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.

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.

CT and MR findings of TolosaGHunt syndrome / 实用放射学杂志

Objective To analyze CT and MRI features of Tolosa-Hunt syndrome (THS),to improve the diagnostic accuracy of this lesion.Methods CT and MRI findings of 1 6 patients with THS confirmed clinically were studied retrospectively.Results Eleven out of 1 6 cases’images were abnormal,including unilateral sinus cavernous enlarged asymmetrically,irregular shapes,the outer edges being smooth and straight or slightly bulging,and the inner edges without clear boundary.The maximum diameters of the lesions varied from 1.2 to 3.8 cm,with an average of (2.3 ± 0.9)cm.Ten cases of CT examination showed uniform densities with significant enhancement after enhanced scan.Six patients underwent MR scan and showed equal signals on T1 WI with equal or low signals on T2 WI,equal signals on DWI in 3 patients and obvious enhancement after contrast agent was inj ected.Among 6 cases with MR examination,5 were found to spread along the ipsilateral optic nerve to the orbital apex,4 temporal dura mater were thickened locally and obvious enhancement, 2 super petrosal veins were thickened,and 1 middle cerebral vein was thickened.Conclusion The diagnosis of THS should be considered when the unilateral cavernous sinus is increased asymmetrically,and other diseases are excluded.The helpful imaging feature includes relatively uniform density or signal of the lesion,significant enhancement,the lesion spreading along the optic nerve to the tip of the sac,and the thickening of temporal dura mater and super petrosal vein in the same lateral.The display of THS by MR seems to be superior to CT.

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).

The clinical analysis of recurrent Tolosa-Hunt syndrome / 中国医师进修杂志

Objective To analyze the clinical features, recurrent characters in patients with recurrent Tolosa-Hunt syndrome (THS). Methods The clinical data of 24 hospitalized patients with recurrent THS from January 2006 to May 2016 were collected The general features, clinical manifestations, disease courses, recurrent features, lab and imaging studies, treatment measures and outcoming of recurrent THS patients was investigated , and compared with 69 patients with first attack THS in corresponding period. Results Recurrent THS patients were 25.8%(24/93) of total THS. The male rate in recurrent group was significantly higher than that in first attack group: 66.7%(16/24) vs. 42.0%(29/69), P<0.05. The involved rate of trigeminal nerves in recurrent group was significantly lower than that in first attack group:16.7%(4/24) vs. 33.0%(23/69), P<0.05. The disease courses were from 3 months to 20 years. The total recurrent frequencies were from 2 to 10 times. The recurrence occurred in the same side in 18 patients, and in contralateral in other 6 patients. The intervals were from 3 months to 6 years, and average intervals were 1.9 years. Two patients recurred in hormone reduction, and 22 patients recurred in hormone withdrawal. All cases received MRI examination. Nineteen patients (79.2%) of them had lesions in cavernous sinus. 16 patients had one side lesions and 3 patients had bilateral lesions. The recurrent patients still had good responds to corticosteroids treatment. Conclusions Recurrences in THS are common, taking place in about 26%total patients, and usually at an interval of months or years from the initial attack. These recurrences may be ipsilateral, contralateral, or rarely, bilateral. Corticosteroids are still effective to recurrent cases.

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.

A Case of Cavernous Sinus Dural Arteriovenous Fistula Following Tolosa-Hunt Syndrome

PURPOSE: To report a case of cavernous sinus dural arteriovenous fistula following Tolosa-Hunt syndrome. CASE SUMMARY: A 64-year-old female with a history of hypertension, presented with blepharoptosis and periorbital pain in the right eye and diplopia. Her right pupil was dilated. She had right exotropia and right hypertropia with inability to elevate, depress, and adduct the right eye. Magnetic resonance imaging including angiography, revealed hyperintensities in the right cavernous sinus consistent with inflammation and no vascular abnormalities. Three days after oral corticosteroid therapy, the pain disappeared. A presumptive diagnosis was Tolosa-Hunt syndrome presenting as a form of complete oculomotor nerve palsy. Two months later, she experienced severe pain in the right periocular area, even though blepharoptosis was resolved and ocular motility was improved. A rapid response to high-dose intravenous corticosteroids was consistent with recurrence of Tolosa-Hunt syndrome. Three months later, she had normal ocular motility, but developed a conjunctival injection, pulsatile orbital bruits, and exophthalmos in the right eye. Cerebral angiography showed a cavernous sinus dural arteriovenous fistula. She received a transvenous coil embolization and her symptoms markedly improved over 2 months. CONCLUSIONS: Tolosa-Hunt syndrome, a granulomatous inflammation in the cavernous sinus may be followed by cavernous sinus dural arteriovenous fistula and should be considered during follow-up.

A Case of Tolosa-Hunt Syndrome Presented with Headache, Ptosis and Vision Loss

Cavernous sinus syndrome (CSS) is defined as the involvement of two or more of the third, fourth, fifth (V1, V2) or sixth cranial nerves or involvement of only one of them in combination with a neuroimaging-confirmed lesion in the cavernous sinus. Some cases of CSS are attributed to Tolosa-Hunt syndrome (THS), an idiopathic inflammatory disease of the cavernous sinus. THS is characterized by painful ophthalmoplegia due to granulomatous inflammation in the cavernous sinus. THS is a diagnosis of exclusion that requires a vigorous series of differential diagnoses, and corticosteroid therapy is known to dramatically resolve clinical findings of THS. We report a case of a patient with painful ophthalmoplegia associated with vision loss, which was suspected to be THS. This patient followed a relatively typical clinical course of THS on steroid pulse therapy. We emphasize the differential diagnosis of THS, its presentation, and treatment.

Idiopathic Hypertrophic Cranial Pachymeningitis Masquerading as Tolosa-Hunt Syndrome / 대한임상신경생리학회지

Idiopathic hypertrophic cranial pachymeningitis (ICHP) is diffuse inflammatory process of the dura mater. ICHP can produce similar presentation with Tolosa-Hunt syndrome (THS) if it involves cavernous sinus. A-29-year old male with persistent headache and no definite neurologic dysfunction was noted. Two weeks later, he complained of ophthalmoplegia, and his symptoms were thought to be manifestations of THS. Brain magnetic resonance images revealed diffuse thickened, enhanced pachymeninges in left tentorium. The patient was diagnosed with IHCP. We report a IHCP patient who showed very similar presentation as THS.

An Idiopathic Hypertrophic Tentorial Pachymeningitis Presented as an Alternating Recurrent Painful Ophthalmoplegia

Alternating recurrent painful ophthalmoplegia is caused by various neurological conditions including Tolosa-Hunt syndrome, sellar mass, and parasagittal meningioma. We experienced a rare case of recurrent painful ophthalmoplegia occurring on the contralateral side as a manifestation of idiopathic hypertrophic tentorial pachymeningitis. We propose that idiopathic hypertrophic pachymeningitis should be considered in the differential diagnosis of alternating recurrent painful ophthalmoplegia.

Tolosa-Hunt Syndrome with Contralateral Diffuse Pachymeningeal and Soft Tissue Enhancement

No abstract available.

Tolosa-Hunt Syndrome with Contralateral Diffuse Pachymeningeal and Soft Tissue Enhancement

No abstract available.

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.

Tolosa-Hunt syndrome: a rare case report with uncommon imaging findings and discussion.

A 45 year old diabetic but non hypertensive female presented with unilateral ptosis and complete external ophthalmoplegia on the left side. All the routine investigations were inconclusive. A signal void change was found in MRI study including angiogram of brain. On performing VEP (visually evoked potential), there was mild left optic pathway dysfunction (axonal and demyelinating). A probable diagnosis of Tolosa-Hunt Syndrome (THS) was made and the patient was started on steroids. She responded dramatically to the therapy and was discharged on steroids. Thus the diagnosis of THS was confirmed. On follow up after one month, both her ptosis and ophthalmoplegia had resolved completely. On subsequent follow up visits, she was free of any ophthalmological symptoms and signs.

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.

Cavernous Sinus Dural Arteriovenous Fistula Mimicking Tolosa-Hunt Syndrome

No abstract available.

Tolosa-Hunt Syndrome in a Child / 대한소아신경학회지

Tolosa-Hunt syndrome is a rare disease caused by non-specific inflammation of the cavernous sinus, superior orbital fissure and the apex of the orbit. It is characterized by ophthalmoplegia, unilateral headache or periorbital pain. Brain magnetic resonance imaging (MRI) usually shows an inflammation of cavernous sinus. It is known that steroid pulse therapy is effective. In this case, we diagnosed a 12-year-old female with Tolosa-Hunt syndrome suffered from left ophthalmoplegia and left sided headache. Her Brain MRI showed mild enlargement of left cavernous sinus. After methylprednisolone pulse therapy and following dexamethasone treatment, her symptoms were markedly improved. In children who suffer from periorbiral pain and ophthalmoplegia, proper brain MRI could be helpful in differential diagnosis including Tolosa-Hunt syndrome.

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.