Wall-eyed bilateral internuclear ophthalmoplegia (webino syndrome) and myelopathy in pyoderma gangrenosum

Lana, Marco Aurélio; Moreira, Paulo Roberto R.; Neves, Leonardo B.

doi:10.1590/S0004-282X1990000400016

Abstracts

A 35-year-old female with pyoderma gangrenosum developed paraparesis with a sensory level at L1. Three months later she complained of diplopia and was found to have bilateral internuclear ophthalmoplegia with exotropia and no ocular convergence. The term Webino syndrome has been coined to design this set of neuro-opthalmologic findings. Although it was initially attributed to lesions affecting the medial longitudinal fasciculus and the medial rectus subnuclei of the oculomotor complex in the midbrain the exact location of the lesion is still disputed. In the present case both myelopathy and Webino syndrome were probably due to vascular occlusive disease resulting from central nervous system vasculitis occurring in concomitance to pyoderma gangrenosum.

O termo Webino é formado pelas iniciais dos componentes da síndrome (walleyed bilateral internuclear ophthalmoplegia), havendo também perda da convergência ocular. Relatamos o caso de uma paciente de 35 anos de idade, com pioderma gangrenosum, que desenvolveu subitamente mielopatia com nível sensitivo em L1 e, três meses depois, quadro súbito de oftalmoplegia internuclear bilateral, exotropia e perda de convergência. Há poucos casos de síndrome de Webino relatados na literatura, a maioria deles secundária a esclerose múltipla e a doença vascular cerebral. A localização da lesão responsável pela síndrome ainda não está bem estabelecida mas, acredita-se que os fascículos longitudinais mediais e os subnúcleos dos retos mediais do complexo oculomotor no mesencéfalo sejam afetados, embora não haja ainda confirmação anátomo-patológica. O pioderma gangrenosum é condição caracterizada por úlceras de crescimento rápido e de bordas elevadas com halo eritematoso localizadas preferencialmente na cabeça, tronco e membros inferiores. Embora sua natureza ainda seja desconhecida, parece ser secundário a vasculite subjacente, estando associado a grande variedade de doenças sistêmicas. No caso presente, a ocorrência da síndrome de Webino e de mielopatia sugere aue as lesões no sistema nervoso sejam de natureza vascular, provavelmente secundárias a vasculite. Este é o primeiro relato da literatura de complicações neurológicas do pioderma gangrenosum e da associação de mielopatia à síndrome. de Webino.

Marco Aurélio Lana^I; Paulo Roberto R. Moreira^II; Leonardo B. Neves^III

^IAssociated Professor of Neurology - Federal University of Minas Gerais Medical School (UFMG)

^IINeurologist - Federal University of Minas Gerais Medical School (UFMG)

^IIIDermatologist - Federal University of Minas Gerais Medical School (UFMG)

SUMMARY

A 35-year-old female with pyoderma gangrenosum developed paraparesis with a sensory level at L1. Three months later she complained of diplopia and was found to have bilateral internuclear ophthalmoplegia with exotropia and no ocular convergence. The term Webino syndrome has been coined to design this set of neuro-opthalmologic findings. Although it was initially attributed to lesions affecting the medial longitudinal fasciculus and the medial rectus subnuclei of the oculomotor complex in the midbrain the exact location of the lesion is still disputed. In the present case both myelopathy and Webino syndrome were probably due to vascular occlusive disease resulting from central nervous system vasculitis occurring in concomitance to pyoderma gangrenosum.

RESUMO

O termo Webino é formado pelas iniciais dos componentes da síndrome (walleyed bilateral internuclear ophthalmoplegia), havendo também perda da convergência ocular. Relatamos o caso de uma paciente de 35 anos de idade, com pioderma gangrenosum, que desenvolveu subitamente mielopatia com nível sensitivo em L1 e, três meses depois, quadro súbito de oftalmoplegia internuclear bilateral, exotropia e perda de convergência. Há poucos casos de síndrome de Webino relatados na literatura, a maioria deles secundária a esclerose múltipla e a doença vascular cerebral. A localização da lesão responsável pela síndrome ainda não está bem estabelecida mas, acredita-se que os fascículos longitudinais mediais e os subnúcleos dos retos mediais do complexo oculomotor no mesencéfalo sejam afetados, embora não haja ainda confirmação anátomo-patológica. O pioderma gangrenosum é condição caracterizada por úlceras de crescimento rápido e de bordas elevadas com halo eritematoso localizadas preferencialmente na cabeça, tronco e membros inferiores. Embora sua natureza ainda seja desconhecida, parece ser secundário a vasculite subjacente, estando associado a grande variedade de doenças sistêmicas. No caso presente, a ocorrência da síndrome de Webino e de mielopatia sugere aue as lesões no sistema nervoso sejam de natureza vascular, provavelmente secundárias a vasculite. Este é o primeiro relato da literatura de complicações neurológicas do pioderma gangrenosum e da associação de mielopatia à síndrome. de Webino.

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Dr. Marco Aurélio Lana - Departamento de Neurologia, Faculdade de Medicina da UFMG -Av. Alfredo Balena 190 - 30130 Belo Horizonte MO - Brasil.

1. Baker RS - Internuclear ophthalmoplegia following head injury. J Neurosurg 51:552, 1979.
2. Baloh RW, Yee RD, Honrubia B - Internuclear ophthalmoplegia: 1. Saccades and dissociated nystagmus. Arch Neurol 35:484, 1978.
3. Bender MB, Weinstein EA - Dissociated monocular nystagmus with paresis of horizontal ocular movements. Arch Ophthalmol 21:228, 1939.
4. Christoff N, Anderson PJ, Nathanson M - Problems in anatomic analysis of lesions of the medial longitudinal fasciculus. Arch Neurol 2:293, 1960.
5. Cogan DG - Internuclear ophthalmoplegia, typical and atypical. Arch Ophthalmol 84:583, 1970.
6. Cogan DG, Wray S - Internuclear ophthalmoplegia as an early sign of brainstem tumors. Neurology 20:629, 1970.
7. Cogan M, Klinne LB, Duvall ER - Bilateral internuclear ophthalmoplegia in systemic lupus erythematosus. J Clin Neuro-Ophthalmol 7:69, 1987.
8. Deveraux MW, Brust JCM, Keane JR - Internuclear ophthalmoplegia caused by subdural hematoma. Neurology 27:251, 1979.
9. Ford SC. Cruz J, Biller J, Wayme L, White DR - Bilateral internuclear ophthalmoplegia in carcinomatous meningitis. J Clin Neuro-Ophthalmol 3:127, 1983.
10. Gonyea EF - Bilateral internuclear ophthalmoplegia: association with occlusive cerebrovascular disease. Arch Neurol 31:168, 1974.
11. Gonyea EF, Helman KM - Neuro-ophthalmic aspects of central nervous system cryptococcosis. Arch Ophthalmol 87:164. 1972.
12. Guilhou JJ, Guillot B, Meynadier J - Pyoderma gangrenosum: 14 observations personnelles et revue de la literature. J Maladies Vase 12:202, 1987.
13. Harrington RB, Hollenhorst RW, Sayre GP - Unilateral internuclear ophthalmoplegia. Arch Neurol 15:29, 1966.
14. Henn V, Buttner-Ennever JA, Kepp K - The primate oculomotor system: I. Motoneurons: a synthesis of anatomical, physiological and clinical data. Hum Neurobiol 1:77, 1982.
15. Holt PJA, Davies MG, Saunders KC, Nuki G - Pyoderma gangrenosum: clinical and laboratory findings in 15 patients with special reference to polyarthritis. Medicine 59:114, 1980.
16. Inocencio FP, Baceller R - Tuberculosis granuloma in the midbrain causing wall-eyed bilateral internuclear ophthalmoplegia (Webino). J Clin Neuro-Ophthalmol 5:31, 1985.
17. Kupfer C, Cogan DG - Unilateral internuclear ophthalmoplegia: a clinicopathological case report. Arch Ophthalmol 75:484, 1966.
18. Lepore FE, Nissenblatt MJ - Bilateral internuclear ophthalmoplegia after intratecal chemotherapy and cranial irradiation. Am J Ophthalmol 92:851, 1981.
19. MacDonald JJ, Sher PK - Ophthalmoplegia as a sign of metabolic disease in the newborn. Neurology 27:971, 1977.
20. McGettrick P, Eustace P - The webino syndrome. Neuro-Ophthalmol 5:109, 1985.
21. Miller NR - Walsh and Hoyt's Clinical Neuro-Ophthalmology. Ed 4. Williams and Wilkins, Baltimore, 1985.
22. Muskens LJJ - An anatomico-physiological study of the posterior longitudinal bundle in its relation to forced movements. Brain 36:362, 1914.
23. Plum F, Posner JB - Diagnosis of Stupor and Coma. Ed 3. F.A. Davis, Philadelphia, 1980.
24. Pola J, Robinson DA - An explanation of eye movements seen in internuclear ophthalmoplegia. Arch Neurol 33:447, 1976.
25. Rich JR, Gregorius FK, Hepler RS - Bilateral internuclear ophthalmoplegia after trauma. Arch Ophthalmol 92:66, 1974.
26. Rizzo M, Corbett J - Bilateral internuclear ophthalmoplegia reversed by naloxone. Arch Neurol 40:242, 1983.
27. Simon RP - Forced downward ocular deviation occurrence during oculo-vestibular testing in sedative drug-induced coma. Arch Neurol 35:456, 1978.
28. Smith JL - Internuclear ophthalmoplegia. J Clin Neuro-Ophthhalmol 6:66, 1986.
29. Smith JL, Cogan DG - Internuclear ophthalmoplegia: a review of 58 cases. Arch Ophthalmol 61:687, 1959.
30. Spector RH, Davidoff RA, Schwartzman RJ - Phenytoin-induced ophthalmoplegia. Neurology 26:1031, 1976.
31. Spiller WG - Ophthalmoplegia internuclearis anterior: a case with necropsy. Brain 47:345, 1924.
32. Strominger MB, Miney EJ, Strominger AI, Strominger ND - Bilateral internuclear ophthalmoplegia with absence of convergent eye movements: clinicopathologic correlation. J Clin Neuro-Ophthalmol 6:57, 1986.
33. Strond MH, Newman NM, Kcltner JL* Gay AJ - Abducting nystagmus in the medial longitudinal fasciculus (MLF) syndrome-intemuclear ophthalmoplegia (INO). Arch Ophthalmol 92:2, 1974.
34. Su WPD, Schroeter AD, Perry HO, Powell FC - Histopathologic and immunopathologic study of pyoderma gangrenosum. J Cut Pathol 13:323, 1986.
35. Weiner LP, Konigsmack BW, Stoll J, Magladery JW - Hereditary olivopontocerebellar atrophy with retinal degeneration: report of a family through six generations. Arch Neurol 16:364, 1976.
36. Wilson SAK - Case no 3, presented at the clinical meeting of the Neurological Society of the United Kindgom, on March 29, 1906. Brain 27:298, 1906.
37. Yee RD, Cogan DG, Zee WS - Ophthalmoplegia and dissociated nystagmus in abetalipoproteinemia. Arch Ophthalmol 94:571, 1976.

Wall-eyed bilateral internuclear ophthalmoplegia (webino syndrome) and myelopathy in pyoderma gangrenosum

Oftalmoplegia internuclear bilateral e exotropia (síndrome de Webino) e mielopatia no pyoderma gangrenosum

Publication Dates

Publication in this collection
19 May 2011
Date of issue
Dec 1990

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

[1] 1. Baker RS - Internuclear ophthalmoplegia following head injury. J Neurosurg 51:552, 1979.

[2] 2. Baloh RW, Yee RD, Honrubia B - Internuclear ophthalmoplegia: 1. Saccades and dissociated nystagmus. Arch Neurol 35:484, 1978.

[3] 3. Bender MB, Weinstein EA - Dissociated monocular nystagmus with paresis of horizontal ocular movements. Arch Ophthalmol 21:228, 1939.

[4] 4. Christoff N, Anderson PJ, Nathanson M - Problems in anatomic analysis of lesions of the medial longitudinal fasciculus. Arch Neurol 2:293, 1960.

[5] 5. Cogan DG - Internuclear ophthalmoplegia, typical and atypical. Arch Ophthalmol 84:583, 1970.

[6] 6. Cogan DG, Wray S - Internuclear ophthalmoplegia as an early sign of brainstem tumors. Neurology 20:629, 1970.

[7] 7. Cogan M, Klinne LB, Duvall ER - Bilateral internuclear ophthalmoplegia in systemic lupus erythematosus. J Clin Neuro-Ophthalmol 7:69, 1987.

[8] 8. Deveraux MW, Brust JCM, Keane JR - Internuclear ophthalmoplegia caused by subdural hematoma. Neurology 27:251, 1979.

[9] 9. Ford SC. Cruz J, Biller J, Wayme L, White DR - Bilateral internuclear ophthalmoplegia in carcinomatous meningitis. J Clin Neuro-Ophthalmol 3:127, 1983.

[10] 10. Gonyea EF - Bilateral internuclear ophthalmoplegia: association with occlusive cerebrovascular disease. Arch Neurol 31:168, 1974.

[11] 11. Gonyea EF, Helman KM - Neuro-ophthalmic aspects of central nervous system cryptococcosis. Arch Ophthalmol 87:164. 1972.

[12] 12. Guilhou JJ, Guillot B, Meynadier J - Pyoderma gangrenosum: 14 observations personnelles et revue de la literature. J Maladies Vase 12:202, 1987.

[13] 13. Harrington RB, Hollenhorst RW, Sayre GP - Unilateral internuclear ophthalmoplegia. Arch Neurol 15:29, 1966.

[14] 14. Henn V, Buttner-Ennever JA, Kepp K - The primate oculomotor system: I. Motoneurons: a synthesis of anatomical, physiological and clinical data. Hum Neurobiol 1:77, 1982.

[15] 15. Holt PJA, Davies MG, Saunders KC, Nuki G - Pyoderma gangrenosum: clinical and laboratory findings in 15 patients with special reference to polyarthritis. Medicine 59:114, 1980.

[16] 16. Inocencio FP, Baceller R - Tuberculosis granuloma in the midbrain causing wall-eyed bilateral internuclear ophthalmoplegia (Webino). J Clin Neuro-Ophthalmol 5:31, 1985.

[17] 17. Kupfer C, Cogan DG - Unilateral internuclear ophthalmoplegia: a clinicopathological case report. Arch Ophthalmol 75:484, 1966.

[18] 18. Lepore FE, Nissenblatt MJ - Bilateral internuclear ophthalmoplegia after intratecal chemotherapy and cranial irradiation. Am J Ophthalmol 92:851, 1981.

[19] 19. MacDonald JJ, Sher PK - Ophthalmoplegia as a sign of metabolic disease in the newborn. Neurology 27:971, 1977.

[20] 20. McGettrick P, Eustace P - The webino syndrome. Neuro-Ophthalmol 5:109, 1985.

[21] 21. Miller NR - Walsh and Hoyt's Clinical Neuro-Ophthalmology. Ed 4. Williams and Wilkins, Baltimore, 1985.

[22] 22. Muskens LJJ - An anatomico-physiological study of the posterior longitudinal bundle in its relation to forced movements. Brain 36:362, 1914.

[23] 23. Plum F, Posner JB - Diagnosis of Stupor and Coma. Ed 3. F.A. Davis, Philadelphia, 1980.

[24] 24. Pola J, Robinson DA - An explanation of eye movements seen in internuclear ophthalmoplegia. Arch Neurol 33:447, 1976.

[25] 25. Rich JR, Gregorius FK, Hepler RS - Bilateral internuclear ophthalmoplegia after trauma. Arch Ophthalmol 92:66, 1974.

[26] 26. Rizzo M, Corbett J - Bilateral internuclear ophthalmoplegia reversed by naloxone. Arch Neurol 40:242, 1983.

[27] 27. Simon RP - Forced downward ocular deviation occurrence during oculo-vestibular testing in sedative drug-induced coma. Arch Neurol 35:456, 1978.

[28] 28. Smith JL - Internuclear ophthalmoplegia. J Clin Neuro-Ophthhalmol 6:66, 1986.

[29] 29. Smith JL, Cogan DG - Internuclear ophthalmoplegia: a review of 58 cases. Arch Ophthalmol 61:687, 1959.

[30] 30. Spector RH, Davidoff RA, Schwartzman RJ - Phenytoin-induced ophthalmoplegia. Neurology 26:1031, 1976.

[31] 31. Spiller WG - Ophthalmoplegia internuclearis anterior: a case with necropsy. Brain 47:345, 1924.

[32] 32. Strominger MB, Miney EJ, Strominger AI, Strominger ND - Bilateral internuclear ophthalmoplegia with absence of convergent eye movements: clinicopathologic correlation. J Clin Neuro-Ophthalmol 6:57, 1986.

[33] 33. Strond MH, Newman NM, Kcltner JL* Gay AJ - Abducting nystagmus in the medial longitudinal fasciculus (MLF) syndrome-intemuclear ophthalmoplegia (INO). Arch Ophthalmol 92:2, 1974.

[34] 34. Su WPD, Schroeter AD, Perry HO, Powell FC - Histopathologic and immunopathologic study of pyoderma gangrenosum. J Cut Pathol 13:323, 1986.

[35] 35. Weiner LP, Konigsmack BW, Stoll J, Magladery JW - Hereditary olivopontocerebellar atrophy with retinal degeneration: report of a family through six generations. Arch Neurol 16:364, 1976.

[36] 36. Wilson SAK - Case no 3, presented at the clinical meeting of the Neurological Society of the United Kindgom, on March 29, 1906. Brain 27:298, 1906.

[37] 37. Yee RD, Cogan DG, Zee WS - Ophthalmoplegia and dissociated nystagmus in abetalipoproteinemia. Arch Ophthalmol 94:571, 1976.