.Journal of neural transmission (Vienna, Austria : 1996) · 2026Article
- Primary central nervous system vasculitis: an update.Journal of neurology · 2026Review
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10 authors.
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Abstract
backgroundReports of primary angiitis of the central nervous system (PACNS) are mainly restricted to clinically suspected cases, but biopsy-proven ones are rare. Here, we present results from a large multicentre cohort of patients with biopsy-proven PACNS (BP-PACNS). In particular, we provide insights into characteristics and treatment responses of PACNS subtypes.
methodsBP-PACNS patients treated between 1999 and 2021 were analysed. The outcome was assessed by the modified Rankin Scale (mRS). Between-group comparisons were performed by Kruskal-Wallis, χ
resultsIn total, 57 patients were analysed (52% male). Of these, n=37 (65%) had a lymphocytic (L-PACNS), n=9 (16%) an amyloid-beta-related angiitis (ABRA), n=8 (14%) a granulomatous (G-PACNS) and n=3 (5%) a necrotising (N-PACNS) PACNS subtype. At the time of diagnosis, age differed significantly between groups (median age (years) L-PACNS 47, ABRA 64.5, G-PACNS 37, N-PACNS 65; p=0.008). The clinical course was mostly monophasic in L-PACNS and ABRA (65% and 75%, respectively), while relapsing-remitting in G-PACNS (63%). Median mRS at last follow up was 2 (IQR 1.25-4) in the study group. Worst outcome (median mRS 4) and highest mortality (25%) were seen in G-PACNS. Good induction treatment response was achieved in 77% of all BP-PACNS patients but was low in those with G-PACNS (29%).
conclusionsIn this large, multicentre series of BP-PACNS patients, G-PACNS had a worse functional outcome, a predominant relapsing-remitting disease and a poorer response to the induction therapy. An optimal first-line treatment regimen for G-PACNS patients should be further examined and established in larger studies to improve the outcome of G-PACNS patients.
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