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At least 253 records · Page 14Linked to original sources
PubMed2001

Tolosa-Hunt syndrome in a patient with systemic lupus erythematosus.

We report a case of Tolosa-Hunt syndrome (THS) in a patient with systemic lupus erythematosus studied with MRI. Magnetic resonance showed enlargement of the cavernous sinus and compression of the carotid syphon by enhancing tissue. In particular, fat-suppressed T1-weighted images before and after contrast agent injection and MR angiography showed extension of the abnormal tissue to the apex of the orbit and narrowing of the internal carotid artery. A presumptive diagnosis of THS was made and steroid treatment was started with rapid relief of symptoms. Follow-up MR study after steroid therapy demonstrated sub-total resolution of the neuroradiological findings. Neuroradiological findings in THS are quite typical but they may be subtle; furthermore, the presence of a systemic disease may suggest secondary involvement of the cavernous sinus. Utilization of the appropriate MR techniques and follow-up exams may contribute to the diagnosis of THS even in the presence of other systemic diseases.

Adult↗
PubMed2002

Idiopathic inflammatory pseudotumor of the orbit and Tolosa-Hunt syndrome--are they the same disease?

Unilateral periorbital pain, cranial nerve palsies and a dramatic response to corticosteroid therapy are the hallmarks of clinical presentation in Tolosa-Hunt-Syndrome (THS) and Idiopathic Pseudotumor of the Orbit (IIPO). Both are unspecific chronic granulomatous diseases of unknown origin, sharing clinical as well as paraclinical characteristics. We observed two patients suffering from acute granulomatous IIPO, who also fulfilled the criteria of THS. Patient 1 developed leftsided infiltration of the medial ocular muscle with periorbital pain and cranial nerve palsy. After an initial response to corticosteroid therapy, contralateral relapse occurred with a THS-like infiltration of the sinus cavernosus and narrowing of the intracavernous internal carotid artery. Granulomatous infiltration of the right sinus cavernosus with secondary involvement of the ipsilateral nervus opticus and a slight exophthalmos was seen in Case 2. According to the literature, MRI and CT show identical signal intensity with different localisation: IIPO preferentially intra- and THS retroorbital. Apart from neuroradiological findings, almost similar histopathology and clinical presentation makes it difficult to distinguish between these two syndromes. Similarities between these two syndromes have been discussed for more than 20 years. Our two cases show their close relationship and we suggest that both diseases belong to the same pathological process.

Adult↗
PubMed2003

Progression of preexisting trigeminalgia to Tolose-Hunt-like syndrome. The importance of neuroimaging for early differential diagnosis.

Recurrent unbearable, paroxysmal, unilateral facial pain in the distribution of one or more branches of the trigeminal nerve often provoked by sensory stimuli is typical for idiopathic trigeminal neuralgia. The less frequent localization in the area of ophthalmic branch (5%) is particularly controversial and should be distinguished from pathological lesions in the brainstem and middle and posterior cranial fossa and from diseases of the orbit and eye. This case study presents a 79-year-old woman with typical clinical features of 1st division trigeminalgia without any neurological loss and with normal results of laryngological, ophthalmological, and stomatological examinations as well as neuroimaging CT, and MR /MRA evaluation. Only the evoked potential blink and masseter reflexes demonstrated the pathological values in the early phase of illness. After 1 year of pharmacological treatment no improvement was achieved and the pain became neuropathic and paresis of 3rd, 4th and 6th nerves developed, as observed in Tolose-Hunt syndrome. MRI of the orbit revealed a pathological mass in its apex with a connection to the superior orbital fissure. However, treatment with steroids was completely ineffective. Surgical resection of the tumor (leiomyosarcoma) only partially reversed oculomotor palsy and diminished aching. In differential diagnosis of idiopathic and symptomatic trigeminalgia, early MR and MRA imaging is the most essential and sometimes may be the best single test to evaluate lesions even in distant areas of the nervous system branches.

Aged↗
PubMed2004

Dynamic MR imaging in Tolosa-Hunt syndrome.

OBJECTIVE: To evaluate the cavernous sinuses with dynamic magnetic resonance (MR) imaging in patients with Tolosa-Hunt syndrome (THS). METHODS: The sellar and parasellar regions of five patients with THS and 12 control subjects were examined with dynamic MR (1.5 T) imaging in the coronal plane. Dynamic images were obtained with spin-echo (SE) sequences in three patients, and with fast spin-echo (FSE) sequences in two patients and control subjects. Conventional MR images of the cranium including sellar and parasellar regions were also obtained on T1-weighted pre- and post-contrast SE, and T2-weighted FSE sequences in the coronal plane. RESULTS: MR images revealed affected cavernous sinus with bulged convex lateral wall in three patients and concave lateral wall in two patients. In all control subjects, cavernous sinuses were observed with concave lateral wall. The signal intensity on T1- and T2-weighted images and contrast enhancement on post-contrast images of the affected cavernous sinuses in patients were similar to those of the unaffected cavernous sinuses in patients and control subjects. The dynamic images in all patients disclosed small areas adjacent to the cranial nerve filling-defects within the enhanced venous spaces of the affected cavernous sinus, which showed slow and gradual enhancement from the early to the late dynamic images. No such gradually enhancing area was observed in control subjects except one. The follow-up dynamic MR images after corticosteroid therapy revealed complete resolution of the gradually enhancing areas in the previously affected cavernous sinus. CONCLUSION: Dynamic MR imaging may facilitate the diagnosis of THS.

Adult↗
PubMed2006

A case of Tolosa-Hunt syndrome affecting both the cavernous sinuses and the hypophysis, and associated with C3 and C4 aneurysms.

BACKGROUND: We describe the first case of Tolosa-Hunt syndrome that is associated with C3 and C4 aneurysms. CASE DESCRIPTION: The patient, a female aged 58 years, had diplopia and right retroorbital pain. Magnetic resonance imaging revealed an enlargement of the hypophysis and bilateral cavernous sinuses, particularly on the right side. Cerebral angiography demonstrated ICA aneurysms of the left C3 and right C4 portions. These symptoms were immediately alleviated after initiation of prednisolone therapy, but recurred after the dose was tapered off. Radiological examination revealed an enlargement of the hypophysis whereas the right C4 aneurysm had decreased in size and no blood flow was apparent in the ipsilateral ophthalmic artery. A biopsy was performed and the results showed a focal inflammatory change. Steroid therapy was represcribed, and after a follow-up period of 10 months without therapy, the patient has been free of symptoms. CONCLUSION: We conclude that bilateral ICA aneurysms might be directly induced by inflammatory infiltration into intracavernous ICAs.

Carotid Artery Diseases↗
PubMed2003

MRI findings in Tolosa-Hunt syndrome before and after systemic corticosteroid therapy.

Tolosa-Hunt syndrome (THS) is characterized by painful ophthalmoplegia due to a granulomatous inflammation in the cavernous sinus. Corticosteroid therapy dramatically resolves both the clinical and radiological findings of THS. We present MRI findings of six patients with a clinical history of at least one episode of unilateral or bilateral orbital-periorbital pain, clinical findings of associated paresis of one or more of 3rd, 4th, 5th or 6th cranial nerves. All of the patients revealed an enlargement of the symptomatic cavernous sinus on magnetic resonance imaging (MRI) scans. Five patients revealed total resolution of the clinical findings within 1-8 weeks, following systemic corticosteroid treatment. One patient revealed only minor regression of clinical findings within 2 weeks after the initiation of the treatment, so the cavernous sinus lesion was reevaluated as meningioma on MRI, and the patient underwent surgical resection of the mass with resultant histopathological finding of cavernous sinus meningioma. A follow-up MRI scan was performed for five patients at the end of 8-weeks of steroid therapy. Three of these five patients showed total resolution of the cavernous sinus lesions whereas two of them revealed a partial regression of the cavernous sinus lesions. MRI findings before and after systemic corticosteroid therapy are important diagnostic criteria to put the definitive diagnosis of THS and to differentiate it from other cavernous sinus lesions that simulate THS both clinically and radiologically.

Adult↗
PubMed1999

Metastatic breast cancer presenting as Tolosa-Hunt syndrome.

Choroidal involvement is a well-recognized manifestation of metastatic disease, particularly from breast and lung primaries. However, breast cancer can involve other ocular structures. The two patients presented illustrate diffuse orbital involvement of the extraocular muscles, simulating Tolosa-Hunt syndrome. This association has not been reported previously. Both patients responded well to local radiotherapy.

Adult↗
PubMed2000

Squamous cell carcinoma with perineural invasion presenting as a Tolosa-Hunt-like syndrome: a potential pitfall in diagnosis.

PURPOSE: To describe a case of perineural invasion resulting from squamous cell carcinoma of forehead. METHODS: Case report. RESULTS: Perineural invasion resulting from squamous cell carcinoma of the periocular skin can present as a Tolosa-Hunt-like syndrome with lack of radiologic findings on magnetic resonance imaging (MRI) in its early stages. CONCLUSION: A high level of suspicion for perineural invasion is required when assessing multiple cranial nerve palsies in patients with a history of cutaneous malignancy, despite negative sequential MRI. Perineural invasion must be ruled out by a biopsy of the involved nerves, whenever possible, before empiric therapy with systemic steroids is contemplated.

Biopsy↗
PubMed2006

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.

Clinical Trials as Topic↗
PubMed2005

Long-term cure of Tolosa-Hunt syndrome after low-dose focal radiotherapy.

Tolosa-Hunt syndrome (THS) is a rare cause of painful ophtalmoplegia due to chronic granulomatous inflammation of the cavernous sinus. While corticoids are the treatment of reference, corticodependence and corticoresistance are frequent, hence radiotherapy has been proposed as an alternative treatment. We report a case of long-term improvement (8 years) of THS after low-dose (20 Gy) radiotherapy in a patient with recurrences and corticodependence.

Adult↗
PubMed2006

MRI in Tolosa-Hunt syndrome associated with facial nerve palsy.

A 44-year-old woman developed a severe right frontotemporal headache, retro-orbital pain, and, later, diplopia owing to right sixth nerve palsy. The brain MRI demonstrated strong enhancement of the right cavernous sinus. The sixth nerve palsy and headache improved with steroid therapy after 6 weeks. At that time, she suffered right peripheral facial nerve palsy. Enhancement of the distal canalicular and labyrinthic segment of the right facial nerve was found on contrast-enhanced MRI. To our knowledge, this is a very rare case of Tolosa-Hunt syndrome with facial nerve palsy, with simultaneous enhancement of the cavernous sinus and facial nerve on contrast-enhanced MRI.

Adult↗
PubMed1999

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".

Humans↗
PubMed2006

Dr Frederick Knight Hunt (1814-54) re-visited: medical man and journalist.

This paper studies Knight Hunt's career after he lost control of the Medical Times, and shows how he was brought to the notice of Charles Dickens who made him a sub-editor on the Daily News in 1845 and subsequently selected him as a pivotal contributor to Household Words, a post he gave up only when he became editor of the newspaper in 1851.

England↗
PubMed1976

Tolosa-Hunt syndrome: one cause of painful ophthalmoplegia.

The author reports 12 cases of Tolosa-Hunt syndrome, a benign steroid-resistant cryptogenic granuloma that presents as painful ophthalmoplegia. This syndrome is differentiated from other causes of painful ophthalmoplegia including tumors, aneurysms, collagen disease, specific infections, mucoceles, and benign granulomas of unknown etiology. These other conditions should be excluded by appropriate tests. Surgical exploration is not necessary if there is a prompt remission on steroid therapy.

Adrenal Cortex Hormones↗
PubMed2000

[A case of idiopathic cranial hypertrophic pachymeningitis presenting Tolosa-Hunt syndrome].

A 48-year-old female was seen because of left orbital pain. The neurological findings were normal at her first visit. She presented temporary double vision during conservative period. Plain CT revealed no mass around the sellar region. Enhanced CT revealed enhanced mass in the left cavernous sinus. MRI revealed low intensity lesion on both T1 and T2 weighted images. Enhanced MRI showed strongly enhanced mass extended from the left cavernous sinus to the dura of sellar floor, the contralateral cavernous sinus, and cerebellar tentorium. Angiography showed stenosis of the left internal cerebral artery. Biopsy from the dura of the tentorium was performed via anterior temporal approach. Abundant collagen fibers with hyalinization were seen in the thickened dura. A final diagnosis of idiopathic cranial hypertrophic pachymeningitis presenting Tolosa-Hunt syndrome was made. Symptoms were free just after the surgery, however, the regrowth of the tentorial lesion was found on MRI after one year. The size of the lesion decreased after administration of steroid.

Female↗
PubMedDate not supplied

[Hypertrophic pachymeningitis. A review of the histories of two cases and pathological relationship with the Tolosa-Hunt syndrome and the orbital pseudotumor].

INTRODUCTION: Hypertrophic pachymeningitis is an infrequent condition which starts with a thickening of the dura mater and whose pathogenesis is unknown. We present two new cases of unknown aetiology. CLINICAL CASE: Case 1. A 53 year old man complained of occipital headache, tinnitus and deafness since February 1981. In October 1981 he was admitted to hospital with a worse headache, perio-orbital pain, dysgeusia and ipsilateral peripheral facial palsy. In December he had generalized tonic-clonic seizures and paralysis of the VII and XI right cranial nerves and IX, X and XII left cranial nerves. In February 1982 he developed right trigeminal neuralgia. He was readmitted in November 1983 with continuous headache, vomiting and a behavior disorder. On CT there was marked attenuation of the posterior dura mater, which the neurosurgical department considered unsuitable for biopsy. He died in March 1985. On necropsy there was hypertrophic pachymeningitis. Case 2. A 62 year old patient consulted in November 1995 complaining of right hypoacusia for the past six months, progressively accompanied by ipsilateral paralysis of the II, IV, VI, VII and VIII cranial nerves but with no other alterations on physical examination. Analytical and serological investigations were normal. Cranial MR showed an extraparenchymatous infiltrating lesion in the middle cranial fossa. Biopsy was decided on when no clinical improvement was seen with corticosteroid treatment. The pathologist reported hypertrophic pachymeningitis. Treatment was started with cyclophosphamide in monthly doses and the condition has remained stable to date. CONCLUSION: With these two cases we wish to establish a pathogenic relation between the Tolosa-Hunt syndrome and orbital pseudotumor and show the role played by immunosuppressive treatment in the control of hypertrophic pachymeningitis.

Brain↗
PubMed2001

[A case of Tolosa-Hunt syndrome starting from abducens nerve palsy alone].

A 59-year-old man was admitted to our hospital because of right abducens nerve palsy without orbital pain. One month later right orbital pain appeared and two months later the pain changed to an aching severe pain and the right oculomotor, trochlear and trigeminal nerves were also involved. The administration of corticosteroid dramatically reduced these symptoms. The diagnosis was Tolosa-Hunt syndrome (THS) based on these findings. THS starting with abducens nerve palsy alone is rare, representing only 5.1% of 118 patients. In our patient, the newly developed dynamic MRI was able to reveal the right cavernous sinus lesion. The diagnostic usefulness of dynamic MRI for cavernous sinus lesions has been demonstrated for such cases.

Abducens Nerve Diseases↗