Recurrence and long-term outcomes of Tolosa-Hunt syndrome.

BACKGROUND AND PURPOSE: Tolosa-Hunt Syndrome (THS) is a rare disorder, and detailed clinical information and treatment outcomes have yet to be fully elucidated. This study aims to investigate the clinical features and factors associated with the treatment outcomes of THS, as defined by the established diagnostic criteria. METHODS: This study retrospectively recruited 91 patients with a diagnosis of THS from 2003 to 2020. We analyzed the clinical features and outcomes, the initial treatment response, recurrences, and the final treatment response. RESULTS: Isolated ocular motor nerve palsy was the most common (82.4%) finding of ophthalmoplegia, involving the oculomotor nerve in more than half of the cases (52.0%). The MRI lesions were mostly observed in the cavernous sinus (94.5%) with an extracavernous extension in about one-third of them. Five patients showed only extracavernous lesions. A total of 25 (27.5%) patients experienced recurrence. Recurrence occurred during steroid tapering as part of the initial treatment in seven, while in 18 patients, it happened after the successful termination of the initial treatment. However, all patients achieved complete remission at the final. Age was associated with a decrease in initial symptom duration (HR = 1.023, CI = 1.004-1.044) as well as an increase in recurrence-free duration (HR = 0.944, CI = 0.911-0.978). High-dose corticosteroid treatment was associated with a decrease in initial symptom duration (HR = 1.642, CI = 1.001-2.695) and total treatment duration (HR = 2.203 CI = 1.302-3.730). CONCLUSIONS: THS can recur frequently especially in younger but have a favorable prognosis. High-dose corticosteroids can be an effective initial treatment and reduce the total treatment duration.

Orbital inflammation following COVID-19 vaccination: A case series and literature review.

PURPOSE: The purpose of the study was to report three cases of orbital inflammation following administration of the COVID-19 vaccination, manifesting as Tolosa-Hunt syndrome (THS) and orbital myositis. METHOD: A retrospective case series and literature review of patients who developed orbital inflammation following a COVID-19 vaccination. RESULTS: One patient presented with Tolosa-Hunt syndrome (THS) 14 days following her third (booster) COVID-19 vaccination, one patient developed orbital myositis 10 days following his first COVID-19 vaccination and one patient developed recurrent orbital myositis 1 and 7 days following her second and fourth COVID-19 vaccination. All patients received the Comirnaty vaccine (Pfizer-BioNTech). A thorough systemic autoimmune disease workup in both patients was unremarkable. Two patients had a prior history of orbital inflammation, with previous involvement of other different orbital structures. Characteristic MRI features for each pathology were observed, supporting the clinical presentation of THS and orbital myositis. There was complete resolution of THS following corticosteroids, with no recurrence at 2 months. Meanwhile, one case of orbital myositis self-resolved at 2 months without use of systemic corticosteroids, while the other patient with orbital myositis required treatment with intra-orbital steroid injections and oral corticosteroids. CONCLUSION: Orbital inflammation has been recognised as a rare adverse effect following COVID-19 vaccination. We present a case series of THS and orbital myositis as varied presentations of this entity.

Tolosa-Hunt syndrome presenting with features of a trigeminal autonomic cephalalgias and pituitary enlargement.

Tolosa-Hunt syndrome is understood as a steroid-responsive, relapsing-remitting, unilateral headache disorder associated with ipsilateral cranial neuropathies, of a probable granulomatous aetiology. The diagnosis is made clinically from the history and examination, supported by appropriate imaging. Here the authors report a case of Tolosa-Hunt syndrome with a headache phenotype mimicking a trigeminal autonomic cephalalgias (hemicrania continua), and serial MRI studies showing a stable enlarged pituitary. Due to her initial lack of clinical signs, she was diagnosed with chronic migraine, revised to hemicrania continua based on indomethacin response, then revised back to chronic migraine. Her final diagnosis was achieved after she developed a left cavernous sinus syndrome 4 years into her disease course. This case shows that Tolosa-Hunt syndrome may present with a non-side-locked headache and delayed development of clinical signs. Clinicians should also maintain a high degree of suspicion when faced with incidental MRI findings.

Recurrent painful ophthalmoplegic neuropathy: a cause for recurrent third nerve palsy in a child.

Recurrent painful ophthalmoplegic neuropathy (RPON), previously called ophthalmoplegic migraine, is a rare condition characterised by recurrent episodes of headache and ophthalmoplegia. We report a case of 11-year-old girl with recurrent painful ophthalmoplegia due to isolated right oculomotor nerve involvement. MR brain imaging showed enhancing lesion of cisternal segment of right oculomotor nerve. A possibility of Tolosa Hunt syndrome was considered and she was treated with glucocorticoids, followed by azathioprine due to recurrence. In the fourth episode, she developed migraine headache followed by right third nerve palsy, after which the diagnosis was revised to RPON. She was started on flunarizine along with short-term glucocorticoids. At 1-year follow-up, she remained asymptomatic. RPON should be considered in patients with recurrent third nerve palsy to avoid inadvertent long-term exposure to immunosuppressive agents.

From left to right: an unusual presentation of Tolosa-Hunt syndrome with bilateral eye involvement.

Here we discuss a presentation of Tolosa-Hunt syndrome (THS) in a 44-year-old female with new right-eye ptosis, ophthalmoplegia and headache. Four days prior, she had almost identical ptosis and ophthalmoplegia in her left eye, which resolved. Cavernous sinus inflammation and symptom improvement with glucocorticoid treatment indicated THS with bilateral eye involvement, a presentation which may be undervalued by the current THS classification.

Multiple Spontaneous Visceral Arterial Dissections in a Patient With Tolosa-Hunt Syndrome on Corticosteroid Therapy.

Multiple spontaneous visceral arterial dissections are an infrequent occurrence. The etiology, risk factors and natural history of these dissections have not been elucidated, and the optimal therapeutic strategy has not been established. We report a rare case of multiple spontaneous visceral arterial dissections involving the celiac artery, splenic artery, superior mesenteric artery, and right renal artery in a patient with Tolosa-Hunt syndrome on short-term corticosteroid therapy. The patient was subjected to conservative treatment and endovascular repair, achieving good clinical and radiological outcomes during the long-term follow-up period.

Tolosa-Hunt Syndrome in Childhood and Adolescence: A Literature Review in the Last 10 Years.

OBJECTIVES: The aim of the study is to review the articles published in the last 10 years on Tolosa-Hunt syndrome (THS) in childhood and adolescence to learn about its clinical and epidemiological characteristics and its therapeutic management. METHODS: Based on the literature search in the major medical databases and using the descriptors "Tolosa-Hunt syndrome and children" and "Tolosa-Hunt syndrome and adolescent" we included articles published between 2009 and 2019. We found 51 articles, but only 11 met the inclusion criteria. RESULTS: There were eight women and four men with THS, whose average age was 12.0 ± 4.3 years, ranging from 4 to 17 years. Magnetic resonance imaging showed inflammation in the cavernous sinus (100%), orbit (25%), and superior orbital fissure (8.3%), unilateral in all patients. The internal carotid artery (58.3%), third (66.7%), sixth (33.3%), and fourth (25%) cranial nerves were injured. Headache was located in the orbital region, ipsilaterally to nerve involvement and had mild to severe intensity. Corticosteroid was the treatment used in 91.7% of the patients. They were followed for an average of 13.4 ± 12.4 months. Only two patients had recurrence of the disease. CONCLUSION: THS is infrequent in childhood and adolescence and knowledge of its clinical and epidemiological characteristics and its therapeutic management is necessary for pediatricians.

Miositis orbitaria, una causa de oftalmoplejia dolorosa en pediatría / Orbital myositis, a cause of pediatric painful ophthalmoplegia

INTRODUCCIÓN: La miositis orbitaria (MO) es un proceso inflamatorio grave de etiología desconocida que compro mete los músculos extraoculares. La presentación en edad pediátrica es rara y con frecuencia afecta a más de un individuo de una familia, lo que sugiere algún grado de predisposición genética. OBJETIVO: Describir un caso de miositis orbitaria de presentación en edad pediátrica, sus características clínicas, y la utilidad de la imagen por resonancia magnética para la confirmación del diagnóstico. CASO CLÍNICO: Paciente femenina de 13 años que presenta cefalea aguda, dolor periorbitario derecho, exacerbado con los movimientos oculares y visión borrosa a quien se le realizaron estudios para miopatía tiroidea, enfermedades infecciosas, autoinmunidad y cáncer que fueron negativos. En la imagen por resonancia magnética se evidenció miositis del músculo recto medio derecho, sin evi dencia de neuritis óptica. Recibió tratamiento con glucocorticoides sistêmicos intravenosos seguido de esteroides orales con mejoría clínica completa. CONCLUSIONES: La MO tiene etiología desconocida, y puede tener un curso maligno. Dada su presentación clínica inespecífica, el estudio diagnóstico diferencial debe ser amplio, y su estudio debe considerar realizar resonanacia magnética. El inicio temprano del tratamiento con esteroides evita el daño permanente de los músculos extraoculares.

INTRODUCTION: Orbital myositis (OM) is a serious inflammation of extraocular muscles with unknown etiology. Pe diatric presentation is rare and often affects more than one individual in a family, suggesting a genetic predisposition. OBJECTIVE: To describe a pediatric case of orbital myositis, its clinical characteristics, and the usefulness of MRI for confirming the diagnosis. CLINICAL CASE: A 13-year-old female patient presenting with acute headache, right periorbital pain, exacerbated by eye movements, and blurred vision. We ruled out thyrotoxic myopathy, infectious diseases, autoimmunity, and malignancy. An MRI showed right medial rectus muscle myositis and no evidence of optic neuritis. She was treated with intravenous systemic glucocorticoids followed by oral steroids with complete clinical resolution. CONCLUSIONS: OM has unknown etiology and can present a malignant course. Due to its unspecific clinical presentation, a comprehensive differential diagnosis should be made and it should consider performing MRI. Early treatment avoids permanent damage of extraocular muscles.

Bilateral facial nerve involvement in a patient with Tolosa-Hunt syndrome.

Tolosa-Hunt syndrome is characterized by unilateral retro-orbital headaches and cranial nerve palsies, usually involving cranial nerves III-VI. It is rare for other cranial nerves to be involved, although this has previously been reported. We report a 19-year-old woman presenting with typical features of Tolosa-Hunt syndrome but ultimately developing bilateral facial nerve palsies and enhancement of both facial nerves on magnetic resonance imaging. The patient presented with unilateral retro-orbital headaches and palsies of cranial nerves III-VI. She was diagnosed with Tolosa-Hunt syndrome but was non-compliant with her corticosteroid treatment due to side effects. She returned with progressive left followed by right facial nerve palsy. Her corresponding follow-up magnetic resonance imaging scans showed sequential enhancement of the left and right facial nerves. She ultimately had clinical improvement with IV methylprednisolone. To our knowledge, Tolosa-Hunt syndrome associated with bilateral facial nerve palsy and corroborative facial nerve enhancement on magnetic resonance imaging has not previously been described. Moreover, our patient's clinical course is instructive, as it demonstrates that this atypical presentation of Tolosa-Hunt syndrome can indeed respond to corticosteroid treatment and should not be mistaken for other entities such as Bell's palsy.

Tolosa-Hunt syndrome with general myasthenia gravis involvement.

Tolosa-Hunt syndrome is an uncommon disease that exhibits unilateral periorbital pain or headache, accompanied by cranial nerve palsies. Myasthenia gravis is an acquired immune system disease involving the neuromuscular junction. One rare case of Tolosa-Hunt syndrome combined with ocular myasthenia gravis had been reported in the literature, but not general myasthenia gravis. We present a patient with a probable coincidence of Tolosa-Hunt syndrome and general myasthenia gravis. A 63-year-old male exhibited episodes of unilateral headache with double vision, bilateral ptosis, vision decrease in the left eye and left facial hypoesthesia, muscle weakness in limbs and neck. The muscle weakness was fluctuating and could be relieved by rest. Blood analysis, cranial magnetic resonance imaging, magnetic resonance angiography/venogram) and orbit/mediastinum computed tomography demonstrated no abnormalities. Serum myasthenia gravis related antibodies detection showed positive titin- antibodies and ryanodine receptor antibodies. Corticosteroid and pyridostigmine bromide treatments were effective. Each of the patient's symptoms had almost disappeared at the third-month follow-up. We speculate on the etiology of Tolosa-Hunt syndrome with general myasthenia.

Use of methotrexate in the management of recurrent Tolosa-Hunt syndrome: Two case reports.

RATIONALE: Tolosa-Hunt syndrome (THS) is rare condition characterized by painful ophthalmoplegia that usually responds well to corticosteroid. About a half of THS patients experience recurrence within intervals of months to years from initial presentation. Recurrence is more common in younger patients, and can be ipsilateral, contralateral, or bilateral. Cyclosporine, azathioprine, methotrexate, mycophenolate mofetil, infliximab, and radiotherapy can be considered as second-line treatment. However, there is insufficient evidence for treatments preventing recurrence of THS. PATIENT CONCERNS: We experienced two patients with THS that recurred twice while tapering or after ceasing corticosteroid administration. DIAGNOSIS: Both patients were diagnosed as recurrent THS. INTERVENTIONS: Methotrexate was treated with a combination of corticosteroid after THS recurred twice with corticosteroid therapy alone. OUTCOMES: After adding methotrexate to the steroid regimen, their symptoms were successfully regulated and ceased to recur LESSONS:: These cases add to the evidence for the use of methotrexate as a second-line therapeutic agent for those patients with recurrent THS attacks. Further studies are in need to prove the risk and benefits of second-line treatments in THS.

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.

[Orbital myositis, a cause of pediatric painful ophthalmoplegia]. / Miositis orbitaria, una causa de oftalmoplejia dolorosa en pediatría.

INTRODUCTION: Orbital myositis (OM) is a serious inflammation of extraocular muscles with unknown etiology. Pe diatric presentation is rare and often affects more than one individual in a family, suggesting a genetic predisposition. OBJECTIVE: To describe a pediatric case of orbital myositis, its clinical characteristics, and the usefulness of MRI for confirming the diagnosis. CLINICAL CASE: A 13-year-old female patient presenting with acute headache, right periorbital pain, exacerbated by eye movements, and blurred vision. We ruled out thyrotoxic myopathy, infectious diseases, autoimmunity, and malignancy. An MRI showed right medial rectus muscle myositis and no evidence of optic neuritis. She was treated with intravenous systemic glucocorticoids followed by oral steroids with complete clinical resolution. CONCLUSIONS: OM has unknown etiology and can present a malignant course. Due to its unspecific clinical presentation, a comprehensive differential diagnosis should be made and it should consider performing MRI. Early treatment avoids permanent damage of extraocular muscles.

A 17-Year-Old Girl With Unilateral Headache and Double Vision.

Tolosa-Hunt syndrome is characterized by a painful ophthalmoplegia secondary to a granulomatous inflammation in or adjacent to the cavernous sinus. Magnetic resonance imaging will show enhancement of the cavernous sinus and/or the orbital apex. Although this syndrome is extremely rare in children, it should be a diagnostic consideration in patients presenting with painful ophthalmoplegia with variable involvement of cranial nerves II to VI. The differential diagnosis for unilateral cavernous sinus lesion is broad, including vascular lesions (cavernous sinus thrombosis), inflammatory processes (sarcoidosis, autoimmune), neoplastic processes (schwannoma, lymphoma), as well as infectious etiologies. We describe a pediatric patient presenting with neurological symptoms from a unilateral cavernous sinus magnetic resonance imaging abnormality and the thorough diagnostic approach to arrive at the diagnosis of Tolosa-Hunt syndrome.

Tolosa-Hunt Syndrome: Clinical Manifestations in Children.

BACKGROUND: Tolosa-Hunt syndrome (THS) is a rare condition in children characterized by painful ophthalmoplegia caused by inflammation of unknown etiology in the cavernous sinus, superior orbital fissure, or orbital apex. Our main purpose was to report two pediatric cases of THS, a typical one and another extremely rare one preceded by facial palsy. METHODS: Both cases were diagnosed with THS based on the 2013 International Classification of Headache Disorders (ICHD-3 beta) criteria. A literature review was also performed concerning epidemiology, clinical and imaging features, diagnostic criteria, treatment, and outcome of THS with a focus on children. RESULTS: The first patient was a 14-year-old boy who presented with third nerve palsy, four weeks after the diagnosis and treatment of peripheral seventh nerve palsy and the second patient was a 10-year-old girl who presented with a five-day history left-sided ptosis, periorbital pain, and diplopia. CONCLUSIONS: THS is a rare condition in pediatric population that requires an extensive evaluation before the final diagnosis is made. Seventh nerve palsy preceding the diagnosis of THS is particularly rare in children. This patient represents only the second reported example of seventh nerve involvement in a child with THS.

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.