ArticleRheumatology international2011
Primary central nervous system vasculitis: clinical experiences with 21 new European cases.
Article in Rheumatology international, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers, 2 of them syntheses that pooled it.
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Who cites it
27 citing papers in PubMed, 2 syntheses or guidelines pooled it.
- European Stroke Organisation (ESO) guidelines on Primary Angiitis of the Central Nervous System (PACNS).European stroke journal · 2023Guideline
- Primary Angiitis of the CNS: A Systematic Review and Meta-analysis.Neurology(R) neuroimmunology & neuroinflammation · 2021Pooled it
- Primary angiitis of the central nervous system: predictors of stroke during immunosuppressant treatment.Insights into imaging · 2026Article
- Temporal evolution of primary angiitis of the central nervous system (PACNS) on MRI following immunosuppressant treatment.Insights into imaging · 2024Article
- Primary angiitis of the CNS and ANCA-associated vasculitis: from pathology to treatment.Rheumatology international · 2024Review
- Anti-neutrophil cytoplasmic antibody associated vasculitis of the brain and oral cavity: a case report.Oxford medical case reports · 2023Article
- Close, but no cigar: an unfortunate case of primary angiitis of the central nervous system.Annals of medicine and surgery (2012) · 2023Article
- Current and future advances in practice: a practical approach to the diagnosis and management of primary central nervous system vasculitis.Rheumatology advances in practice · 2023Review
- Headache and immunological/autoimmune disorders: a comprehensive review of available epidemiological evidence with insights on potential underlying mechanisms.Journal of neuroinflammation · 2021Review
- Initial experience with [European journal of nuclear medicine and molecular imaging · 2020Article
- Clinical Perspective on Primary Angiitis of the Central Nervous System in Childhood (cPACNS).Frontiers in pediatrics · 2020Review
- Primary Angiitis of the Central Nervous System Mimicking Glioblastoma: A Case Report and Literature Review.Frontiers in neurology · 2019Article
- Primary angiitis of the CNS (PACNS) and Behçet disease.Neurological research and practice · 2019Article
- Diagnosis and Treatment of Primary Central Nervous System Angiitis.Current treatment options in neurology · 2018Review
- Primary angiitis of the central nervous system: diagnosis and treatment.Therapeutic advances in neurological disorders · 2018Review
- Diagnostic test results in primary CNS vasculitis: A systematic review of published cases.Neurology. Clinical practice · 2017Review
- [Clinical aspects and diagnostics of cerebral vasculitis].Der Radiologe · 2016Review
- The Role of Catheter Angiography in the Diagnosis of Central Nervous System Vasculitis.Interventional neurology · 2016Review
- A rare case of tumor-mimicking primary angiitis of the central nervous system.Molecular and clinical oncology · 2016Article
- [Neuroimaging of cerebral vasculitis].Der Radiologe · 2016Review
Corrections and comments
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Authors and funding
2 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Primary central nervous system vasculitis (PCNSV) represents a rare and poorly understood form of vascular inflammatory disease restricted to the brain and spinal cord. This study was undertaken to better characterize demographic and clinical features and to evaluate treatments and outcome. Our cohort included 21 white patients with PCNSV treated in a single German institution between 2003 and 2008. Clinical and diagnostic features were obtained by retrospective chart review; follow-up information and outcome were obtained prospectively. Clinical findings and outcomes were compared among patients categorized by different characteristics. The mean age at onset of symptoms was 42.48 years. In 6 (28.6%) of 21 patients, PCNSV diagnosis was established based on positive CNS tissue histopathology. Additional angiography was only performed in two of these six patients and was negative in both cases. For 13 patients (61.9%), the diagnosis was confirmed by positive cerebral angiogram. The most frequent clinical presentations over the whole follow-up period were hemiparesis (76.2%), cerebral ischemia (66.7%), headache (57.1%) and altered cognition (52.4%). Patients with MR-tomographic evident microangiopathy showed significantly worse modified Rankin disability scores during the onset and at last visit compared to the other patients (P = 0.047, P = 0.039). Clinical features and course of PCNSV of Europeans analyzed in this German study are comparable to American results but some further reported characteristics of different subtypes could not be duplicated. Although this well-defined analysis adds to our knowledge of PCNSV, it also raises important open questions. The enigma of PCNSV could only be solved by implementing large prospective multicenter databases and elucidating animal models.
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