ReviewTherapeutic advances in neurological disorders2018
Primary angiitis of the central nervous system: diagnosis and treatment.
Review in Therapeutic advances in neurological disorders, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 56 papers, 3 of them syntheses that pooled it.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
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.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
56 citing papers in PubMed, 3 syntheses or guidelines pooled it.
- Cerebral vasculitis related to neurosarcoidosis: a case series and systematic literature review.Journal of neurology · 2025Pooled it
- Vessel wall MR imaging of central nervous system vasculitis: a systematic review.Neuroradiology · 2022Pooled it
- Primary Angiitis of the CNS: A Systematic Review and Meta-analysis.Neurology(R) neuroimmunology & neuroinflammation · 2021Pooled it
- Increased serum phenylalanine and tyrosine concentration related to inflammation in patients with primary angiitis of the central nervous system.Biochemistry and biophysics reports · 2026Article
- Diagnostic and therapeutic challenges of isolated spinal vasculitis: A case report.Experimental and therapeutic medicine · 2026Article
- Percutaneous transluminal angioplasty for vertebral artery stenosis due to primary central nervous system vasculitis: A case report.Radiology case reports · 2026Article
- Article
- Complete remission of intracranial lymphocytic vasculitis following resection: illustrative case.Journal of neurosurgery. Case lessons · 2026Article
- Differentiating primary angiitis of the central nervous system from paraneoplastic neurological syndrome.BMJ case reports · 2025Article
- Pediatric Moyamoya Disease in Nepal and Challenges in a Resource-Limited Setting: A Case Report.Clinical case reports · 2025Article
- Article
- MR findings of intravascular large B-cell lymphoma of the central nervous system: report of three cases.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2025Article
- Article
- Beyond the "string of beads": case-based exploration of diagnostic pitfalls and solutions in reversible cerebral vasoconstriction syndrome.The journal of headache and pain · 2025Review
- Diagnosis, pathomechanisms and therapy of cerebral amyloid angiopathy-related inflammation (CAA-ri).Neurological research and practice · 2025Review
- Tumefactive primary angiitis of the central nervous system mimicking glioblastoma: A case-based systematic review.Surgical neurology international · 2025Review
- Glial Fibrillary Acidic Protein Astrocytopathy: Review of Pathogenesis, Imaging Features, and Radiographic Mimics.AJNR. American journal of neuroradiology · 2024Review
- A Rare Case of Idiopathic Reversible Cerebral Vasoconstriction Syndrome.The American journal of case reports · 2024Article
- Predictive Value of Clinical, CSF and Vessel Wall MRI Variables in Diagnosing Primary Angiitis of the CNS.Neurology. Clinical practice · 2024Article
- Primary Angiitis of the Central Nervous System with Pathological Findings of Eosinophilic Granulomatosis with Polyangiitis.Internal medicine (Tokyo, Japan) · 2024Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
7 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Primary angiitis of the central nervous system (PACNS) represents a rare inflammatory disease affecting the brain and spinal cord. Stroke, encephalopathy, headache and seizures are major clinical manifestations. The diagnosis of PACNS is based on the combination of clinical presentation, imaging findings (magnetic resonance imaging and angiography), brain biopsy, and laboratory and cerebral spinal fluid (CSF) values. PACNS can either be confirmed by magnetic resonance angiography (MRA)/conventional angiography or tissue biopsy showing the presence of typical histopathological patterns. Identification of PACNS mimics is often challenging in clinical practice, but crucial to avoid far-reaching treatment decisions. In view of the severity of the disease, with considerable morbidity and mortality, early recognition and treatment initiation is necessary. Due to the rareness and heterogeneity of the disease, there is a lack of randomized data on treatment strategies. Retrospective studies suggest the combined administration of cyclophosphamide and glucocorticoids as induction therapy. Immunosuppressants such as azathioprine, methotrexate or mycophenolate mofetil are often applied for maintenance therapy. In addition, the beneficial effects of two biological agents (anti-CD20 monoclonal antibody rituximab and tumour necrosis factor-α blocker) have been reported. Nevertheless, diagnosis and treatment is still a clinical challenge, and further insights into the immunopathogenesis of PACNS are required to improve the diagnosis and management of patients. The present review provides a comprehensive overview of diagnostics, differential diagnoses, and therapeutic approaches of adult PACNS.
Indexed as
Identifiers
What OpenQuestion holds
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.