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.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Rice CM,Kurian KM,Renowden S,Whiteway A,Price C,Scolding NJdoi
10.1007/s00415-015-7720-9subject
Has Abstractpub_date
2015-05-01 00:00:00pages
1354-9issue
5eissn
0340-5354issn
1432-1459journal_volume
262pub_type
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journal_title：Journal of neurology
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journal_title：Journal of neurology
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journal_title：Journal of neurology
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pub_type： 杂志文章
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pub_type： 临床试验,杂志文章
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journal_title：Journal of neurology
pub_type： 临床试验,杂志文章,随机对照试验
doi：10.1007/BF00878534
更新日期：1996-01-01 00:00:00
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journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF00868397
更新日期：1995-06-01 00:00:00
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journal_title：Journal of neurology
pub_type： 杂志文章
doi：10.1007/BF02430346
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pub_type： 杂志文章,评审
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pub_type： 杂志文章
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pub_type： 临床试验,杂志文章
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