ArticleJournal of neurology2010
Primary central nervous system vasculitis and moyamoya disease: similarities and differences.
Article in Journal of neurology, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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Who cites it
15 citing papers in PubMed, 48 citations in OpenAlex.
- The Hemodynamics of Grief: Emotional Distress as a Precipitant for Ischemia in Moyamoya Syndrome.Cureus · 2026Article
- A complex case of moyamoya disease mimicking vasculitis: diagnostic challenges and systemic complications in a young adult.Radiology case reports · 2025Article
- Angiogenesis-related genes and immune microenvironment in moyamoya disease: a transcriptomic and functional analysis.Orphanet journal of rare diseases · 2025Article
- Blurred by a "Puff of Smoke"-A Case-Based Review on the Challenging Recognition of Coexisting CNS Demyelinating Disease and Moyamoya Angiopathy.International journal of molecular sciences · 2025Review
- Combining machine learning with external validation to explore necroptosis and immune response in moyamoya disease.BMC immunology · 2025Article
- Recovery of intracranial stenoses in varicella zoster virus vasculitis after long-term treatment with valacyclovir and prednisolone.Neurological research and practice · 2022Article
- A Patient With Multiple Sclerosis and Coexisting Moyamoya Disease: Why and How.Frontiers in neurology · 2020Article
- Misdiagnoses and delay of diagnoses in Moyamoya angiopathy-a large Caucasian case series.Journal of neurology · 2019Article
- Primary angiitis of the CNS (PACNS) and Behçet disease.Neurological research and practice · 2019Article
- Primary angiitis of the central nervous system: diagnosis and treatment.Therapeutic advances in neurological disorders · 2018Review
- Cerebral vasculitis in adults: what are the steps in order to establish the diagnosis? Red flags and pitfalls.Clinical and experimental immunology · 2014Review
- The spectrum of CNS vasculitis in children and adults.Nature reviews. Rheumatology · 2011Review
- [Reversible cerebral vasoconstriction syndrome vs cerebral vasculitis? On the importance and difficulty of differentiating].Der Nervenarzt · 2011Article
- Paroxysmal dysarthria and ataxia in multiple sclerosis and corresponding magnetic resonance imaging findings.Journal of neurology · 2011Article
- Angiogenesis in steno-occlusive vasculopathies as a common pathway for intracranial haemorrhage. A report of six cases.Interventional neuroradiology : journal of peritherapeutic neuroradiology, surgical procedures and related neurosciencesArticle
Corrections and comments
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Primary central nervous system vasculitis (PCNSV) and moyamoya disease (MMD) represent rare and poorly-understood causes of stroke. Both may present with similar clinical and auxiliary findings, but differentiation is extremely important because they require different treatment regimens. Our cohort included 21 white patients with PCNSV and 21 white patients with MMD. Clinical and diagnostic features were obtained by retrospective chart review; follow-up information and outcome were obtained prospectively. Data were compared between patients with PCNSV and MMD using Chi square test or Fisher's exact test for categorical data and Mann-Whitney U test for continuous data. The mean age at symptom onset was 42.48 years in PCNSV and 31.0 years in MMD (p = 0.008). All patients with MMD presented with ischemic events while cerebral ischemia was observed in only 14 of 21 patients (66.7%) with PCNSV (p = 0.004). There was no significant difference regarding the frequency of headaches, which represented an important symptom in both conditions. Conventional cerebral angiography verified correct diagnosis in 13 of 17 patients (76.5%) with PCNSV while angiogram verified correct diagnosis in all patients with MMD (p = 0.032). MRI and cerebrospinal fluid studies were appropriate to differentiate between the inflammatory and the non-inflammatory disease. Three PCNSV patients and two MMD patients died within documented follow-up. Despite important pathophysiological and angiographic differences, PCNSV and MMD may present with similar clinical and auxiliary findings. An intensive workup including MRI, conventional cerebral angiography and CSF studies is required to avoid misdiagnosis.
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