ArticleEuropean journal of neurology2022
Diagnosis, differential diagnosis and misdiagnosis of Susac syndrome.
Article in European journal of neurology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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Who cites it
23 citing papers in PubMed, 35 citations in OpenAlex.
- Toward Safer Diagnoses: A SEIPS-Based Narrative Review of Diagnostic Errors.Diagnostics (Basel, Switzerland) · 2026Review
- A 14-Year-Old Male With Susac Syndrome: A Case Report.Case reports in neurological medicine · 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
- Incomplete Susac Syndrome With Recurrent Retinal Occlusions and Vertigo: A Case of Early Immunosuppressive Success.Cureus · 2025Article
- Fluorescein Angiography as a Surrogate Marker of Disease Activity in Susac Syndrome.Neurology(R) neuroimmunology & neuroinflammation · 2025Article
- Clinical Characterization and Prognostic Risk Factors of Susac Syndrome: A Retrospective Multicenter Study.Neurology(R) neuroimmunology & neuroinflammation · 2025Article
- Successful Natalizumab Treatment of Two Female Individuals With Susac Syndrome.European journal of neurology · 2025Article
- Atypical Presentation of Susac Syndrome in 55-Year-Old: From Unremarkable Stroke Workup to Rapid Diagnosis of "Snowball Strokes" and Successful Immunosuppressive Treatment.Case reports in neurological medicine · 2025Article
- Susac syndrome: A rare pediatric case.Radiology case reports · 2024Article
- Misdiagnosis of Multiple Sclerosis: Past, Present, and Future.Current neurology and neuroscience reports · 2024Review
- The neurocognitive and neuropsychiatric manifestations of Susac syndrome: a brief review of the literature and future directions.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2024Review
- Successful treatment of a complete Susac syndrome in a 16-year-old boy: A case report.Clinical case reports · 2024Article
- Diffuse Cerebral Edema With Uncal Herniation Leading to Brain Death in Patient With Fulminant Susac syndrome: Rare Complication of Rare Disease.The Neurohospitalist · 2024Article
- Case report: Susac syndrome-two ends of the spectrum, single center case reports and review of the literature.Frontiers in neurology · 2024Article
- Susac syndrome: neurological update (clinical features, long-term observational follow-up and management of sixteen patients).Journal of neurology · 2023Article
- Clinical characteristics and treatment approaches in patients with Susac syndrome: a scoping review of cases.Journal of neurology · 2023Article
- Susac Syndrome With Retinal Vasculitis: A Rare Case Report and Brief Review.Journal of clinical neurology (Seoul, Korea) · 2023Article
- Susac Syndrome With Good Response to Intravenous Immunoglobulin: A Case Report.Journal of clinical neurology (Seoul, Korea) · 2023Article
- Article
- Fulminant Susac syndrome-a rare cause of coma: The history of the fatal course in a young man.SAGE open medical case reports · 2023Article
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Authors and funding
23 authors at 13 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
background and purposeSusac syndrome (SuS) is an inflammatory condition of the brain, eye and ear. Diagnosis can be challenging, and misdiagnosis is common.
methodsThis is a retrospective review of the medical records of 32 adult patients from an Australasian cohort of SuS patients.
resultsAn alternative diagnosis prior to SuS was made in 30 patients (94%) with seven patients receiving two or more diagnoses. The median time to diagnosis of SuS was 3 months (range 0.5-100 months). The commonest misdiagnoses were migraine in 10 patients (31%), cerebral vasculitis in six (19%), multiple sclerosis in five (16%) and stroke in five (16%). Twenty-two patients were treated for alternative diagnoses, 10 of whom had further clinical manifestations prior to SuS diagnosis. At presentation seven patients (22%) met criteria for definite SuS, 19 (59%) for probable SuS and six (19%) for possible SuS. Six patients (19%) presented with brain-eye-ear involvement, 14 with brain-ear (44%), six with brain-eye (19%) and six (19%) with only brain involvement. In patients with the complete triad of symptoms the median delay to diagnosis was 3 months (range 1-9 months) compared to 5.25 months (range 0.5-100 months) for patients with encephalopathy and ocular symptoms at presentation.
conclusionsSusac syndrome patients are frequently misdiagnosed at initial presentation, despite many having symptoms or radiological features that are red flags for the diagnosis. Delayed diagnosis can lead to patient morbidity. The varied ways in which SuS can present, and clinician failure to consider or recognize SuS, appear to be the main factors leading to misdiagnosis.
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