The clinical features and outcomes of Tolosa-Hunt syndrome.

BACKGROUND: The objective of this study was to investigate the clinical features and outcomes of Tolosa-Hunt syndrome (THS). METHODS: A retrospective review of the medical records was performed on patients with THS between March 2016 and January 2020. A total of eleven patients fulfilling the International Classification of Headache Disorders (ICHD-3 beta) diagnostic criteria for THS were included in this study. RESULTS: The average age of the patients with THS was 57.18 ± 15.32 years and the mean duration of recovery was 26.91 ± 24.35 days. All eleven patients had orbital or periorbital pain as the first symptom followed by diplopia. Ptosis was found in five patients (45.45 %) in the involved eye. Sixth cranial nerve (CN) palsy was most common (eight cases, 72.73 %), followed by third and fourth CN palsy (five cases, 45.45 %, respectively), optic neuritis (two cases, 18.18 %), and trigeminal nerve and facial nerve palsy (one case, 9.09 %, respectively). One patient with optic neuritis failed to recover visual acuity and the other ten patients completely recovered their ocular motor limitation. All patients were initially treated with steroids. One patient relapsed after five weeks and one patient had a history of THS five years earlier. CONCLUSIONS: THS responded well to steroid treatment, but if it was accompanied by optic neuritis as optic nerve involvement, we suggest follow-up with high dose steroid treatment especially was important.

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.

The aetiologies of unilateral oculomotor nerve palsy: a clinical analysis on 121 patients.

Background and purpose: To strengthen the understanding, increase the early diagnostic rate, and improve the outcome of unilateral oculomotor nerve palsy through the analysis of the 121 patients suffering from this disease in our hospital. Methods: A retrospective study was performed on the 121 patients with unilateral oculomotor nerve palsy diagnosed at the Affiliated Hospital of Xuzhou Medical University from October 2014 to October 2015. The clinical data, such as gender, age, aetiology, clinical features, laboratory tests, and six months follow up reports were analyzed. Results: The main causes identified in the 121 patients with unilateral oculomotor nerve palsy were intracranial aneurysm (29.8%), diabetic peripheral neuropathy (26.5%), painful ophthalmoplegia (9.9%), and other causes (33.9%). The results from the six month follow up showed that in all the patients, 53.7% were fully recovered, 38.0% improved, and 8.3% had no significant change in symptoms. The results also indicated that the patients with diabetic peripheral neuropathy had the best outcome with 71.9% full recovery rate, which was significantly higher than that in the patients with intracranial aneurysm (36.1%, p < .05), and idiopathic causes (44.5%, p < .05). Conclusions: Our data indicates that intracranial aneurysm is the leading cause of unilateral oculomotor nerve palsy, and that diabetic peripheral neuropathy has better outcome. Understanding the common causes and clinical features of unilateral oculomotor nerve paralysis is helpful for its early diagnosis and treatment.

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 con remisión espontánea y recurrencia / Tolosa-Hunt syndrome with spontaneous remission and recurrence

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A comparison of benign and inflammatory manifestations of Tolosa-Hunt syndrome.

BACKGROUND: Tolosa-Hunt syndrome (THS) manifests as a benign or an inflammatory type disease. The nosography differences between these types remain to be elucidated. We aimed to analyze and compare the clinical presentations of benign and inflammatory THS. METHODS: The ward patients who presented with THS from January 1990 to May 2011 were retrospectively reviewed. THS was diagnosed according to the recommendations of the International Headache Society. RESULTS: Of the 53 THS cases (49 patients), 30 (56.6%) were classified as benign and 23 (43.4%) as inflammatory THS. There were strong similarities between the groups in terms of clinical manifestations, laboratory findings, responses to glucocorticoid treatment, and outcomes. However, patients with inflammatory THS tended to be younger (mean age, 43.4 years; P 0.05) and have optic nerve dysfunction (56.5%; P 0.05) and longer disease duration (2.3 ± 1.0 months; P 0.05) compared to those with benign THS (mean age, 56.4 years; mean disease duration, 1.6 ± 0.7 months). The patients with additional involvement of both the optic nerve and the second division of the trigeminal nerve experienced a longer disease duration ( P 0.05). Additionally, patients with orbital pseudotumors had diplopia that responded poorly to treatment with glucocorticoids ( P 0.05). High-dose (>0.5 mg/kg/day) and low-dose (≤0.5 mg/kg/day) prednisolone were equally effective in relieving symptoms in both groups ( P > 0.05). CONCLUSION: Benign and inflammatory THS were highly similar in terms of nosography. The responses to glucocorticoid treatment were generally good except in patients with orbital pseudotumors.

ICHD-II diagnostic criteria for Tolosa-Hunt syndrome in idiopathic inflammatory syndromes of the orbit and/or the cavernous sinus.

A bibliographical search was conducted for papers published between 1999 and 2007 to verify the validity of International Classification of Headache Disorders (ICHD)-II criteria for the Tolosa-Hunt syndrome (THS) in terms of (i) the role of magnetic resonance imaging (MRI); (ii) which steroid treatment should be considered as adequate; and (iii) the response to treatment. Of 536 articles, 48, reporting on 62 patients, met the inclusion criteria. MRI was positive in 92.1% of the cases and it normalized after clinical resolution. There was no evidence of which steroid schedule should be considered as adequate; high-dose steroids are likely to be more effective both to induce resolution and to avoid recurrences. Pain subsided within the time limit required by the ICHD-II criteria, but signs did not. We conclude that THS diagnostic criteria can be improved on the basis of currently available data. MRI should play a pivotal role both to diagnose and to follow-up THS.

Tolosa-Hunt syndrome: critical literature review based on IHS 2004 criteria.

In 2004, the International Headache Society (IHS) re-defined the diagnostic criteria of Tolosa-Hunt syndrome (THS) specifying that granuloma, demonstrated by magnetic resonance imaging (MRI) or biopsy, is required for diagnosis. We reviewed the literature on THS published from 1988 (year of publication of first IHS criteria) to 2002, analysing individual cases in relation to the new IHS criteria. One hundred and twenty-four cases were identified. As far as it was possible to discern, clinical presentation was similar in all, but 44 (35%) were reported to have inflammation on MRI or bioptic evidence of granuloma, 41/124 (33%) had normal neuroimaging findings and 39 (31%) had a specific lesion, so the THS was secondary. These data confirm that clinical criteria for THS are common to several conditions and their application alone does not guarantee a correct diagnosis. The requirement for inflammation on MRI will result in better classification of painful ophthalmoplegias; nevertheless, an MRI protocol that best defines inflammation should be specified. The status of cases which fulfil the clinical criteria but have normal MRI remains to be clarified.

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.