Síndrome de Tolosa-Hunt: dificuldades no diagnóstico e padrão de resposta à prednisona

Carrilho, Paulo Eduardo Mestrinelli; Yamamoto, Fábio Iuji; Scaff, Milberto

doi:10.1590/S0004-282X1997000100016

Resumos

A síndrome de Tolosa-Hunt (STH) consiste de oftalmoplegia dolorosa relacionada a processo inílamatório granulomatoso no seio cavernoso. Seu diagnóstico só pode ser firmado quando outras causas potenciais de oftalmoplegia dolorosa forem descartadas. Resposta satisfatória e padronizada a corticosteróides, associada a evolução benigna, pode reforçar essa possibilidade, embora pseudotumor orbitário e neoplasias do tipo linfoma e até meningioma possam suscitar dúvidas no diagnóstico diferencial, pois também podem apresentar boa resposta a essa medicação. Faz-se necessário, portanto, ampla investigação complementar. Apresentamos estudo clínico-radiológico-laboratorial de cinco pacientes com STH. Prednisona foi utilizada em doses entre 40 e 80 mg/dia. Houve resposta analgésica excelente, com remissão da dor em menos de 48 horas em 4 pacientes. Houve melhora mais lenta da oftalmoplegia, que regrediu completamenteentre 4 e 45 dias em todos os pacientes.

síndrome de Tolosa Hunt; oftalmoplegia; doenças orbitárias; prednisona

The Tolosa-Hunt syndrome (THS) consists of a painful ophthalmoplegia related to granulomatous inflammatory process in the cavernous sinus. According to recent concepts, the diagnosis is established only when other causes of painful ophthalmoplegia are ruled out. A typical pattern of response to corticosteroids associated with a benign evolution may reinforce this possibility. Tumors such as lymphoma and meningioma and orbital pseudotumors can make difficult the differential diagnosis because they also may respond to steroids. Thus it is always necessary to make an extensive ancillary investigation. We performed a clinical, laboratory and radiologic study of five patients with THS. Prednisone was used in all, with dosages ranging from 40 to 80 mg/day. In four patients there was a dramatic analgesic effect in less than 48 hours. Improvement of the ophthalmoplegia was not so fast but occurred in all with a complete remission in 4 to 45 days.

Tolosa-Hunt syndrome; ophthalmoplegia; orbital diseases; prednisone

Síndrome de Tolosa-Hunt: dificuldades no diagnóstico e padrão de resposta à prednisona

Tolosa-Hunt syndrome: troubles in diagnosing and pattern of response to prednisone

Paulo Eduardo Mestrinelli Carrilho^I; Fábio Iuji Yamamoto^II; Milberto Scaff^III

^IMédico pós-graduando. Departamento de Neurologia da Faculdade de Medicina da Universidade de São Paulo (FMUSP)

^IIMestre em Neurologia, Médico Supervisor da Clínica Neurológica do Hospital das Clínicas da FMUSP

^IIIProfessor Titular. Departamento de Neurologia da Faculdade de Medicina da Universidade de São Paulo (FMUSP)

RESUMO

A síndrome de Tolosa-Hunt (STH) consiste de oftalmoplegia dolorosa relacionada a processo inílamatório granulomatoso no seio cavernoso. Seu diagnóstico só pode ser firmado quando outras causas potenciais de oftalmoplegia dolorosa forem descartadas. Resposta satisfatória e padronizada a corticosteróides, associada a evolução benigna, pode reforçar essa possibilidade, embora pseudotumor orbitário e neoplasias do tipo linfoma e até meningioma possam suscitar dúvidas no diagnóstico diferencial, pois também podem apresentar boa resposta a essa medicação. Faz-se necessário, portanto, ampla investigação complementar. Apresentamos estudo clínico-radiológico-laboratorial de cinco pacientes com STH. Prednisona foi utilizada em doses entre 40 e 80 mg/dia. Houve resposta analgésica excelente, com remissão da dor em menos de 48 horas em 4 pacientes. Houve melhora mais lenta da oftalmoplegia, que regrediu completamenteentre 4 e 45 dias em todos os pacientes.

Palavras-chave: síndrome de Tolosa Hunt, oftalmoplegia, doenças orbitárias, prednisona.

ABSTRACT

The Tolosa-Hunt syndrome (THS) consists of a painful ophthalmoplegia related to granulomatous inflammatory process in the cavernous sinus. According to recent concepts, the diagnosis is established only when other causes of painful ophthalmoplegia are ruled out. A typical pattern of response to corticosteroids associated with a benign evolution may reinforce this possibility. Tumors such as lymphoma and meningioma and orbital pseudotumors can make difficult the differential diagnosis because they also may respond to steroids. Thus it is always necessary to make an extensive ancillary investigation. We performed a clinical, laboratory and radiologic study of five patients with THS. Prednisone was used in all, with dosages ranging from 40 to 80 mg/day. In four patients there was a dramatic analgesic effect in less than 48 hours. Improvement of the ophthalmoplegia was not so fast but occurred in all with a complete remission in 4 to 45 days.

Key words: Tolosa-Hunt syndrome, ophthalmoplegia, orbital diseases, prednisone.

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Aceite: 31-outubro-1996.

Dr. Paulo Eduardo Mestrinelli Carrilho - Divisão de Clínica Neurológica, Hospital das Clínicas FMUSP - Avenida Dr. Eneas de Carvalho Aguiar 255, Instituto Central, Sala 5011 - 05403-000 São Paulo SP - Brasil.

1. Aktan S, Aykut C, Erzen C. Computed tomography and magnetic resonance imaging in three patients with Tolosa-Hunt syndrome. Eur Neurol 1993;33:393-396.
2. Goadsby PJ, Lance JW. Clinicopathological correlation in a case of painful ophthalmoplegia: Tolosa-Hunt syndrome. J Neurol Neurosurg Psychiatry 1989;52:1290-1293.
3. Goto Y, Goto I, Hosokawa S. Neurological and radiological studies in painful ophthalmoplegia: Tolosa-Hunt syndrome and orbital pseudotumor. J Neurol 1989;236:448-451.
4. Hannerz J. Recurrent Tolosa-Hunt syndrome. Cephalalgia 1992; 12:45-51.
5. Hunt WE, Brightman RP. The Tolosa-Hunt syndrome: a problem in differential diagnosis. Acta Neurochir 1988;42(Suppl.):248-252.
6. Hunt WE, Meagher JN, LeFever HE, Zeman W. Painful ophthalmoplegia: its relation to indolent inflammation of the cavernous sinus. Neurology 1961; 11:56-62.
7. Kline LB. The Tolosa-Hunt syndrome. Surv Ophthalmol 1982;27:79-95
8. Muhletaler CA, Gerlock AJ. Orbital venography in painful ophthalmoplegia (Tolosa-Hunt). Am J Roetgenol 1979;72:600-616.
9. Nalli DR, Malheiros SMF, Nogueira RG, Abdala N, Caldas JGP, Carrete Jr H, Massaro AR. Avaliação da flebografia orbitáría em oito casos de síndrome de Tolosa-Hunt. Arq Neuropsiquiatr 1994;52:231-236.
10. Rosenbaum DH, Davis MJ, Sony IS. The syndrome of painful ophthalmoplegia: a case with intraorbital mass and hypervascularity. Arch Neurol 1979;36:41-43.
11. Rousseaux P, Peruzzi P, Schaison-Cusin M. Le syndrome de Tolosa-Hunt: doit il encore être un diagnostic délimination ? Rev Neurol (Paris)1993;149:315-319.
12. Spector RH, Fiandaca MD. The "sinister" Tolosa-Hunt syndrome. Neurology 1986;36:198-203.
13. Tolosa E. Periarteritic lesions of the carotid siphon with the clinical features of a carotid infraclinoidal aneurysm. J Neurol Neurosurg Psychiatry 1954;17:300-302.
14. Vailati A, Marene C, Comis S, Anstre L, Sozza E, Galende P, Mastignori E, Bossolo PA, Ascari E. Hashimoto's thyroiditis in association with Tolosa-Hunt syndrome: a case report. Thyroid 1993;3:1257.
15. Youssem DM, Atlas SW, Grosman RI, Sergott RC, Savino PS, Bosley TM. MR imaging of Tolosa-Hunt syndrome. AJNR 1989;10:1181-1184.

Datas de Publicação

Publicação nesta coleção
10 Nov 2010
Data do Fascículo
1997

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

[1] 1. Aktan S, Aykut C, Erzen C. Computed tomography and magnetic resonance imaging in three patients with Tolosa-Hunt syndrome. Eur Neurol 1993;33:393-396.

[2] 2. Goadsby PJ, Lance JW. Clinicopathological correlation in a case of painful ophthalmoplegia: Tolosa-Hunt syndrome. J Neurol Neurosurg Psychiatry 1989;52:1290-1293.

[3] 3. Goto Y, Goto I, Hosokawa S. Neurological and radiological studies in painful ophthalmoplegia: Tolosa-Hunt syndrome and orbital pseudotumor. J Neurol 1989;236:448-451.

[4] 4. Hannerz J. Recurrent Tolosa-Hunt syndrome. Cephalalgia 1992; 12:45-51.

[5] 5. Hunt WE, Brightman RP. The Tolosa-Hunt syndrome: a problem in differential diagnosis. Acta Neurochir 1988;42(Suppl.):248-252.

[6] 6. Hunt WE, Meagher JN, LeFever HE, Zeman W. Painful ophthalmoplegia: its relation to indolent inflammation of the cavernous sinus. Neurology 1961; 11:56-62.

[7] 7. Kline LB. The Tolosa-Hunt syndrome. Surv Ophthalmol 1982;27:79-95

[8] 8. Muhletaler CA, Gerlock AJ. Orbital venography in painful ophthalmoplegia (Tolosa-Hunt). Am J Roetgenol 1979;72:600-616.

[9] 9. Nalli DR, Malheiros SMF, Nogueira RG, Abdala N, Caldas JGP, Carrete Jr H, Massaro AR. Avaliação da flebografia orbitáría em oito casos de síndrome de Tolosa-Hunt. Arq Neuropsiquiatr 1994;52:231-236.

[10] 10. Rosenbaum DH, Davis MJ, Sony IS. The syndrome of painful ophthalmoplegia: a case with intraorbital mass and hypervascularity. Arch Neurol 1979;36:41-43.

[11] 11. Rousseaux P, Peruzzi P, Schaison-Cusin M. Le syndrome de Tolosa-Hunt: doit il encore être un diagnostic délimination ? Rev Neurol (Paris)1993;149:315-319.

[12] 12. Spector RH, Fiandaca MD. The "sinister" Tolosa-Hunt syndrome. Neurology 1986;36:198-203.

[13] 13. Tolosa E. Periarteritic lesions of the carotid siphon with the clinical features of a carotid infraclinoidal aneurysm. J Neurol Neurosurg Psychiatry 1954;17:300-302.

[14] 14. Vailati A, Marene C, Comis S, Anstre L, Sozza E, Galende P, Mastignori E, Bossolo PA, Ascari E. Hashimoto's thyroiditis in association with Tolosa-Hunt syndrome: a case report. Thyroid 1993;3:1257.

[15] 15. Youssem DM, Atlas SW, Grosman RI, Sergott RC, Savino PS, Bosley TM. MR imaging of Tolosa-Hunt syndrome. AJNR 1989;10:1181-1184.