ArticleJournal of neurology2015
Idiopathic hypereosinophilic syndrome: a new cause of vasculitis of the central nervous system.
Article in Journal of neurology, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed.
- Primary central nervous system vasculitis: an update.Journal of neurology · 2026Review
- Ischemic Stroke in Hypereosinophilic Syndrome: A Clinicopathologic Study of Two Cases.Neuropathology : official journal of the Japanese Society of Neuropathology · 2026Article
- Complete remission of intracranial lymphocytic vasculitis following resection: illustrative case.Journal of neurosurgery. Case lessons · 2026Article
- Review
- Tumefactive eosinophil-rich non-granulomatous small vessel vasculitis in the cerebrum in a patient with idiopathic hypereosinophilic syndrome.Neuropathology : official journal of the Japanese Society of Neuropathology · 2022Article
- A Worm's Tale or Why to Avoid the Raccoon Latrine: A Case ofCase reports in radiology · 2022Article
- Hyperleucocytosis as an unusual presentation of hypereosinophilic pneumonitis with acute respiratory distress syndrome.SAGE open medical case reports · 2022Article
- Literature reviews of stroke with hypereosinophilic syndrome.International journal of cardiology. Heart & vasculature · 2021Review
- Dapsone-induced DRESS after infliximab-induced vasculitis: a case of cerebral infarction in the context of multiple drug reactions.BMJ case reports · 2020Article
- Associations and Outcomes Between Chronic Traumatic Encephalopathy and Vasculitis in Adult Patients.Cureus · 2020Review
- A Closer Look at Angiitis of the central nervous system.Neurosciences (Riyadh, Saudi Arabia) · 2017Article
- Idiopathic hypereosinophilic syndrome with pulmonary hypertension.Pulmonary circulationArticle
Corrections and comments
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Authors and funding
6 authors.
Funding
Abstract
Idiopathic hypereosinophilic syndrome (IHES) is a primary haematological condition characterised by persistent, otherwise unexplained hypereosinophilia sufficient to cause organ damage. Various neurological complications are reported, but very few have mentioned CNS pathology and none has included CNS vasculitis. Our objective here is to report IHES as a new cause of histopathologically confirmed CNS vasculitis. A 39-year-old man presented with a relapsing sub-acute encephalopathy, with severe headaches, confusion and drowsiness, myoclonus, ataxia and papilloedema. He had a history of nephrotic syndrome 18 years earlier, stable for the past 5 years on low-dose corticosteroids and low-dose tacrolimus (2 mg bd); lichen planus, and (15 years previously) aloplecia totalis. On admission, he had a marked peripheral eosinophilia (up to 9.1 × 10(9)/dL), which—it subsequently became clear—had been intermittently present for 16 years. After extensive investigation, biopsies of brain and bone marrow confirmed diagnoses of cerebral vasculitis, with lymphocytic and macrophage (but not eosinophilic) cellular infiltration of blood vessel walls, and IHES. CNS vasculitis can therefore now be added to the list of neurological complications of IHES. A dramatic and sustained neurological improvement, and likewise of the eosinophilia, following treatment with corticosteroids and cyclophosphamide, emphasises the tractability of this newly described form of CNS vasculitis.
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