Review of Tolosa-Hunt Syndrome, Recent Updates.

PURPOSE OF REVIEW: We aim to present diagnostic considerations and management insights on Tolosa-Hunt syndrome. We highlight recent controversies in this field and emerging literature. RECENT FINDINGS: The diagnostic categorization of Tolosa-Hunt syndrome remains controversial, with imaging negative cases described in the literature and an increasing literature of secondary causes falsely diagnosed as Tolosa-Hunt syndrome. Response to steroids can fulfill diagnostic criteria, but newer management strategies are available in treatment-resistant patients, such as steroid-sparing agents or radiotherapy. Tolosa-Hunt syndrome has become controversial; the entity of granulomatous inflammation of the cavernous sinus and possible extension into orbital fissure and posterior orbit of late has been re-evaluated. Recent case series and reviews have outlined multiple false positive (neoplasm or infection) and negative (no imaging findings) diagnoses. In the future, when assessing whether a patient has this entity, we caution readers to closely follow patients for secondary causes and consider this entity in differential even if MRI is non-revealing. Additionally, biopsy should be regarded as a gold standard for diagnosis and utilized, especially in uncertain cases. The diagnostic categorization of Tolosa-Hunt syndrome may need reconsideration in future versions of the ICHD.

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.

Bilateral Tolosa-Hunt syndrome mimicking pituitary adenoma.

The authors report a rare case of bilateral Tolosa-Hunt syndrome, which occurred in a 80-year-old female and remitted spontaneously. Inflammatory lesions were found not only in typical locations, i.e. superior orbital fissures and cavernous sinuses, but also in the pituitary; these imitated gland's macroadenoma in imaging studies.

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.

Unusual clinical course in pediatric Tolosa-Hunt syndrome.

We report on a 7-year-old immunocompetent boy initially presenting with right-sided frontal cephalalgia, painful ophthalmoplegia, and ptosis for 1 month. Initial cerebrospinal fluid analysis produced normal results. Magnetic resonance imaging revealed an inflammatory pseudotumor of the right cavernous sinus after intravenous gadolinium administration, indicating a rare idiopathic inflammatory disorder of the cavernous sinus, i.e., Tolosa-Hunt syndrome. Ptosis and cephalalgia resolved after steroid treatment, although right-sided ophthalmoplegia remained. Breakthrough headache, associated with signs of meningeal irritation, developed 6 weeks later. Follow-up contrast-enhanced computed tomography revealed no enhancing cavernous soft tissue mass. A further lumbar puncture disclosed central nervous system infection with Staphylococcus saprophyticus. After 6 weeks of vancomycin, the headache resolved completely, and neuroimaging produced normal results. A diagnosis of Tolosa-Hunt syndrome should be rendered cautiously, because the etiology may involve a rare but not "idiopathic" infection. Moreover, if clinical signs are not fully responsive to steroid treatment, the underlying problems should receive careful investigation.

[Tolosa-Hunt syndrome: a case report]. / Tolosa-Huntov sindrom: prikaz bolesnika.

Tolosa-Hunt syndrome is a nonspecific granulomatous inflammation of the cavernous sinus, superior orbital fissure and apex of the orbit. It involves episodes of unilateral orbital pain which may last several weeks, lesions of cranial nerve III, IV or VI, and rarely II, V, VII and VIII. It is characterized by remissions and exacerbations, and can cause permanent neurological disorder of the affected nerves. We present the course of the disease in a patient whose initial symptom was gradual visual loss in the right eye, followed by neuralgic pain in the right orbit and face. The diagnosis was confirmed by biopsy and corticosteroid therapy was administered. Exacerbation of the disease required repeated surgery, excision of the fibrous tissue of the cavernous sinus, as well as prolonged corticosteroid therapy.

[Simultaneous presentation of Tolosa-Hunt syndrome and oculomotor nerve palsy due to the nonruptured internal carotid-posterior communicating artery aneurysm: a case report].

A 45-year-old female developed mild dysesthesia and swelling, followed by ptosisand trigeminal pain, in the right side of the face. Her past medical history was unremarkable, and she had not been aware of any infectious sign. A local otolaryngologist administered glucocorticoid therapy that resolved the face pain, but the ptosis persisted. Neurological examination found complete right oculomotor nerve paresis and mild sensory loss in the first and second segments of the right trigeminal nerve. Blood examination found no abnormalities. Neuroimaging revealed a saccular aneurysm at the branching site of the posterior communicating artery, projecting posteriorly and adjacent to the dorsum sellae, without other intracranial abnormalities. Cerebral angiography demonstrated poor opacification of the superior ophthalmic vein and cavernous sinus on the right side. The patient underwent coil embolization under a diagnosis of symptomatic aneurysm, but her oculomotor neuropathy was only partially improved. We thought that the impairment of the oculomotor function by inflammatory reaction in the cavernous sinus and mechanical compression by the aneurysm had already persisted for too long for post-treatment recovery. We think that the simultaneous occurrence of Tolosa-Hunt syndrome and oculomotor nerve palsy may have resulted because trigeminal neuralgia had increased the blood pressure to induce rapid growth of the preexisting aneurysm, or the inflammatory reaction in the cavernous sinus had promoted the growth of the aneurysm, or that the association was by chance.

An approach to the patient with painful ophthalmoplegia, with a focus on Tolosa-Hunt syndrome.

The differential diagnosis of painful ophthalmoplegia is extensive and consists of numerous sinister etiologies, including neoplasms (ie, primary intracranial tumors, local or distant metastases), vascular (eg, aneurysm, carotid dissection, and carotid-cavernous fistula), inflammatory (ie, orbital pseudotumor, giant cell arteritis, sarcoidosis, and Tolosa-Hunt syndrome), and infectious etiologies (ie, fungal and mycobacterial), as well as other miscellaneous conditions (ie, ophthalmoplegic migraine and microvascular infarcts secondary to diabetes). A systematic approach to the evaluation of painful ophthalmoplegia can lead to prompt recognition of serious disorders that can be associated with significant morbidity or mortality if left untreated. Inflammatory conditions such as Tolosa-Hunt syndrome and orbital pseudotumor are highly responsive to corticosteroids but should be diagnoses of exclusion.

Painful ophthalmoplegia: overview with a focus on Tolosa-Hunt syndrome.

Painful ophthalmoplegia is an important presenting complaint to emergency departments, ophthalmologists, and neurologists. The etiological differential diagnosis of painful ophthalmoplegia is extensive and consists of numerous sinister etiologies including vascular (eg, aneurysm, carotid dissection, carotid-cavernous fistula), neoplasms (eg, primary intracranial tumors, local or distant metastases), inflammatory conditions (eg, orbital pseudotumor, sarcoidosis, Tolosa-Hunt syndrome), infectious etiologies (eg, fungal, mycobacterial), and other conditions (eg, microvascular infarcts secondary to diabetes, ophthalmoplegic migraine, giant cell arteritis). A systematic approach to the evaluation of painful ophthalmoplegia can lead to prompt recognition of serious disorders that if left untreated, can be associated with significant morbidity or mortality. Inflammatory conditions such as Tolosa-Hunt syndrome and orbital pseudotumor are highly responsive to corticosteroids, but should be diagnoses of exclusion.

Síndrome de Tolosa - Hunt en el embarazo / Tolosa-Hunt Syndrome in pregnancy

El síndrome de Tolosa-Hunt es una entidad neurológica que consiste en una oftalmoplejia dolorosa causada por una inflamación granulomatosa del seno cavernoso anterior. Se reporta un caso clínico de una paciente embarazada con esta rara enfermedad.

[Painful ophthalmoplegia secondary to larynx carcinoma]. / Oftalmoplejía dolorosa secundaria a carcinoma de laringe.

CASE REPORT: We present a case of metastatic larynx cancer in the cavernous sinus. It presented itself as a right sixth cranial nerve palsy which progressed into a painful ophthalmoplegia with normal CT and RNM in its early stages. DISCUSSION: We must suspect a cavernous sinus invasion in patients with cranial nerve palsies and with a history of malignancy, despite negative radiologic findings. Tumoral invasion of the skull base has been described in pharyngeal neoplasms but it is exceptional in larynx carcinomas.

Síndrome de Tolosa-Hunt: presentación de dos casos / Tolosa-Hunt syndrome: a review of two cases

En 1954 Eduardo Tolosa y posteriormente Hunt describieron una entidad definida por un intenso dolor orbitario unilateral, disfunción de los nervios oculomotores y de la rama oftálmica del trigémino. Este cuadro se caracterizaba por tener una respuesta espectacular a los corticoides y los escasos estudios histopatológicos mostraron una reacción granulomatosa inespecífica del seno cavernoso. Presentamos 2 casos del síndrome de Tolosa-Hunt. El primero corresponde a una mujer de 24 años, con dolor orbitario izquierdo, compromiso de todos los oculomotores y de la rama oftálmica de trigémino. En los exámenes complementarios destacó la RNM donde se vio un engrosamiento isointenso en T1 e impregnación con el uso de contraste del seno cavernoso izquierdo. El segundo caso correspondió a una mujer de 50 años, con un cuadro clínico de características similares a la primera enferma. Todos los exámenes complementarios resultaron normales, incluyendo la RNM. Las 2 pacientes tuvieron una excelente respuesta al uso de corticoides, permaneciendo asintomáticas a más de 2 años de seguimiento. Destacamos la utilidad de la RNM en el diagnóstico diferencial. El uso empírico de corticoides y el seguimiento clínico de las pacientes, permitieron plantear la hipótesis de un síndrome de Tolosa-Hunt. Esta conducta, evita la realización de procedimientos invasivos de alto riesgo, como una biopsia del seno cavernoso, única manera de llegar a la confirmación histopatológica de esta enfermedad.