Diagnosis Of Tolosa-Hunt Syndrome Research Articles

Tolosa Hunt Syndrome: An Atypical Presentation of a Rare Condition

Abstract Purpose: Tolosa-Hunt Syndrome (THS) is a rare disorder with signs and symptoms that mimic various other diseases. Diagnosis of THS, especially in atypical cases, can be challenging due to the need to exclude other diseases. Observations: A 59-year-old-male presented with acute vision loss of his left eye preceded by acute worsening of chronic headaches. Additionally, he had near complete ophthalmoplegia of this eye. He underwent extensive workup including numerous hematologic studies, cerebrospinal fluid studies, and neuro-radiological imaging studies. All tests had normal results except for an elevated ESR of 92 mm/hour and an abnormal MRI of the brain and orbits. The MRI revealed the left orbit as abnormal with proptosis and intraconal inflammatory change. It also showed abnormal enhancement of the left orbital apex and adjacent lateral aspect of the left sphenoid sinus. He was diagnosed with THS and rapidly responded to corticosteroid treatment. Conclusions and Importance: THS can be challenging to diagnose because it is a diagnosis of exclusion. An added difficulty with THS is the need to quickly recognize and diagnose it since early treatment with corticosteroids can produce a rapid treatment response and decreases the chance of severe residual symptoms. THS does not usually affect the optic nerve, but optic nerve damage can occur and involvement of the optic nerve further increases the urgency of diagnosing it and promptly initiating treatment.

Case Report: Tolosa-Hunt syndrome, a neuroinflammatory origin of painful ophthalmoplegia

Background Tolosa-Hunt syndrome is a rare neuroinflammatory disease that affects the cavernous sinus and superior orbital fissure, resulting in symptoms of neuropathy. Case presentation We report a case of a 35-year-old male patient with no past medical history who presented to the emergency department (ED) complaining of a four-day history of progressively increasing headaches associated with right eye pain and double vision. A physical examination showed findings suggestive of abducens nerve palsy. A computed tomography (CT) scan showed subtle asymmetric thickening of the right cavernous sinus, which prompted further investigation by magnetic resonance imaging (MRI) scan. The MRI scan showed intensely enhanced soft tissue thickening in the right cavernous sinus region, slightly extending to the right orbital apex, which caused enlargement of the cavernous sinus and appeared isointense to the adjacent muscles on T1 and T2. The diagnosis of Tolosa-Hunt syndrome was made after the exclusion of all other possible pathologies. The patient received prednisolone therapy; his symptoms improved, and he was discharged after three days. Two weeks later, the patient presented again to the ED with the same symptoms and was treated symptomatically. Conclusions This case highlights a very rare etiology for painful ophthalmoplegia, which is Tolosa-Hunt syndrome. Thickening of the cavernous sinus on MRI images with a background history of painful ophthalmoplegia and headache should raise clinician’s suspicion towards the disease. In addition, exclusion of other possible causes is very important, as Tolosa-Hunt syndrome is a diagnosis of exclusion.

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

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. 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. 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). 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.

TOLOSAHUNT SYNDROME: A RARE CAUSE OF OCULOMOTORPARALYSIS

Tolosa-Hunt syndrome is related to an idiopathic granulomatous inflammatory process. Its clinical manifestation is painful ophthalmoplegia. We report the case of a young patient who presented with oculomotor paralysis, and whose diagnosis of Tolosa-Hunt syndrome was accepted, given the negativity of all the complementary examinations carried out. Treatment with corticosteroids led to a rapid regression of symptoms.

Tolosa-Hunt syndrome after COVID-19 infection

Tolosa-Hunt syndrome (THS) is a rare, idiopathic, non-specific inflammation within the cavernous sinus and/or superior orbital fissure leading to painful ophthalmoplegia. The authors describe the first case of a 12-year-old otherwise healthy girl who presented with painful ophthalmoplegia after a documented COVID-19 infection. Neuroimaging revealed inflammation within the ipsilateral cavernous sinus, Meckel’s cave, and orbital apex. After a comprehensive work-up was negative, the patient experienced prompt clinical and radiographic improvement with high-dose corticosteroids, and a diagnosis of THS was made.

Tolosa-Hunt Syndrome and IgG4 Diseases in Neuro-Ophthalmology.

Tolosa-Hunt syndrome (THS) remains a challenging diagnosis for many neurologists. Often believed to be a rare presentation, the classical presentation is known to involve cranial nerves and tissues surrounding the cavernous sinus. Traditionally, a diagnosis of THS is considered when all secondary conditions have been ruled out. Yet, newer findings have elaborated a complex pathogenetic process with some overlap from the IgG4 spectrum of disorders, with which it shares many phenotypic similarities. In this narrative review, we present an updated picture of the condition focusing on the latest developments in the pathogenesis, diagnosis, and clinical management of these two conditions and use illustrative examples to highlight the salient features of this rare presentation.

Tolosa Hunt Syndrome

Background: Tolosa Hunt syndrome (THS) is an uncommon case defined as unilateral orbital or periorbital pain associated with weakness of one or more of the third, fourth and/or sixth cranial nerves, the etiology is still unknown. Pain and paresis Tolosa-Hunt syndrome is cured when adequately treated with corticosteroids
 Case: We present a case of young female patient with reccurent painful opthalmoplegia since 2 years ago. She also had ptosis and protopsis on her left eye. Contrast Ct scan showed thick lateral rectus muscle and superior rectus muscle. The diagnosis of Tolosa Hunt Syndrome was made according to clinical features, contrast Ct scan of the head and a positive response to corticosteroid treatment.
 Conclusions: Tolosa Hunt Syndrome is a diagnosis of exclusion. Clinical Features, Neuroimaging, and positive response to corticosteroid treatment are very helpful in determining the diagnosis. Patient must be told that the disease can recur.

Cavernous sinus syndrome: A prospective study of 73 cases at a tertiary care centre in Northern India

ObjectivesTo study the clinical and etiological profile of patients with cavernous sinus syndrome (CSS) and identify factors which could determine the etiology and influence the outcome of these patients. Patients and methodsThis prospective observational study included 73 consecutive patients satisfying the criteria of CSS (i.e. involvement of any 2 of the 3rd, 4th, 5th and 6th cranial nerves or any one of them with radiological evidence of cavernous sinus involvement). All these patients were subjected to detailed haematological, biochemical and radiological investigations and diagnosed and treated as per guidelines. The clinical and investigational data was recorded and analysed meticulously. ResultsA definitive etiological diagnosis of CSS could be achieved in 86% of patients. Tumours, fungal infections and Tolosa Hunt syndrome (THS) were most common causes. On univariate analysis, diabetes, severe vision loss (visual acuity of <3/60 in at least one eye), and presence of nasal discharge showed a significantly positive association with a fungal CSS. Evidence of paranasal sinusitis, bone erosion and ICA (internal carotid artery) involvement on Gadolinium enhanced MRI (magnetic resonance imaging) of brain were significantly associated with a fungal CSS (p=0.0001), whereas involvement of orbital apex had a negative association with a neoplastic etiology (p=0.014). On multiple logistic regression, orbital apex involvement on MRI was associated with diagnosis of THS (p=0.019, OR: 18.7; 95% CI: 1.6–217.4) while MRI evidence of paranasal sinusitis (p=0.014, OR: 45; 95% CI: 2.1–94.3) and bone erosion ((p=0.019, OR: 12.5; 95% CI: 1.5–103) correlated with diagnosis of fungal CSS. 65.2% of patients (fungal CSS- 70%) had a good prognosis at six months follow up. ConclusionMost patients with CSS can be diagnosed accurately and managed properly with good outcomes.

Tolosa-Hunt syndrome—a rare cause of headache and ophthalmoplegia

A 54-year-old African American woman came to our hospital with a 1-week history of severe right-sided retro-orbital pain and blurry vision. 2 days after onset of unilateral throbbing eye pain she developed intermittent blurry vision, which was worse with rightward gaze. She had no facial droop or language impairment, and no previous episodes of headache or visual changes. She had no history of orbital trauma. Her only previous medical diagnoses were schizophrenia and depression. She was taking aripiprazole and escitalopram daily.

English

Tolosa-Hunt syndrome (THS) is a painful ophthalmoplegia caused by nonspecific inflammation of the cavernous sinus or superior orbital fissure. The syndrome consists of periorbital or hemicranial pain, combined with ipsilateral ocular motor nerve palsies, oculosympathetic paralysis, and sensory loss in the distribution of the ophthalmic and occasionally the maxillary division of the trigeminal nerve. Although they have relapsing and remitting course, they respond promptly to systemic corticosteroid therapy. The diagnostic eponym Tolosa-Hunt syndrome has been applied to these patients and it is this entity which forms the basis of this review. CASE REPORT: A 42 year old man presented with painful diminution of vision since one month and diplopia in the left eye since 7 days. Tingling sensation was noted over left half of upper face. On clinical examination –V/A was 6/12 (B/E) improving to 6/6 with pinhole, IOP and fundus was normal both eyes, extra ocular movement was completely restricted in left eye with painful movements. Left III, IV and VI Cranial nerve palsies were noted with decreased sensation over the area supplied by V Cranial Nerve. The patient was sent for an MR examination. MR imaging was performed on a 1.5 Tesla MR scanner (GE). 10ml Gadolinium was injected in the left antecubital vein to obtain post contrast T1WI.Study revealed moderately enhancing soft tissue in the region of left cavernous sinus and orbital apex. It was isointense to muscle on T1WI and hypo intense to fat on T2WI. MANAGEMENT: On the basis of history and imaging findings, a diagnosis of THS was made and the patient was started on corticosteroid therapy: Injection Methyl-Prednisolone (1gm.I.V for 3 days).Dramatic improvement in diplopia and pain was noted within 48 hours of starting IV steroid therapy. Oral Prednisolone 40mg daily was started after 3 days and continued for 6 weeks. At the end of therapy the patient's ophthalmoplegia also recovered. DISCUSSION: Tolosa–Hunt syndrome (THS) is a rare disorder characterized by severe and unilateral headaches with extra ocular palsies, usually involving the third, fourth, fifth, and sixth cranial nerves, and pain around the sides and back of the eye, along with weakness and paralysis (ophthalmoplegia) of certain eye muscles1. The syndrome of painful ophthalmoplegia consists of periorbital or hemicranial pain, combined with ipsilateral ocular motor nerve palsies, oculosympathetic paralysis, and sensory loss in the distribution of the ophthalmic and occasionally the maxillary division of the

Utility of Thallium-201 Scintigraphy in Tolosa-Hunt Syndrome

Tolosa-Hunt syndrome (THS) is a rare disorder, especially in the pediatric population, characterized by unilateral painful ophthalmoplegia with a relapsing-remitting course. Because the diagnosis of THS is based on the exclusion of other causes of painful ophthalmoplegia, attention should be paid to possible alternative diagnoses. Thallium-201 chloride ((201)Tl) scintigraphy has been used to evaluate tissue histology in clinical oncology with a marker, the retention index (RI). A higher value indicates histological malignancy. Although its utility in pediatric THS has not been discussed, we suggest that (201)Tl scintigraphy may be informative as a marker in the diagnosis. We present an 11-year-old boy with THS who was evaluated with (201)Tl scintigraphy before treatment with corticosteroids, when he had headache, photophobia, and diplopia. The RI of (201)Tl indicated that the lesion would be benign. Although his clinical symptoms did not fulfill the THS criteria completely, his eye symptoms disappeared 2 weeks after corticosteroid treatment, which was not within the 72 h as in the diagnostic criteria of THS. He has been symptom-free for more than 2 years with only an initial 4-week corticosteroid therapy. This report not only shows the potential of (201)Tl scintigraphy to contribute to the correct diagnosis of pediatric THS but also suggests the possibility that the diagnosis of THS could be supported uniquely even in a pediatric THS-suspicious patient who did not fulfill the current THS criteria completely. In conclusion, we suggest that (201)Tl scintigraphy may be useful for making the diagnosis of THS, especially in pediatric patients.

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.

Metastasis of Breast Cancer to the Sphenoid Sinus Presenting as Tolosa-Hunt Syndrome

Breast cancer with sphenoid sinus metastasis has rarely been reported. A 57-year-old woman presented with Tolosa-Hunt syndrome 1 year after undergoing surgery and chemoradiation therapy for ductal breast cancer. The diagnosis of Tolosa-Hunt syndrome was based on a computed tomography scan and granulomatous pathologic findings from the ethmoid sinuses. Magnetic resonance images revealed an enhanced soft tissue mass mainly in the right sphenoid sinus; the second sinoscopic biopsy revealed metastatic invasive ductal breast carcinoma with intact surface epithelium. The patient died 20 days after the second biopsy. In this report, we discuss the possible metastatic pathway and choice of imaging modalities for the diagnosis of this rare metastatic sphenoid sinus cancer.

The efficacy of radiation monotherapy for Tolosa-Hunt syndrome

Sirs, Tolosa-Hunt syndrome (THS) is characterized by painful ophthalmoplegia due to an idiopathic chronic granulomatous inflammation of the cavernous sinus [9]. THS usually improves with corticosteroids, however, in cases showing an insufficient effect of corticosteroid therapy or side effects, alternative therapies such as immunosuppressive agents [3] or focal radiation therapy have been reported [2, 5–7]. We describe a patient with THS who was successfully treated with radiation monotherapy. A 36-year-old man with chronic hepatitis B and impaired glucose tolerance since age 30 developed acute left orbital pain and double vision at the end of July 2006. Neurologic examination on 3 August 2006 demonstrated only mild restriction of abduction of the left eye. Magnetic resonance imaging (MRI) of the brain showed no abnormality including the cavernous sinus region at that time. His illness progressed rapidly and he was admitted to our hospital on 14 August 2006. On admission, body temperature and blood pressure were normal. Neurologic examination demonstrated almost complete ophthalmoplegia in all directions and blepharoptosis without pupil abnormality on the left. Spontaneous left ophthalmic pain was also reported. There were no ophthalmic symptoms on the right and there were no symptoms of lower cranial nerves on both sides. Urinalysis and routine hematological examination were normal. Blood chemistry examination demonstrated mild elevation of aspartate aminotransferase (60 IU/L; normal range 11–34 IU/L), alanine aminotransferase (151 IU/L; normal range 6–39 IU/L) and glycohemoglobin A1c (6.1%; normal range 4.3–5.8%). Surface antigen and core antibody of hepatitis B virus were positive. The following immunological and inflammatory investigations were normal or negative; antinuclear antibody, cytoplasmic and perinuclear antineutrophil cytoplasmic antibody, angiotensin converting enzyme level and b-D-glucan. Chest radiography and Gallium scintigraphy of the whole body showed no abnormality suggestive of systemic inflammatory disease. Cerebrospinal fluid examinations were normal including cytology and culture for bacteria and mycobacteria. Brain MRI showed abnormal soft tissue enhanced with gadolinium within the left cavernous sinus (Fig. 1). Based on a diagnosis of THS, we first considered corticosteroid therapy for THS with simultaneous antiviral therapy for hepatitis; however, the patient refused corticosteroid therapy because of the possible exacerbation of hepatitis. One week after admission, the left pupil was dilated and light response was diminished, indicating progression of THS. Thereafter, the patient received a total of 30 Gy in 15 fractions focused on the left cavernous sinus by three-dimensional conformal radiotherapy (3D-CRT). Left blepharoptosis and ophthalmoplegia gradually improved within 2 weeks after the initiation of radiation therapy; blepharoptosis had almost completely disappeared by the middle of October 2006. Ophthalmoplegia also Y. Furukawa W. Yamaguchi K. Ito T. Hamada H. Miyaji Department of Neurology, Fukui Prefectural Hospital, Fukui, Japan

Tolosa-Hunt syndrome responsive to infliximab therapy

JO N 3 01 6 cavernous sinus or superior orbital fissure. ESR, serum ACE and neoplastic markers, and CSF examination were normal. MRI scans of brain, including preand post-contrast thin section coronal views of the cavernous sinus and MRI cerebral arteriogram, were normal. CT scanning of thorax and abdomen was normal. A diagnosis of TolosaHunt syndrome was made. The response to two courses of intravenous methylprednisolone (a total of 10 g) resulted in partial (20 %) improvement in her pain but no improvement in the neurological deficit. After three weeks without further improvement, it was decided to begin infliximab therapy. With an infliximab infusion (300 mg – approximately 4 mg/kg) she had good symptomatic pain relief within a few days. At her second course of infliximab four weeks later, her pain and sensory symptoms had entirely resolved, and her diplopia had lessened; she was completely asymptomatic with no abnormal signs after four infusions of infliximab.

Surgery With Cavernous Sinus Syndrome

The etiologies of well-known cavernous sinus syndrome are many. It is necessary to identify clinical and radiologic findings of various cavernous sinus syndromes to avoid major mistakes in treatment. As an example, pitfalls in the diagnosis of Tolosa-Hunt syndrome (THS) are not rare. In this study, we report a case series of THS and review and discuss the mistakes in the diagnosis of cavernous sinus syndromes. This study was conducted during a 10-year-period between 1997 and 2007. There were 10 patients (6 males, 4 females) in this series. Patients were included in this series only if they fulfilled the International Headache Society criteria for Tolosa-Hunt syndrome. Follow-up duration of the patients ranged from 6 months to 9 years (mean=3.8 y). Age of the patients (n=10) ranged from 23 to 70 years (mean=44 y). According to magnetic resonance scans, the lesion was located in the cavernous sinus only in 6 cases; in the orbit only in 1 case; and in cavernous sinus as well as superior orbital fissure and ipsilateral orbit in 1 case; and in both the cavernous sinus and ipsilateral superior orbital fissure in 1 patient. In 1 patient, there was no magnetic resonance imaging abnormality. Nine out of 10 patients completely recovered after steroid therapy. In conclusion, good knowledge of clinical presentation of orbital pseudotumor and THS is very helpful in clinical decision-making in a patient with cavernous sinus syndrome.

Les ophtalmoplégies douloureuses de l'enfant : place du syndrome de Tolosa-Hunt et de la migraine ophtalmoplégique

In children with painful ophthalmoplegia, the diagnosis of Tolosa-Hunt syndrome or of ophthalmoplegic migraine should only be considered when tumoral, infectious, inflammatory or vascular causes have been excluded by appropriate investigations. Both entities are classified as “neuralgia” by the International Headache Society, and seem to share a similar pathogenic mechanism. Both diseases have many clinical similarities with slight differences concerning pain characteristics or ocular associated symptoms. High resolution CT scan or contrast enhanced MRI can be necessary to exclude other causes of painful ophthalmoplegia. They can sometimes objectify an inflammatory process of the cavernous sinus in Tolosa–Hunt syndrome or a reversible enhancement and thickening of the cisternal segment of the oculomotor nerve during an ophthalmoplegic migraine. Pain and ophthalmoplegia quickly resolve with corticosteroids. Such treatment may decrease the risk of recurrence. It is important to follow-up these patients for a 2 years period and to repeat the etiologic assessment. We report here 2 cases of children with painful ophtalmoplegia.

Tolosa-Hunt Syndrome: Report of a Case

Tolosa-Hunt syndrome (THS) is an idiopathic, granulomatous inflammatory disease within the cavernous sinus or superior orbital fissure, which is characterized by painful ophthalmoplegia and dramatic responses of the symptoms to systemic steroid therapy. We present a patient with THS. This 75-Year-old woman suffered from severe retro-orbital pain with III and IV, and impaired pinprick sensation of the first division of the trigeminal nerve in the III and IV, and impaired pinprick sensation of the first division of the trigeminal nerve in the right side. Contrast-enhanced computed tomography of the brain showed only multiple lacunar infarctions over the right side external capsule and basal ganglion, which were not consistent with the clinical symptoms. One gram of methylprednisolone per day was given 8 days after onset of symptoms. Her retro-orbital pain resolved within 48 hours beginning steroid therapy. However, neurological deficits were still noted. Delayed institution of steroid therapy might be the major cause of her neurological sequela. We think steroid therapy should be instituted as soon as possible when THS is suggested. Thorough investigation and long-term follow-up studies are the only methods to confirm the diagnosis of THS.

Really Painful Double Vision

A 67-year-old woman with a history of chronic headache and recent removal of two squamous cell lesions from her forehead presented with left facial pain and diplopia. A diagnosis of Tolosa-Hunt syndrome was made based on clinical presentation and imaging studies. When the patient did not respond to steroids, further studies were done, including biopsy, which revealed perineural spread of squamous cell carcinoma. ( Surv Ophtholmol 45:155–159, 2000.

Tolosa-Hunt syndrome: Focus on MRI Diagnosis

Experience with modern neuroimaging techniques, computed tomography (CT) and magnetic resonance imaging (MRI) scans, in the diagnosis of Tolosa-Hunt syndrome (THS) is reviewed. Conventional CT scan remains normal in about two-thirds of these patients. In the reported 22 patients meeting the IHS criteria for a THS diagnosis on whom an MRI study was performed, MRI revealed a convex enlargement of the symptomatic cavernous sinus by an abnormal tissue isointense with gray matter on short TR/TE images and isohypointense on long TR/TE images. This abnormal tissue markedly increases in signal intensity after contrast injection. MRI seems also to be the ideal technique to follow progressive resolution of the abnormal tissue after steroids. Therefore, normal MRI would probably exclude THS, whereas in the appropriate clinical setting of steroid-responsive painful ophthalmoplegia, MRI showing the cavernous sinus abnormality described here suggests a diagnosis of THS. From these data, we propose that the fourth IHS criterion for THS diagnosis, "Exclusion of other causative lesions by neuroimaging and (not compulsory) carotid angiogram" should be changed to "Finding by MRI of specific cavernous sinus abnormalities (with the characteristics described herein) which slowly resolve with steroid treatment".