Peripheral neuropathy in hypereosinophilic syndrome with vasculitis

Nascimento, Osvaldo; Freitas, Marcos de; Chimelli, Leila; Scaravilli, Francesco

doi:10.1590/S0004-282X1991000400015

Abstracts

A 53-year-old woman with non-productive cough of unexplained aetiology for two years, developed a sub-acute symmetrical polyneuropathy involving all four limbs, accompanied by fever, cutaneous rash and myalgia in lower limbs. Laboratory studies revealed a leukocytosis with 70% eosinophils and excluded any cause for the hypereosinophilia. An echocardiogram showed increase in thickness of the atrial septum. Motor and sensory conduction velocity were reduced in ulnar and median nerve, and unrecordable in peroneal and tibial nerves. A sural nerve biopsy showed an axonal degeneration involving myelinated and unmyelinated fibers as well as a vasculitis with fibrinoid necrosis and perivascular infiltration of eosinophils. There was considerable clinical and laboratorial improvement with the use of steroids. The differential diagnosis between idiopathic hypereosinophilic syndrome and other disorders known to course with vasculitis and hypereosinophilia is discussed.

Uma paciente de 53 anos de idade com tosse não produtiva de etiologia inexplicada durante dois anos desenvolveu polineuropatia subaguda envolvendo os quatro membros, acompanhada de febre, erupção cutânea e mialgia nos membros inferiores. Os estudos laboratoriais revelaram leucocitose com 70% de eosinófilos e excluiram qualquer causa para esta hipereosinofilia. Um ecocardiograma mostrou aumento da espessura do septo atrial. As velocidades de condução motora e sensitiva estavam reduzidas nos nervos ulnar e mediano e não foram registradas nos nervos tibial e fibular. A biópsia do nervo sural mostrou degeneração axonal envolvendo fibras mielínicas e amielínicas, assim como vasculite com necrose fibrinóide e infiltração perivascular de eosinófilos. Ocorreu considerável melhora com o uso de esteróide. O diagnóstico diferencial entre a síndrome hipereosinofílica idiopática e outras afecções que cursam com vasculite e hipereosinofilia é comentado.

CONTENTS CONTEÚDO

Osvaldo NascimentoI; Marcos de FreitasI; Leila ChimelliII; Francesco ScaravilliIII

IDepartment of Clinical Medicine (Neurology)

IIDepartment of Pathology (Neuropathology), Hospital Universitário Antonio Pedro, Universidade Federal Fluminense, Rio de Janeiro, Brazil

IIIDepartment of Neuropathology, Institute of Neurology, London, England

SUMMARY

A 53-year-old woman with non-productive cough of unexplained aetiology for two years, developed a sub-acute symmetrical polyneuropathy involving all four limbs, accompanied by fever, cutaneous rash and myalgia in lower limbs. Laboratory studies revealed a leukocytosis with 70% eosinophils and excluded any cause for the hypereosinophilia. An echocardiogram showed increase in thickness of the atrial septum. Motor and sensory conduction velocity were reduced in ulnar and median nerve, and unrecordable in peroneal and tibial nerves. A sural nerve biopsy showed an axonal degeneration involving myelinated and unmyelinated fibers as well as a vasculitis with fibrinoid necrosis and perivascular infiltration of eosinophils. There was considerable clinical and laboratorial improvement with the use of steroids. The differential diagnosis between idiopathic hypereosinophilic syndrome and other disorders known to course with vasculitis and hypereosinophilia is discussed.

RESUMO

Uma paciente de 53 anos de idade com tosse não produtiva de etiologia inexplicada durante dois anos desenvolveu polineuropatia subaguda envolvendo os quatro membros, acompanhada de febre, erupção cutânea e mialgia nos membros inferiores. Os estudos laboratoriais revelaram leucocitose com 70% de eosinófilos e excluiram qualquer causa para esta hipereosinofilia. Um ecocardiograma mostrou aumento da espessura do septo atrial. As velocidades de condução motora e sensitiva estavam reduzidas nos nervos ulnar e mediano e não foram registradas nos nervos tibial e fibular. A biópsia do nervo sural mostrou degeneração axonal envolvendo fibras mielínicas e amielínicas, assim como vasculite com necrose fibrinóide e infiltração perivascular de eosinófilos. Ocorreu considerável melhora com o uso de esteróide. O diagnóstico diferencial entre a síndrome hipereosinofílica idiopática e outras afecções que cursam com vasculite e hipereosinofilia é comentado.

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Acknowledgements - The authors gratefully acknowledge Prof. P.K. Thomas who gave his valuable opinion about this case. We should like to thank Miss Ana Rodrigues and Rita de Cassia Costa Cunha for technical assistance and Rosely Costa Cunha for the photographic work.

Dr. Osvaldo J. M. Nascimento - Rua Mal. Ramon Castilla 251/903 - 22290 Rio de Janeiro RJ - Brasil.

1. Binaghi M, Gaudric A, Farcet J-P, Coscas G. Artérite rétinienne bilatérale extensive au cours d'un syndrome hypereosinophilique chez un malade porteur d'ne tuberculose. Bull Soc Opht France, 1982, 82:59-63.
2. Binaghi M, Perrenoud F, Dhermy P, Coscas G. Syndrome hypereosinophilique avec atteinte oculaire. J Fr Ophtalmol 1985, 8:309-314.
3. Chumbley LC, Harrison EG Jr, DeRemee RA. Allergic granulomatosis and angiitis (Churg-Strauss syndrome): report and analysis of 30 cases. Mayo Clin Proc 1977, 52:477-484.
4. Chusid MJ, Dale DC. Eosinophilic leukemia : remission with vincristine and hydroxyurea. Am J Med 1975, 59:297-300.
5. Chusid MJ, Dale DC, West BC, Wolff SM. The hypereosinophilic syndrome : analysis of fourteen cases with review of the literature. Medicine 1975, 54:1-27.
6. Dorfman LJ, Ransom BR, Forno LS, Kelts A. Neuropathy in the hypereosinophilic syndrome. Muscle & Nerve 1983, 6:291-298.
7. Dyck PJ, Benstead TJ, Conn DL, Stevens JC, Windebank AJ, Low PA. Non-systemic vasculitic neuropathy. Brain 1987, 110:843-854.
8. Even P, Sors H, Venet A, Dennewald G, Reynald P. Angéite granulomateuse allergique de Churg-Strauss, granulomatose de Wegener et vascularites pulmonaires apparentées. In Maladies Systémiques Paris; Flammarion 1986, p 446-483.
9. Fauci AS, Harley JB, Roberts WC, Ferrans VJ, Gralnick HR, Bjornson BH. The idiopathic hypereosinophilic syndrome : clinical, pathophysiologic and therapeutic considerations. Ann Intern Med 1982, 97:78-92.
10. Godeau P, Herreman G, de Saint-Maur P, Guillevin L. Périartérite noueuse et autres angéites nécrosantes. J Mal Vasc (Paris) 1977, 2 239-276.
11. Grisold W, Jellinger K. Multifocal neuropathy with vasculitis in hypereosinophilic syndrome : an entity or drug-induced effect? J Neurol 1985, 231:301-306.
12. Hardy WE, Anderson RE. The hypereosinophilic syndromes. Ann Intern Med 1968, 68:1220-1229.
13. Kazmierowski LJ, Chusid MJ, Parrillo JE, Fauci AS, Wolff SM. Dermatologic manifestations of the hypereosinophilic syndrome. Arch Dermatol 1978, 114:531-535.
14. Marolda M, Orefice G, Barbieri F, Striano S, Camporeale FS, Santagelo R, Catalano A, Crisci C, Ragno M, Cioffi M, DeMattia G. The idiopathic hypereosinophilic syndrome: clinical, electrophysiologic and histologic study of a case. CI Neuropathol 1988, 7:186 (abstr).
15. McCluskey RT, Fienberg R. Vasculitis in primary vasculitides, granulomatoses, and connective tissue diseases. Hum Pathol 1983, 14:305-315.
16. Monaco S, Lucci B, Laperchia N, Tezzon F, Curro-Dossi B, Nardelli E, Giannini G, Rizzuto N. Polyneuropathy in hypereosinophilic syndrome. Neurology 1988, 38:494-496.
17. Moore PM, Cupps TR. Neurological complications of vasculitis. Ann Neurol 1983, 14:155-167.
18. Moore PM, Harley JB, Fauci AS. Neurological dysfunction in the idiopathic hypereosinophilic syndrome. Ann Inter Med 1985, 102:109-114.
19. Samoszuk MK, Nathwani BN, Lukes RJ. Extensive deposition of eosinophil peroxidase in Hodgkin's and non-Hodgkin's lymphomas. Am J Pathol 1986, 125:426-429.
20. Spry CJF, Davis J, Tai PC, Olsen EGJ, Oakley CM, Goodwin JF. Clinical features of fifteen patients with the hypereosinophilic syndrome. Q J Med 1983, 52:1-22.
21. Vital A, Vital C. Polyarteritis nodosa and peripheral neuropathy: ultrastructural study of 13 cases. Acta Neuropathol (Berlin) 1985, 67:136-141.
22. Weaver DF, Heffernan LP, Purdy RA, Ing VW. Eosinophil-induced neurotoxicity : axo-nal neuropathy, cerebral infarction, and dementia. Neurology 1988, 38:144-146.
23. Wees SJ, Sunwood IN, Oh SJ. (1981) Sural nerve biopsy in systemic necrotizing vasculitis. Am J Med 1981, 71:525-532.
24. Wichman A, Buchthal F, Pezeshkpour GH, Fauci AS. Peripheral neuropathy in hypereosinophilic syndrome. Neurology 1985, 35:1140-1145.

Peripheral neuropathy in hypereosinophilic syndrome with vasculitis

Neuropatia periférica na síndrome hipereosinofílica com vasculite

Publication Dates

Publication in this collection
22 Feb 2011
Date of issue
Dec 1991

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

[1] 1. Binaghi M, Gaudric A, Farcet J-P, Coscas G. Artérite rétinienne bilatérale extensive au cours d'un syndrome hypereosinophilique chez un malade porteur d'ne tuberculose. Bull Soc Opht France, 1982, 82:59-63.

[2] 2. Binaghi M, Perrenoud F, Dhermy P, Coscas G. Syndrome hypereosinophilique avec atteinte oculaire. J Fr Ophtalmol 1985, 8:309-314.

[3] 3. Chumbley LC, Harrison EG Jr, DeRemee RA. Allergic granulomatosis and angiitis (Churg-Strauss syndrome): report and analysis of 30 cases. Mayo Clin Proc 1977, 52:477-484.

[4] 4. Chusid MJ, Dale DC. Eosinophilic leukemia : remission with vincristine and hydroxyurea. Am J Med 1975, 59:297-300.

[5] 5. Chusid MJ, Dale DC, West BC, Wolff SM. The hypereosinophilic syndrome : analysis of fourteen cases with review of the literature. Medicine 1975, 54:1-27.

[6] 6. Dorfman LJ, Ransom BR, Forno LS, Kelts A. Neuropathy in the hypereosinophilic syndrome. Muscle & Nerve 1983, 6:291-298.

[7] 7. Dyck PJ, Benstead TJ, Conn DL, Stevens JC, Windebank AJ, Low PA. Non-systemic vasculitic neuropathy. Brain 1987, 110:843-854.

[8] 8. Even P, Sors H, Venet A, Dennewald G, Reynald P. Angéite granulomateuse allergique de Churg-Strauss, granulomatose de Wegener et vascularites pulmonaires apparentées. In Maladies Systémiques Paris; Flammarion 1986, p 446-483.

[9] 9. Fauci AS, Harley JB, Roberts WC, Ferrans VJ, Gralnick HR, Bjornson BH. The idiopathic hypereosinophilic syndrome : clinical, pathophysiologic and therapeutic considerations. Ann Intern Med 1982, 97:78-92.

[10] 10. Godeau P, Herreman G, de Saint-Maur P, Guillevin L. Périartérite noueuse et autres angéites nécrosantes. J Mal Vasc (Paris) 1977, 2 239-276.

[11] 11. Grisold W, Jellinger K. Multifocal neuropathy with vasculitis in hypereosinophilic syndrome : an entity or drug-induced effect? J Neurol 1985, 231:301-306.

[12] 12. Hardy WE, Anderson RE. The hypereosinophilic syndromes. Ann Intern Med 1968, 68:1220-1229.

[13] 13. Kazmierowski LJ, Chusid MJ, Parrillo JE, Fauci AS, Wolff SM. Dermatologic manifestations of the hypereosinophilic syndrome. Arch Dermatol 1978, 114:531-535.

[14] 14. Marolda M, Orefice G, Barbieri F, Striano S, Camporeale FS, Santagelo R, Catalano A, Crisci C, Ragno M, Cioffi M, DeMattia G. The idiopathic hypereosinophilic syndrome: clinical, electrophysiologic and histologic study of a case. CI Neuropathol 1988, 7:186 (abstr).

[15] 15. McCluskey RT, Fienberg R. Vasculitis in primary vasculitides, granulomatoses, and connective tissue diseases. Hum Pathol 1983, 14:305-315.

[16] 16. Monaco S, Lucci B, Laperchia N, Tezzon F, Curro-Dossi B, Nardelli E, Giannini G, Rizzuto N. Polyneuropathy in hypereosinophilic syndrome. Neurology 1988, 38:494-496.

[17] 17. Moore PM, Cupps TR. Neurological complications of vasculitis. Ann Neurol 1983, 14:155-167.

[18] 18. Moore PM, Harley JB, Fauci AS. Neurological dysfunction in the idiopathic hypereosinophilic syndrome. Ann Inter Med 1985, 102:109-114.

[19] 19. Samoszuk MK, Nathwani BN, Lukes RJ. Extensive deposition of eosinophil peroxidase in Hodgkin's and non-Hodgkin's lymphomas. Am J Pathol 1986, 125:426-429.

[20] 20. Spry CJF, Davis J, Tai PC, Olsen EGJ, Oakley CM, Goodwin JF. Clinical features of fifteen patients with the hypereosinophilic syndrome. Q J Med 1983, 52:1-22.

[21] 21. Vital A, Vital C. Polyarteritis nodosa and peripheral neuropathy: ultrastructural study of 13 cases. Acta Neuropathol (Berlin) 1985, 67:136-141.

[22] 22. Weaver DF, Heffernan LP, Purdy RA, Ing VW. Eosinophil-induced neurotoxicity : axo-nal neuropathy, cerebral infarction, and dementia. Neurology 1988, 38:144-146.

[23] 23. Wees SJ, Sunwood IN, Oh SJ. (1981) Sural nerve biopsy in systemic necrotizing vasculitis. Am J Med 1981, 71:525-532.

[24] 24. Wichman A, Buchthal F, Pezeshkpour GH, Fauci AS. Peripheral neuropathy in hypereosinophilic syndrome. Neurology 1985, 35:1140-1145.