Hypereosinophilic syndrome is a rare disorder of the hematopoietic system. The disease is characterized by continuously high number of eosinophils (>1.5x109/L) for more than six months. Other possible causes of hypereosinophilia, such as allergic and parasitic diseases, malignant disease, Churg-Strauss disease and infection should be eliminated. The most common manifestations of hypereosinophilic syndrome are pulmonary, skin, gastrointestinal, cardiac difficulties and neurologic lesions. Numerous neurologic lesions have been described, in particular of the central and peripheral nervous systems. Review of the literature revealed the following to have been recorded so far: mononeuritis multiplex, sensory polyneuropathy, radiculopathy, myalgia, myositis and perimyositis, neuropathy, ataxia, paraplegia, ophthalmologic abnormalities, optic neuritis, hemiplegia-hemiparesis, spasmodic quadriplegia, seizures, meningitis, cerebral infarction, organic psychosyndrome, other mental changes, stroke, temporal arteritis, leptomeningeal dissemination, memory deficits and dysarthria.Hipereozinofilni sindrom je rijetka bolest hematopoetskog sustava. Bolest je obilježena visokim brojem eozinofila (>1,5x109/L) duže od šest mjeseci, uz uvjet da su isključeni drugi mogući uzroci hipereozinofilije kao što su alergija i parazitne bolesti, maligne bolesti, Churg-Straussova bolest i druge infekcijske bolesti. Većina kliničkih manifestacija hipereozinofilnog sindroma obuhvaća oštećenja pluća, kože, probavnog sustava, srčane poremećaje i neurološka oštećenja. Opisana su brojna neurološka oštećenja središnjeg i perifernog živčanog sustava. Pretraživanjem literature nalaze se moguće neurološke manifestacije bolesti kao što su mononeuritis multiplex, senzorna polineuropatija, radikulopatija, mijalgija, miozitis i perimiozitis, neuropatija, ataksija, paraplegija, oftalmološki poremećaji, optički neuritis, hemiplegija-hemipareza, spastička tetraplegija, epileptični napadaji, meningitis, ishemijski moždani udar, psihoorganski sindrom i drugi mentalni poremećaji, temporalni arteritis, leptomeningealna diseminacija, poremećaji pamćenja i dizartrija

Dragan Lončar

Katica Kodžoman

Marina Titlić

English

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Acta Clin Croat,  Vol. 51,  No. 1,  2012 65Acta Clin Croat 2012; 51:65-69 ReviewNeuRologiC MANifestAtioNs of HypeReosiNopHiliC syNdRoMe – Review of tHe liteRAtuReMarina titlić1,2, Katica Kodžoman2 and dragan lončar31university department of Neurology, split university Hospital Center; 2school of Medicine, university of split, split, Croatia; 3school of Medicine, university of Kragujevac, Kragujevac, serbiasuMMARy – Hypereosinophilic syndrome is a rare disorder of the hematopoietic system. The disease is characterized by continuously high number of eosinophils (>1.5x109/l) for more than six months. other possible causes of hypereosinophilia, such as allergic and parasitic diseases, mali-gnant disease, Churg-strauss disease and infection should be eliminated. The most common mani-festations of hypereosinophilic syndrome are pulmonary, skin, gastrointestinal, cardiac difficulties and neurologic lesions. Numerous neurologic lesions have been described, in particular of the cen-tral and peripheral nervous systems. Review of the literature revealed the following to have been recorded so far: mononeuritis multiplex, sensory polyneuropathy, radiculopathy, myalgia, myosi-tis and perimyositis, neuropathy, ataxia, paraplegia, ophthalmologic abnormalities, optic neuritis, hemiplegia-hemiparesis, spasmodic quadriplegia, seizures, meningitis, cerebral infarction, organic psychosyndrome, other mental changes, stroke, temporal arteritis, leptomeningeal dissemination, memory deficits and dysarthria.Key words:  Manifestations, neurologic; Hypereosinophilic syndromeCorrespondence to: Marina Titlić, MD, PhD, university depar-tment of Neurology, split university Hospital Center, spinčićeva 1, HR-21000 split, Croatiae-mail: marina.titlic@gmail.comReceived september 1, 2010, accepted december 15, 2011Introduction Hypereosinophilic syndrome (Hes) is a rare and heterogeneous group of disorders defined as persistent marked blood eosinophilia (>1.5x109/l for more than six consecutive months) associated with evidence of eosinophil-induced organ damage, where other causes of hypereosinophilia such as allergic, parasitic, and ma-lignant disorders have been excluded1-4. The prevalence is unknown, younger to middle-aged patients are most frequently affected, with a male predominance (4-9:1 ratio)5. Hes is a disease of multifactorial genesis, from clonal tumorous proliferation to reactive changes. The myeloproliferative variant caused by interstitial dele-tion in chromosome 4q12 and the lymphoproliferative variant associated with clonal proliferation of pheno-typically abnormal t cells have been differentiated. An autosomal dominant familial form that has been mapped to chromosome 5q31, episodic form (gleich’s syndrome) and a clinically silent or benign form have also been described. in patients with the myeloprolif-erative variant, well responding to imatinib therapy, fip1l1/pdgfRA (f/p) has been identified, being deemed an imatinib responsive Hes. The lymphop-roliferative variant Hes (l-Hes) is characterized by a phenotypically distinct clonal t cell population in peripheral blood. Hypereosinophilia is a consequence of the increased production of the eosinophilopoietic cytokine, especially interleukin 5 (il-5). diagnosis is based on establishing a population of t cells with an aberrant phenotype, most often Cd3-Cd4+Cd8- in peripheral blood. However, around 50% of patients with Hes can be classified as neither myeloprolifera-tive nor lymphoproliferative variant6-13. 66 Acta Clin Croat,  Vol. 51,   No. 1,  2012Marina titlić et al. Neurologic manifestations of hypereosinophilic syndrome – review of the literatureHes is treated by steroids, cytotoxic agents, im-munomodulatory therapies, tyrosine kinase inhibi-tors, monoclonal antibody therapy and bone marrow transplantation6-8. The organs affected by hypereosinophilia dif-fer from case to case. Most commonly, these are the skin, heart, lungs, central and peripheral nervous sys-tems. frequent manifestations are also hepato- and/or splenomegaly, eosinophilic gastroenteritis, coagu-lation disorders, etc.14-17. Neurologic symptomatology is nonspecific and varies; therefore we are presenting here the neurologic manifestations of Hes recorded so far.Review of the Literature and Discussionwe performed systematic review of peer-reviewed publications identified through MedliNe databases (searched in May 2011). The search term was Hypere-osinophil syndrome, and the search was limited to clinical trials and articles in english. The search was extended by reviewing bibliographies from pertinent original reports of data and review articles. unpub-lished trials and data presented only in abstract form are not included.literature reports damages to the central nervous system and peripheral nervous system. Authors very Table 1. Neurologic manifestations of the hypereosinophilic syndromeAuthors Neurologic manifestationsgardner-Thorpe et al.18delaporte et al.19farcet et al.20Chaine et al.21Kessler et al.22Moore et al.23weingarten et al.24Martin-gonzález et al.25Zagami et al.26Haraoka et al.27Kumara et al.28otto29lee et al.30Arnaud et al.31Koto et al.32pellissier et al.33Cengiz et al.34prunier et al.35endo and Miyake36wolf et al.37lincoff and schlesinger38Numagami et al.39srinavasan et al.40Noureen and Rana41perini et al.42sethi and schmidley43ito et al.44Kanamori et al.45optic ataxia, paralysis of visual fixationHemiplegia, spasmodic quadriplegia, seizuresArterial occlusions of the retina, neurologic signsBlurred vision on one or both eyesNeurologic disturbancesCentral nervous system dysfunction, encephalopathy characterized by behav-ioral disturbances, motor neuron signs, peripheral neuropathy with sensory polyneuropathy, mononeuritis multiplex, radiculopathyMeningitisNeurologic dysfunctionAtaxia-telangiectasiaHemiparesisNeurologic deficitfrequent neurologic signs, cerebral infarction, organic psychosyndromeAbnormalities of the central and peripheral nervous systemMononeuritis multiplex (acute myelino-axonal degeneration with endoneural edema), myalgia, axonal pattern of neuropathyBilateral papilledemaperimyositis, myositis, myalgiaperipheral neuropathy, mental changesvisual and cognitive disordersparaplegia, dysuriaAtaxia, memory deficits, dysarthriaoptic neuritisCerebral sinus thrombosis, cerebral hemorrhagesciatic neuropathiesstroke, hemiplegiastrokestroketemporal arteritisleptomeningeal dissemination, intraventricular mass lesionActa Clin Croat,  Vol. 51,  No. 1,  2012 67Marina titlić et al. Neurologic manifestations of hypereosinophilic syndrome – review of the literatureoften report numerous associated detrimental effects manifested by a number of symptoms such a monon-euritis multiplex, sensory polyneuropathy, radiculopa-thy, myalgia, myositis and perimyositis, neuropathy, ataxia, paraplegia, ophthalmologic abnormalities, optic neuritis, hemiplegia-hemiparesis, spasmodic quadriplegia, seizures, meningitis, cerebral infarction, organic psychosyndrome and other mental changes, memory deficits, dysarthria, etc. (table 1). Hes appears to be a very rare disease, manifest-ed in damages to a number of organs. Most often af-fected are the skin, heart, lungs, central and peripheral nervous systems, in as many as 50% of cases1. diffuse encephalopathy presents as altered behavior and cog-nitive function, confusion, and memory loss. periph-eral neuropathies present as symmetric or asymmetric sensory changes, pure motor deficits, or mixed sensory and motor complaints. stroke or transient ischemic episodes may result from cardiac difficulties and heart originating thrombus, but also from increased inclina-tion to thrombosis1,2,38. it is the increased inclination to thrombosis in Hes that probably causes thrombosis of intracranial veins (longitudinal and/or lateral sinus).Thus, hypereosinophilic syndrome may result in a number of neurologic signs. Therefore, in cases of neurologic signs with no clinically established cause, especially in younger people, the possible Hes should be considered.References 1. Roufosse fe, goldMAN M, CogAN e. Hypere-osinophilic syndromes. orphanet J Rare dis 2007;2:37-48.2. HAgHpANAH v, lAsHKARi A, tAvANgAR sM, MoRAdZAdeH K. Hypereosinophilia as the presenta-tion of metastatic medullary thyroid carcinoma: a remarkable event. Am J Med sci 2007;334:131-2. 3. tHiAM A, diop Ag, NdiAye MM, NdiAye ip. Churg-strauss syndrome disclosed by polyneuritis apropos of a case in a senegalese patient. dakar Med 1996;41:69-73.4. BeNveNisti ds, ultMANN Je. eosinophilic leuke-mia. Report of five cases and review of literature. Ann intern Med 1969;71:731-45.5. welleR pH, BuBley gJ. The idiopathic hypereosino-philic syndrome. Blood 1994;83:2759-79.6. KlioN Ad. Recent advances in the diagnosis and treatment of hypereosinophilic syndromes. Hematology Am soc He-matol educ program 2005;209-14.7. KlioN Ad, RoByN J, MARiC i, fu w, sCHMid l, leMeRy s, lAw MA, HARtsell M, tAllAR- wil-liAMs C, fAy Mp, duNBAR Ce, NutMAN tB. Re-lapse following discontinuation of imatinib mesylate therapy for fip1l1/pdgfRA-positive chronic eosinophilic leuke-mia: implications for optimal dosing. Blood 2007;110:3552-6.  8. KlioN Ad. Approach to the therapy of hypereosinophilic syn-dromes. immunol Allergy Clin North Am 2007;27:551-60.  9. KlioN Ad, BoCHNeR Bs, gleiCH gJ, NutMAN tB, RotHeNBeRg Me, siMoN Hu, weCHsleR Me, welleR pf, the Hypereosinophilic syndromes working group. J Allergy Clin immunol 2006;17:1292-302.10. KlioN Ad. How i treat hypereosinophilic syndromes. Blood 2009;114:3736-41.11. siMoN d, siMoN Hu. eosinophilic disorders. J Allergy Clin immunol 2007;119:1291-300.12. BAiN BJ, fletCHeR sH. Chronic eosinophilic leukemias and the myeloproliferative variant of the hypereosinophilic syndrome. immunol Allergy Clin North Am 2007;27:377-88.13. Roufosse f. Hypereosinophilic syndrome variants: diag-nostic and therapeutic considerations. Haematologica 2009; 94:1188-93. 14. CHusid MJ, dAle dC, west BC, wolff sM. The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine (Baltimore) 1975;54:1-27.15. AMBăRus v, CosovANu A. The evolutionary charac-teristics of the hypereosinophilic syndrome. Rev Med Chir soc Med Nat iasi 1998;102:69-75.16. sCHooley Rt, flAuM MA, gRAlNiCK HR, fAu-Ci As. A clinicopathologic correlation of the idiopathic hy-pereosinophilic syndrome. ii Clinical manifestations. Blood 1981;58:1021-6.17. dAHABReH iJ, giANNouli s, Zoi C, Zoi K, voul-gARelis M, Moutsopoulos HM. Management of hypereosinophilic syndrome: a prospective study in the era of molecular genetics. Medicine (Baltimore) 2007;86:344-54.18. gARdNeR-tHoRpe C, HARRiMAN dg, pARsoNs M, Rudge p. loffler’s eosinophilic endocarditis with Bal-int’s syndrome (optic ataxia and paralysis of visual fixation). Q J Med 1971;40(158):249-60.19. delApoRte p, sAlAMA J, CouloMBel l, leJe-uNe f, loRtHolARy p. An essential hypereosinophilic syndrome. Cytological, cyto-enzymological and ultrastruc-tural studies (author’s transl). Ann Med interne (paris) 1980;131:531-4.20. fARCet Jp, BiNAgHi M, KueNtZ M, MeRlieR Jf, MAyAud C, NeBout t, NAss p, feRRet J. A hyper-eosinophilic syndrome with retinal arteritis and tuberculosis. Arch intern Med 1982;142:625-7.21. CHAiNe g, dAvies J, KoHNeR eM, HAwARtH s, spRy CJ. ophthalmologic abnormalities in the hypereosino-philic syndrome. ophthalmology 1982;89:1348-56.68 Acta Clin Croat,  Vol. 51,   No. 1,  2012Marina titlić et al. Neurologic manifestations of hypereosinophilic syndrome – review of the literature22. KessleR A, RAdwAN H, Kott e. Neurological distur-bances in idiopathic hypereosinophilic syndrome. Harefuah 1984;107:8-9.23. MooRe pM, HARley JB, fAuCi As. Neurologic dys-function in the idiopathic hypereosinophilic syndrome. Ann intern Med 1985;102:109-14.24. weiNgARteN Js, o’sHeAl sf, MARgolis ws. eosinophilic meningitis and the hypereosinophilic syndrome. Case report and review of literature. Am J Med 1985;78:674-6.25. MARtíN-goNZáleZ e, yeBRA M, gARCíA-MeRiNo A, liAño H. Neurologic dysfunction in the idiopathic hypereosinophilic syndrome. Ann intern Med 1986;104:448-9.26. ZAgAMi As, ColeBAtCH HJ, wAKefield d. Chronic eosinophilic pneumonia in a patient with ataxia te-langiectasia. Aust N Z J Med 1987;17:592-5.27. HARAoKA H, sAto H, KoudA s, MitsuwA M, AgAtsuMA K, sHiNouRA A. A case of hypereosino-philic syndrome associated with consciousness disorder and hemiparesis. Nippon Naika gakkai Zasshi 1990;79:233-4.28. KuMAR KA, ANJANeyulu A, MuRtHy JM. idio-pathic hypereosinophilic syndrome presenting as childhood hemiplegia. postgrad Med J 1992;68(804):831-3.29. otto v. Neurologic complications in hypereosino-philia syndrome – a case report. fortschr Neurol psychiatr 1993;61:310-2.30. lee Jd, CHeN st, CHeN CJ, CHeN CM, Ro ls. idiopathic hypereosinophilic syndrome with eosinophilic myositis, peripheral neuropathy and central nervous system involvement. J formos Med Assoc 1996;95:933-6.31. ARNAud A, lAgueNy A, HeRMosillA e, feR-ReR X, vitAl C, JulieN J. Multineuritis in essential hy-pereosinophilia syndrome. Rev Neurol (paris) 1997;153:785-9. 32. Koto R, NisHiMuRA H, tACHiBANA H, sugi-tA M. Case of hypereosinophilic syndrome with bilateral papilledema. Nippon Naika gakkai Zasshi 1997;86:1967-8.33. pellissieR Jf, figARellA-BRANgeR d, seRRA-tRiCe g. Neuromuscular diseases with eosinophilia. Med trop (Mars) 1998;58(4 suppl):471-6.34. CeNgiZ B, suCAK g, KuRuoglu R, tAN e. Hy-pereosinophilic syndrome: progression of peripheral neurop-athy despite controlled eosinophil levels. Acta Neurol Belg 1999;99:133-7.35. pRuNieR C, HoMMet C, AutRet A, de tof-fol B. primary hypereosinophilia syndrome manifesting as encephalopathy and vision disorders. Rev Neurol (paris) 2001;157:1283-6.36. eNdo C, MiyAKe s. A case of hypereosinophilic syndrome associated with paraplegia. No to Hattatsu 2003;35:411-6.37. wolf d, gAstl g, RuMpold H. Complete remission of an idiopathic hypereosinophilic syndrome while using im-antinib. dtsch Med wochenschr 2004;129:2104-6.38. liNCoff Ns, sCHlesiNgeR d. Recurrent optic neu-ritis as the presenting manifestation of primary hypereosino-philic syndrome: a report of two cases. J Neuroophthalmol 2005;25:116-21.39. NuMAgAMi y, toMitA t, MuRAKAMi K, KuBo K, MiCHiHARu N. sinus thrombosis in idiopathic hypere-osinophilic syndrome causing fatal cerebral haemorrhage. J Clin Neurosci 2008;15:585-7. 40. sRiNivAsAN J, esColAR d, RyAN M, dARRAs B, JoNes HR. pediatric sciatic neuropathies due to unusual vascular causes. J Child Neurol 2008;23:738-41.41. NouReeN N, RANA Mt. Hypereosinophilic syndrome causing acute hemiplegia in childhood. J Coll physicians surg park 2008;18:384-5.42. peRiNi gf, KAssAB C, Bley C, MoNZillo pH, tHoMAZ RB, HAMeRsCHlAK N. Acute cerebral in-farction in watershed distribution in a patient with hypereo-sinophilic syndrome. Arq Neuropsiquiatr 2009;67(2B):510-2.43. setHi Hs, sCHMidley Jw. Cerebral infarcts in the set-ting of eosinophilia: three cases and a discussion. Arch Neu-rol 2010;67:1275-7.44. ito K, HARA K, oKAdA t, teRui t. Hypereosino-philic syndrome with various skin lesions and juvenile tempo-ral arteritis. Clin exp dermatol 2009;34(5):e192-5.45. KANAMoRi M, suZuKi H, sAto i, oHyAMA K, teZuKA f, KAtAKuRA R. A case of idiopathic hypereo-sinophilic syndrome with leptomeningeal dissemination and intraventricular mass lesion: an autopsy report. Clin Neuro-pathol 2009;28:197-202. Acta Clin Croat,  Vol. 51,  No. 1,  2012 69Marina titlić et al. Neurologic manifestations of hypereosinophilic syndrome – review of the literaturesažetakNeuRoloŠKe MANifestACiJe HipeReoZiNofilNog siNdRoMA – pRegled liteRAtuReM. Titlić, K. Kodžoman i D. LončarHipereozinofilni sindrom je rijetka bolest hematopoetskog sustava. Bolest je obilježena visokim brojem eozinofila (>1,5x109/l) duže od šest mjeseci, uz uvjet da su isključeni drugi mogući uzroci hipereozinofilije kao što su alergija i para-zitne bolesti, maligne bolesti, Churg-straussova bolest i druge infekcijske bolesti. većina kliničkih manifestacija hipere-ozinofilnog sindroma obuhvaća oštećenja pluća, kože, probavnog sustava, srčane poremećaje i neurološka oštećenja. opi-sana su brojna neurološka oštećenja središnjeg i perifernog živčanog sustava. pretraživanjem literature nalaze se moguće neurološke manifestacije bolesti kao što su mononeuritis multiplex, senzorna polineuropatija, radikulopatija, mijalgija, mi-ozitis i perimiozitis, neuropatija, ataksija, paraplegija, oftalmološki poremećaji, optički neuritis, hemiplegija-hemipareza, spastička tetraplegija, epileptični napadaji, meningitis, ishemijski moždani udar, psihoorganski sindrom i drugi mentalni poremećaji, temporalni arteritis, leptomeningealna diseminacija, poremećaji pamćenja i dizartrija. Ključne riječi: Manifestacije, neurološke; Hipereozinofilni sindrom

Neurološke manifestacije hipereozinofilnog sindroma – pregled literature

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