ArticleJournal of neurology2019
Misdiagnoses and delay of diagnoses in Moyamoya angiopathy-a large Caucasian case series.
Article in Journal of neurology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers, 1 of them a synthesis that pooled it.
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Who cites it
19 citing papers in PubMed, 1 synthesis or guideline pooled it, 38 citations in OpenAlex.
- Moyamoya disease in Southeast Asians: genetic and autopsy data, new cases, systematic review, and meta-analysis of all patients from the literature.Journal of neurology · 2024Pooled it
- Delayed Recognition and Stroke-Predominant Presentation in Down Syndrome-Associated Moyamoya Syndrome.Stroke · 2026Article
- Argo Delphi consensus statement on red flags and clinical gateways towards rare disease diagnosis.Scientific reports · 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
- Misdiagnosis of Multiple Sclerosis: Past, Present, and Future.Current neurology and neuroscience reports · 2024Review
- Moyamoya Disease: Clinical and Radiological Characteristics in Adult Greek Patients.Journal of clinical medicine · 2023Article
- Diagnosis and management of adult primary angiitis of the central nervous system: an international survey on current practices.Journal of neurology · 2023Article
- European Stroke Organisation (ESO) Guidelines on Moyamoya angiopathy Endorsed by Vascular European Reference Network (VASCERN).European stroke journal · 2023Article
- Identification of associations and distinguishing moyamoya disease from ischemic strokes of other etiologies: A retrospective case-control study.Annals of medicine and surgery (2012) · 2022Article
- Recovery of intracranial stenoses in varicella zoster virus vasculitis after long-term treatment with valacyclovir and prednisolone.Neurological research and practice · 2022Article
- The angiographic presentation of European Moyamoya angiopathy.Journal of neurology · 2022Observational
- Epidemiology of Moyamoya Angiopathy in Eastern India.Frontiers in neurology · 2022Article
- Clinical Management of Moyamoya Patients.Journal of clinical medicine · 2021Review
- Extended stereotactic brain biopsy in suspected primary central nervous system angiitis: good diagnostic accuracy and high safety.Journal of neurology · 2021Article
- Migraine and rare neurological disorders.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2020Review
- Genetic Modifiers and Rare Mendelian Disease.Genes · 2020Review
- A Patient With Multiple Sclerosis and Coexisting Moyamoya Disease: Why and How.Frontiers in neurology · 2020Article
- Histopathology of Moyamoya angiopathy in a European patient.Journal of neurology · 2019Article
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Corrections and comments
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Authors and funding
8 authors at 3 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundThe lacking awareness of healthcare providers bears the risk of delayed or false diagnoses in rare diseases. No systematic data about misdiagnoses of Moyamoya angiopathy (MMA) are available.
objectiveTo evaluate the rate and pattern of missed diagnoses in MMA.
methodsRetrospective analysis of a consecutive case series from a single German referral center. Rates of missed or delayed diagnoses in Caucasian MMA patients were calculated based on discharge letters from other hospitals and systematic chart review.
resultsOut of 192 Caucasian patients eventually diagnosed with MMA at our center, an initial misdiagnosis was identified in 119 patients (62%). The time between onset and diagnosis was 1 year in 24 patients, 2 years in 23 patients, 3 years in 10 patients, and > 3 years in 49 patients (mean 5.28, median 3, standard deviation 5.11, and range 4-26 years). The most common misdiagnoses were cerebral vasculitis (31%), etiologically ill-defined stroke diagnoses (30.2%), and MS (3.6%).
conclusionsThis is the first systematic report which shows that patients with MMA are at high risk to be falsely diagnosed and treated. Depiction of typical vascular abnormalities in angiopathy is essential. Normal CSF cell counts, negative oligoclonal bands, and lack of infratentorial lesions as well as gadolinium-positive T1 lesions on MRI may be red flags differentiating this vasculopathy from vasculitis and MS.
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Registered trials
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