title: Idiopathic hypereosinophilic syndrome: a new cause of vasculitis of the central nervous system.
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.
authors.affiliation: Department of Neurology, University of Bristol Institute of Clinical Neurosciences, Southmead Hospital, Bristol, UK.
authors.forename: C M
authors.initials: CM
authors.lastname: Rice
authors.forename: K M
authors.initials: KM
authors.lastname: Kurian
authors.forename: S
authors.initials: S
authors.lastname: Renowden
authors.forename: A
authors.initials: A
authors.lastname: Whiteway
authors.forename: C
authors.initials: C
authors.lastname: Price
authors.forename: N J
authors.initials: NJ
authors.lastname: Scolding
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D003520:Cyclophosphamide / Q000627:therapeutic use; D004802:Eosinophilia / Q000473:pathology; D006801:Humans; D017681:Hypereosinophilic Syndrome / Q000150:complications* / Q000188:drug therapy; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D020293:Vasculitis, Central Nervous System / Q000209:etiology*
chemical_list: D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide
references.citation: Muscle Nerve. 1983 May;6(4):291-8
references.pmid: 6866008
references.citation: Lancet Neurol. 2011 Jun;10(6):561-72
references.pmid: 21601163
references.citation: J Neurol Neurosurg Psychiatry. 1990 May;53(5):440-1
references.pmid: 2351980
references.citation: Br J Neurosurg. 2011 Oct;25(5):614-20
references.pmid: 21501048
references.citation: Clin Neuropathol. 2009 May-Jun;28(3):197-202
references.pmid: 19537138
references.citation: Neurology. 1999 Sep 11;53(4):858-60
references.pmid: 10489055
references.citation: Intern Med. 2003 May;42(5):424-7
references.pmid: 12793714
references.citation: Ann Neurol. 2010 Nov;68(5):602-10
references.pmid: 21031577
references.citation: J Clin Neurosci. 2010 Mar;17(3):377-8
references.pmid: 20071179
references.citation: Intern Med. 2011;50(11):1219-25
references.pmid: 21628939
references.citation: J Neuroophthalmol. 2005 Jun;25(2):116-21
references.pmid: 15937435
references.citation: Annu Rev Med. 2009;60:293-306
references.pmid: 19630574
references.citation: Neurology. 2008 Sep 2;71(10):779; author reply 779-80
references.pmid: 18765657
references.citation: Neurology. 1989 Oct;39(10):1388-91
references.pmid: 2797461
references.citation: Am J Med. 1985 Apr;78(4):674-6
references.pmid: 3985040
references.citation: Clin Neurol Neurosurg. 1988;90(3):269-73
references.pmid: 3197357
references.citation: Brain. 2005 Mar;128(Pt 3):500-15
references.pmid: 15659428
references.citation: Neurol India. 2010 Sep-Oct;58(5):793-4
references.pmid: 21045519
references.citation: Lancet. 2012 Aug 25;380(9843):767-77
references.pmid: 22575778
references.citation: Intern Med. 2003 May;42(5):381-2
references.pmid: 12793705
references.citation: J Neurol. 2001 Apr;248(4):279-84
references.pmid: 11374091
references.citation: Arq Neuropsiquiatr. 1991 Dec;49(4):450-5
references.pmid: 1668782
references.citation: Ann Intern Med. 1985 Jan;102(1):109-14
references.pmid: 2981493
references.citation: Neurology. 1988 Mar;38(3):494-6
references.pmid: 2831471
references.citation: Neurology. 1985 Aug;35(8):1140-5
references.pmid: 2991813
references.citation: Arch Phys Med Rehabil. 1990 May;71(6):433-5
references.pmid: 2159275
references.citation: Haematologica. 2009 Sep;94(9):1188-93
references.pmid: 19734412
references.citation: Acta Neurol Belg. 1996 Jun;96(2):137-40
references.pmid: 8711988
references.citation: J Clin Neurosci. 2012 Dec;19(12):1746-8
references.pmid: 22999560
references.citation: Acta Neurol Belg. 1991;91(1):12-9
references.pmid: 1851360