Síndrome de Tolosa-Hunt una extraña causa de oftalmoplejía dolorosa: reporte de caso / Tolosa-Hunt syndrome a strange cause of painful ophthalmoplegia: case report

RESUMEN INTRODUCCIÓN: El síndrome de Tolosa-Hunt (STH) se caracteriza por una oftalmoplejía dolorosa, de etiología desconocida. De acuerdo con los hallazgos histopatológicos, se ha descrito la formación de un tejido granulomatoso en los senos cavernosos. PRESENTACIÓN DEL CASO: Una mujer de 22 años con cuadro clínico de 3 semanas de evolución caracterizado por cefalea hemicránea derecha, dolor ocular derecho y diplopía. Su examen físico evidenció la existencia de una oftalmoplejía derecha; la resonancia magnética (RM) de silla turca demostró engrosamiento y realce en la región del seno cavernoso derecho. Se presenta el caso clínico de una causa inusual de oftalmoplejía dolorosa. DISCUSIÓN: La oftalmoplejía dolorosa tiene múltiples diagnósticos diferenciales que incluyen causas neoplá-sicas, vasculares, inflamatorias e infecciosas que pueden afectar el seno cavernoso o la fisura orbitaria superior. El STH, que es una causa rara de oftalmoplejía dolorosa, sigue siendo un diagnóstico de exclusión. Por otra parte, se caracteriza por tener una adecuada respuesta al tratamiento con glucocorticoides. CONCLUSIÓN: La negatividad en las investigaciones de las etiologías de oftalmoplejía, los hallazgos imagenológicos en la RM y la adecuada respuesta cínica con el uso de los corticoides permiten confirmar el diagnóstico. No debería ser necesaria la biopsia del seno cavernoso ante la sospecha de STH con adecuada respuesta al manejo corticoide.

ABSTRACT INTRODUCTION: Tolosa-Hunt syndrome (THS) is characterized by painful ophthalmoplegia of unknown etiology, the formation of a granulomatous tissue in the cavernous sinuses has been described in histopatho-logical findings. CASE PRESENTATION: A 22-year-old woman presenting with 3 weeks of right sided headache, right eye pain and diplopia. Physical examination revealed the existence of a right ophthalmoplegia; magnetic resonance imaging (MRI) of the sella turcica showed thickening and enhancement of the right cavernous sinus. A clinical case of an unusual cause of painful ophthalmoplegia is presented. DISCUSSION: Painful ophthalmoplegia has multiple differential diagnoses that include neoplastic, vascular, inflammatory and infectious causes that can affect the cavernous sinus or the superior orbital fissure. STH is a rare case of painful ophthalmoplegia that continues to be a diagnosis of exclusion characterized by an adequate response to treatment with glucocorticoids. CONCLUSION: The negativity of the investigations for the causes of ophthalmoplegia, the imaging findings in the MRI and the adequate response to corticosteroids allows the diagnosis to be made. Biopsy should not be necessary when THS is suspected and there is an adequate response to corticosteroid management.

Steroid responsive cavernous sinus syndrome due to Rosai-Dorfman disease: beyond Tolosa-Hunt syndrome - a case report.

BACKGROUND: The term "Tolosa-Hunt syndrome" (THS) has been used to refer to painful ophthalmoplegia associated with nonspecific inflammation of the cavernous sinus and many processes can result in a similar clinical picture, including infectious, inflammatory and neoplastic diseases. Rosai-Dorfman disease (RDD) is a lymphoproliferative disorder that rarely affects the central nervous system. We report a case of isolated CNS Rosai-Dorfman disease involving the cavernous sinus and presenting as "Tolosa-Hunt syndrome". CASE PRESENTATION: Our patient presented with horizontal diplopia due to impairment of cranial nerves III, IV and VI and a stabbing/throbbing headache predominantly in the left temporal and periorbitary regions. There was a nonspecific enlargement of the left cavernous sinus on MRI and the patient had a dramatic response to steroids. Biopsy of a frontal meningeal lesion was compatible with RDD. CONCLUSIONS: We highlight the importance of including Rosai-Dorfman disease as a differential diagnosis in cavernous sinus syndrome and demonstrate a satisfactory long-term response to steroid treatment in this disease.

Total remission of Tolosa-Hunt Syndrome with single-dose of infliximab / Remissão total da Síndrome de Tolosa-Hunt com dose única de infliximabe

Abstract Tolosa-Hunt syndrome is a painful ophthalmoplegia caused by non-specific granulomatous inflammation, corticoid-sensitive, of the cavernous sinus. The etiology is unknown. Recurrences are common. The diagnosis is made by exclusion, and a variety of other diseases involving the orbital apex, superior orbital fissure and cavernous sinus should be ruled out. This study reports a case of a 29-year-old woman, diagnosed with Tolosa-Hunt Syndrome, who presented ophthalmoparesis and orbital pain. She had poor response to corticotherapy and developed colateral effects, so she was treated with single infliximab dose immunosuppression, evolving total remission of the disease.

Resumo A Síndrome de Tolosa-Hunt é uma oftalmoplegia dolorosa causada por uma inflamação granulomatosa não específica, sensível a corticoides, do seio cavernoso. A etiologia é desconhecida. Recorrências são comuns. O diagnóstico é feito por exclusão, devendo ser descartada uma variedade de outras doenças que envolvem o ápice orbitário, fissura orbitária superior e seio cavernoso. O presente estudo trata-se de um relato de caso de uma paciente de 29 anos, diagnosticada com Síndrome de Tolosa-Hunt, que apresentou paresia e dor em região orbital. Obteve resposta pouco efetiva a corticoterapia e desenvolveu efeitos colaterais, por isso foi tratada com dose única de infliximabe, evoluindo com remissão total da doença.

Síndrome de Tolosa-Hunt, uma oftalmoplegia dolorosa / Tolosa Hunt Syndrome, a painful ophthalmoplegia

Resumo A síndrome de Tolosa-Hunt (STH) é uma doença rara caracterizada por oftalmoplegia dolorosa unilateral de início súbito causada por uma inflamação granulomatosa inespecífica no seio cavernoso ou fissura orbital superior (ou ambos). A oftalmoparesia ocorre quando os nervos cranianos III, IV e VI são acometidos pela inflamação. Disfunções pupilares podem estar presentes e está relacionado com acometimento das fibras simpáticas que passam pelo seio cavernoso na porção da artéria carótida interna ou fibras parassimpáticas ao redor do nervo oculomotor. O acometimento do primeiro ramo do trigêmeo pode provocar parestesia território correspondente à distribuição desde ramo (testa). Raramente, pode haver extensão da inflamação para além do seio cavernoso ou fissura orbital superior podendo acometer também o nervo óptico. Há uma boa resposta com o uso de corticoides e pode haver remissões espontâneas. Recidivas ocorrem em 40% dos casos. A doença é mais comum após a segunda década de vida. Afeta ambos os gêneros de forma igualitária. O presente estudo trata-se de um relato de caso de um paciente que se apresentou com oftalmoplegia dolorosa de início súbito à direita com 4 dias de evolução seguido de amaurose ipslateral após um dia do início da dor.

Abstract Tolosa-Hunt syndrome (STH) is a rare disease characterized by sudden onset unilateral painful ophthalmoplegia caused by non-specific granulomatous inflammation in the cavernous sinus or superior orbital fissure (or both). Ophthalmoparesis occurs when the cranial nerves III, IV and VI are affected by inflammation. Pupillary dysfunctions may be present and is related to involvement of the sympathetic fibers that pass through the cavernous sinus in the portion of the internal carotid artery or parasympathetic fibers around the oculomotor nerve. The involvement of the first branch of the trigeminal can cause paresthesia corresponding to the distribution from the first branch (forehead). Rarely, there may be extension of inflammation beyond the cavernous sinus or superior orbital fissure and may also affect the optic nerve. There is a good response with the use of corticosteroids and there may be spontaneous remissions. Relapses occur in 40% of cases. The disease is most common after the second decade of life. It affects both genders equally. The present study is a case report of a patient who presented with painful ophthalmoplegia of sudden onset on the right with 4 days of evolution followed by ipsilateral amaurosis after one day of onset of pain.

Tolosa-Hunt Syndrome / Síndrome de Tolosa-Hunt

Abstract We present a case study of Tolosa-Hunt syndrome, a rare idiopathic disease, that is characterized by painful ophthalmoplegia of strong intensity, generally affecting the third cranial nerve, and, less frequently, the fourth or the sixth cranial nerves. Usually, there is no visual impairment. The treatment is based on corticosteroids with satisfactory results in most cases although recurrences can occur at intervals from months to years. In our case, the patient presented sudden pain periorbital associated with cranial nerves involvement, which have an excellent outcome after treatment with corticosteroids, with no relapses until today.

Resumo Nós apresentamos um caso de Síndrome de Tolosa-Hunt, uma doença idiopática rara, caracterizada por oftalmoplegia dolorosa, de forte intensidade, geralmente afetando o terceiro par craniano, e, menos frequentemente, o quarto e/ou o sexto par. Geralmente, não há acometimento visual. O tratamento é feito com base em corticóides com resultados satisfatórios na maior parte dos casos, embora recorrências possam ocorrer após meses a anos. Relatamos caso de paciente masculino de 36 anos, com diagnóstico prévio de sífilis congênita e esquizofrenia, com dor periocular súbita associada com envolvimento de pares cranianos, que teve melhora total após vigência de corticoterapia, sem recorrências até a presente data.

Síndrome de Tolosa Hunt / Tolosa-Hunt syndrome

RESUMEN El síndrome de Tolosa-Hunt es una entidad poco frecuente cuya etiopatogenia y mecanismos fisiopatológicos se mantienen controversiales. La cefalea asociada a parálisis de uno o más pares craneales, así como diplopía, estrabismo y ptosis palpebral causan un gran temor en el paciente que la padece. Su diagnóstico es por exclusión. Con el objetivo de describir y actualizar el conocimiento sobre esta enfermedad se presenta el cuadro de una paciente que acudió a consulta por cefalea marcada y dolor retroocular. Los hallazgos clínicos y la resonancia magnética confirman el diagnóstico del síndrome de Tolosa Hunt (AU).

ABSTRACT The Tolosa-Hunt syndrome is a few frequent entity whose etiopathogeny and physio-pathological mechanisms are still controversial. The headache associated to the paralysis of one or more cranial paired nerves, and also diplopia, strabismus, and palpebral ptosis cause a great fear in patients suffering it. Its diagnosis is by exclusion. With the objective of describing and up-dating the knowledge on this disease, it is presented the history of a patient who assisted the consultation because of remarked headache and retro-ocular pain. The clinical findings and magnetic resonance confirm the diagnosis of Tolosa-Hunt syndrome (AU).

Síndrome de Tolosa Hunt / Tolosa-Hunt syndrome

RESUMEN El síndrome de Tolosa-Hunt es una entidad poco frecuente cuya etiopatogenia y mecanismos fisiopatológicos se mantienen controversiales. La cefalea asociada a parálisis de uno o más pares craneales, así como diplopía, estrabismo y ptosis palpebral causan un gran temor en el paciente que la padece. Su diagnóstico es por exclusión. Con el objetivo de describir y actualizar el conocimiento sobre esta enfermedad se presenta el cuadro de una paciente que acudió a consulta por cefalea marcada y dolor retroocular. Los hallazgos clínicos y la resonancia magnética confirman el diagnóstico del síndrome de Tolosa Hunt (AU).

ABSTRACT The Tolosa-Hunt syndrome is a few frequent entity whose etiopathogeny and physio-pathological mechanisms are still controversial. The headache associated to the paralysis of one or more cranial paired nerves, and also diplopia, strabismus, and palpebral ptosis cause a great fear in patients suffering it. Its diagnosis is by exclusion. With the objective of describing and up-dating the knowledge on this disease, it is presented the history of a patient who assisted the consultation because of remarked headache and retro-ocular pain. The clinical findings and magnetic resonance confirm the diagnosis of Tolosa-Hunt syndrome (AU).

Síndrome de Tolosa-Hunt / Tolosa-Hunt syndrome

RESUMO A Síndrome de Tolosa Hunt é uma doença rara, cuja etiopatogenia é desconhecida. Apresenta-se como uma oftalmoplegia dolorosa de um ou mais nervos cranianos oculomotores, que regride espontaneamente e responde bem ao tratamento com corticoides. O presente estudo trata-se de um relato de caso de um paciente que apresentou seguidos casos de oftalmoplegias dolorosas, envolvendo o nervo oculomotor e o abducente sendo tratado com corticoesteroides obteve uma resposta dramática. Objetiva-se ainda descrever as características fisiopatológicas, clínicas, o diagnóstico diferencial, visto que é um diagnóstico de exclusão, e medidas terapêuticas instituídas de acordo com o International Headache Society 2004 (ISH-2004) através da apresentação do caso clínico conduzido com as normas do estudo supracitado.

ABSTRACT Tolosa Hunt syndrome is a rare disease, whose etiology is unknown. It presents as a painful ophthalmoplegia of one or more oculomotor cranial nerves, which resolves spontaneously and responds well to treatment with corticosteroids. This study is a case report of a patient who had followed painful oftalmoplegias cases involving the oculomotor and abdcens nerves being treated with corticosteroids, obtaining a dramatic response. Another goal is to describe the pathophysiological, clinical, differential diagnosis, since it is a diagnosis of exclusion, and the therapeutic measures adopted according to the International Headache Society 2004 (ISH-2004) by presenting the case study conducted with the standards the study cited above.

Oftalmoparesia dolorosa episódica asociada a rinosinusitis aguda recurrente: un caso de síndrome de Tolosa Hunt / Painful episodic ophtalmoparesis associated to acute recurrent rhinosinusitis: a case of Tolosa Hunt Syndrome

El síndrome de Tolosa Hunt es un cuadro inflamatorio del seno cavernoso, idiopático y caracterizado por uno o más episodios de dolor orbital unilateral asociado o seguido de paresia oculomotora (afección del III, IV y VI nervio craneal) y en ocasiones con compromiso de la rama maxilar del nervio trigémino. Nosotros presentamos un hombre de 27 años con episodios de oftalmoparesia dolorosa derecha concomitantes a cuadros de rinosinusitis agudas. Su estudio fue negativo y en una de sus recurrencias se encontró en la RM de alta resolución de senos cavernosos, compromiso inflamatorio con captación de gadolinio de los nervios III, IV, V2 y VI derechos. Dado los hallazgos, se planteó el diagnóstico de STH exacerbado por la rinosinusitis e inició tratamiento corticoidal prolongado.

Tolosa Hunt Syndrome is the idiopathic inflammation of cavernous sinus, characterized by one or more episodes of unilateral orbital pain followed by ophtalmoparesis (III, IV o VI nerve palsy) and sometimes the affection of maxillary branch of the trigeminal nerve. We describe the case of a 27 years old man with episodes of painful right ophtalmoparesis associated with acute rhinosinusitis. On high resolution MRI there was inflammation of the III, IV, V2 and VI right nerves with gadolinium enhancement. We propose the THS diagnosis exacerbated by rhinosinusitis and started on chronic steroid therapy.

Síndrome de Tolosa-hunt: reporte de caso y revisión de la literatura / Tolosa-Hunt Syndrome: Case Report And Literature Review

El Síndrome de Tolosa-Hunt es una patología poco frecuente, definida como una inflamación granulomatosa idiopática del seno cavernoso o de la fisura orbitaria superior, caracterizado clínicamente por oftalmoplejía unilateral dolorosa, de evolución aguda o subaguda, asociada a anormalidades en la resonancia magnética de cerebro como el aumento en el volumen y la hipercaptación del medio de contraste del seno cavernoso comprometido. A continuación presentamos el caso de un paciente masculino de 23 años de edad quien consultó al Hospital Universitario del Valle por oftalmoplejía dolorosa de su ojo izquierdo, y en quien se concluyó la existencia de un Síndrome de Tolosa Hunt después de la realización estudios diagnósticos diferenciales exhaustivos.

The Tolosa-Hunt syndrome is an uncommon disease, described as an idiopathic granulomatous inflammation of the cavernous sinus or the superior orbital fissure. Its diagnosis is based on the presence of unilateral ophtalmoplegia associated with the enlargement and the enhanced contrast of the affected cavernus sinus in the MRI. We present the case of a 23-year-old man with ophthalmoplegia of the left eye, who was admitted to the Hospital Universitario del Valle. After performing extensive diagnostic studies, the Tolosa-Hunt syndrome was considered the cause of symptoms.

Oftalmoplejia dolorosa, un desafío diagnóstico: a propósito de un caso clínico de Tolosa-Hunt / Painful ophthalmoplegia, a diagnostic challenge: on a case of Tolosa-Hunt's syndrome

El clínico frente a una oftalmoplejia dolorosa, debe encarar diferentes alternativas diagnósticas, donde la disciplina semiológica y los estudios imagenológicos, son de capital importancia para arribar al diagnóstico. El síndrome de Tolosa-Hunt es una probable etiología, donde el uso de critérios diagnósticos, el estudio por resonancia magnética y la remisión con tratamiento corticoideo, son fundamentales para llegar al mismo, sabiendo que el seguimento evolutivo es de suma importancia para descartar los diagnósticos diferenciales

Oftalmoplejia dolorosa, un desafío diagnóstico: a propósito de un caso clínico de Tolosa-Hunt / Painful ophthalmoplegia, a diagnostic challenge: on a case of Tolosa-Hunt's syndrome

El clínico frente a una oftalmoplejia dolorosa, debe encarar diferentes alternativas diagnósticas, donde la disciplina semiológica y los estudios imagenológicos, son de capital importancia para arribar al diagnóstico. El síndrome de Tolosa-Hunt es una probable etiología, donde el uso de critérios diagnósticos, el estudio por resonancia magnética y la remisión con tratamiento corticoideo, son fundamentales para llegar al mismo, sabiendo que el seguimento evolutivo es de suma importancia para descartar los diagnósticos diferenciales.