SynthesisNeurology(R) neuroimmunology & neuroinflammation2021
Primary Angiitis of the CNS: A Systematic Review and Meta-analysis.
Synthesis in Neurology(R) neuroimmunology & neuroinflammation, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 37 papers, 2 of them syntheses that pooled it.
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Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
37 citing papers in PubMed, 2 syntheses or guidelines pooled it, 70 citations in OpenAlex.
- Spinal cord involvement in primary central nervous system vasculitis. A systematic review of clinical, neuroradiological and pathological findings.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2025Pooled it
- European Stroke Organisation (ESO) guidelines on Primary Angiitis of the Central Nervous System (PACNS).European stroke journal · 2023Guideline
- Increased serum phenylalanine and tyrosine concentration related to inflammation in patients with primary angiitis of the central nervous system.Biochemistry and biophysics reports · 2026Article
- Ischemic stroke and transient ischemic attack associated with neuroborreliosis: a Danish retrospective cohort.Journal of neural transmission (Vienna, Austria : 1996) · 2026Article
- Tumor-mimicking primary CNS vasculitis: illustrative case.Journal of neurosurgery. Case lessons · 2026Article
- Treatment Response and Prognosis of Primary Angiitis of the Central Nervous System: A Real-World Observation.CNS neuroscience & therapeutics · 2026Observational
- Primary angiitis of the central nervous system: predictors of stroke during immunosuppressant treatment.Insights into imaging · 2026Article
- A corona-like distribution and patchy pattern of cerebellar infarcts identify patients with giant cell arteritis.Therapeutic advances in neurological disorders · 2026Article
- Overview of Primary Angiitis of the Central Nervous System: Current Insights.Archives of internal medicine research · 2026Article
- Susac syndrome - different treatment approaches for one disease (analysis of case series).Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2025Review
- Disease Characteristics and Treatments Associated with Outcome in Primary Angiitis of the Central Nervous System-A Multicenter Cohort Study in 163 Patients.Annals of neurology · 2025Observational
- Article
- Iatrogenic cerebral amyloid angiopathy: two new cases and systematic review of case reports with neuropathological data.Neurological research and practice · 2025Article
- Biopsy-proven PACNS: results from the large, multicentre cohort of cerebral vasculitis patients.Journal of neurology, neurosurgery, and psychiatry · 2025Article
- Biopsy-Confirmed Small Vessel Primary CNS Vasculitis: Clinical Features and Impact of Early Intensive Treatment on Remission.Neurology(R) neuroimmunology & neuroinflammation · 2025Article
- Unraveling cerebrovascular involvement in EGPA through digital subtraction angiography: case presentation and systematic literature review.BMC rheumatology · 2025Article
- Data-Driven Clustering Approach to Identify Different Phenotypes of Primary Central Nervous System Vasculitis.European journal of neurology · 2025Article
- Integrated Metabolomics and Transcriptomics Analyses Reveal Metabolic Changes in Primary Angiitis of the Central Nervous System.Journal of inflammation research · 2025Article
- Size of affected vessels in primary angiitis of the CNS: associations with clinical features, medical management and functional outcomes.Frontiers in neurology · 2025Article
- Tumefactive primary angiitis of the central nervous system mimicking glioblastoma: A case-based systematic review.Surgical neurology international · 2025Review
Corrections and comments
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Authors and funding
11 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
BACKGROUND AND
objectivesTo facilitate and improve the diagnostic and therapeutic process by systematically reviewing studies on patients with primary angiitis of the CNS (PACNS).
methodsWe searched PubMed, looking at the period between 1988 and February 2020. Studies with adult patients with PACNS were included. We extracted and pooled proportions using fixed-effects models. Main outcomes were proportions of patients with certain clinical, imaging, and laboratory characteristics and neurologic outcomes.
resultsWe identified 46 cohort studies including a total of 911 patients (41% biopsy confirmed, 43% angiogram confirmed, and 16% without clear assignment to the diagnostic procedure). The most frequent onset symptoms were focal neurologic signs (63%), headache (51%), and cognitive impairment (41%). Biopsy- compared with angiogram-confirmed cases had higher occurrences of cognitive impairment (55% vs 39%) and seizures (36% vs 16%), whereas focal neurologic signs occurred less often (56% vs 95%). CSF abnormalities were present in 75% vs 65% and MRI abnormalities in 97% vs 98% of patients. Digital subtraction angiography was positive in 33% of biopsy confirmed, and biopsy was positive in 8% of angiogram-confirmed cases. In 2 large cohorts, mortality was 23% and 8%, and the relapse rate was 30% and 34%, during a median follow-up of 19 and 57 months, respectively. There are no randomized trials on the treatment of PACNS. The initial treatment usually includes glucocorticoids and cyclophosphamide. DISCUSSION: PACNS is associated with disabling symptoms, frequent relapses, and significant mortality. Differences in symptoms and neuroimaging results and low overlap between biopsy and angiogram suggest that biopsy- and angiogram-confirmed cases represent different histopathologic types of PACNS. The optimal treatment is unknown.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.