Cases reported "Tolosa-Hunt Syndrome"

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1/13. Resolution of MRI abnormalities of the oculomotor nerve in childhood ophthalmoplegic migraine.

    ophthalmoplegic migraine is an uncommon disorder, usually starting in older childhood. Its physiopathology remains obscure and diagnosis is reliant on clinical grounds and exclusion of other disorders. We report four cases of childhood ophthalmoplegic migraine, one of them starting in infancy. association with other types of migraine is common. Two of the three patients studied by magnetic resonance imaging (MRI) showed enhancement and enlargement of the cisternal portion of the oculomotor nerve, which spontaneously resolved after 2 and 4 years, respectively. Persistence of clinical recurrences was associated with long-lasting presence of the MRI finding, and possibly with mild sequelae. These radiological abnormalities suggest a common physiopathological mechanism with other inflammatory diseases, except for a benign evolution which, added to its specific anatomic site, seems to be the only neuroradiological marker, besides normality, in ophthalmoplegic migraine. The very long potential duration of MRI changes and the scarcity of clinical episodes make feasible its incident discovery once the migraine attack has become a remote memory.
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2/13. tolosa-hunt syndrome preceded by facial palsy.

    The tolosa-hunt syndrome consists of a painful ophthalmoplegia related to a granulomatous inflammatory process in the cavernous sinus, which may be documented by cerebral magnetic resonance imaging with gadolinium enhancement. Two cases of tolosa-hunt syndrome preceded by facial palsy observed in 1998 at the Department of neurosurgery of the Second University of Naples are presented here. Both patients developed tolosa-hunt syndrome following an ipsilateral facial palsy that resolved in about 15 days with medical treatment. Cerebral magnetic resonance imaging with gadolinium enhancement showed, in both cases, inflammatory tissue in the cavernous sinus. The patients underwent corticosteroid therapy (prednisolone, 80 mg per day, intravenously) with pain regression. In the first case, the patient experienced recurrence of the syndrome that was definitively resolved with further corticosteroid treatment. The rare reports of facial palsy in patients with tolosa-hunt syndrome suggest the inclusion of this disease in the so-called multiple cranial nerve palsy syndrome. It is probable that tolosa-hunt syndrome has an inflammatory pathogenesis.
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3/13. Superior orbital fissure syndrome in a latent type 2 diabetic patient.

    Although isolated cranial nerve palsies are common in diabetic patients, multiple, simultaneous cranial neuropathies are rare. We describe the second case of a complete superior orbital fissure syndrome including the optic nerve in a middle-aged Papuan man with newly diagnosed diabetes mellitus. The differential diagnosis included septic cavernous sinus thrombosis and Tolosa Hunt syndrome, and management was initially directed at excluding these serious, treatable conditions.
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4/13. 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.
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5/13. Tolosa Hunt Syndrome--intractable pain treatment with acupuncture?

    PURPOSE: The Tolosa Hunt Syndrome (THS) is a painful granular inflammation of the cerebral vessels followed by pain and disorders of the extrabulbar muscles. The therapy consists of corticosteroids and analgetics. There was a 70 year old woman who suffered from painful paresis of the abducent and oculomotor nerves following an infection with borrelia burgdorferi--but without ocular symptoms. The treatment with corticosteroids reduced the palsy but she complained of excessively painful attacks in the region of the first branch of the trigeminal nerve. Opiold analgetic therapy did not bring about any relief. acupuncture is an irritative method with a physical effect on the nervous system: its pain-reducing effect is caused by the activation of transmitters like endorphins in thalamus and brain stem. Knowing this effect, the THS patient, after informed consent, was treated with acupuncture. To measure the extent of pain, a visual analog scale (0: no pain - 10: maximum pain) was used. acupuncture was performed according to the empirical rules of the Traditional Chinese medicine (TCM), during a period of 10 weeks and 12 weeks. There was a significant pain relief after acupuncture from VAS 10 to VAS 5. The effect vanished during the next four months. After a second series of 12 sessions pain reduction was reported from VAS 10 to 4. One year after the last Tolosa Hunt Syndrome - intractable pain pain strength ranged between VAS 4 - 6. Therefore acupuncture seems to be a good additional method for reduction of intractable pain.
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6/13. Epidermoid cyst of the cavernous sinus: clinical features, pathogenesis and treatment.

    Epidermoid cyst may rarely arise within the lateral wall of the cavernous sinus (CS) and produce clinical symptoms mimicking the tolosa-hunt syndrome. Pathogenically, it is suggested that some of the subpopulation of cells arising from the neural crest which give rise to skin, may remain entrapped in the meninges around the nerves in the lateral wall in an uncompleted stage of maturation and may develop epidermoid cysts. A case is presented of a 68-year-old man with a 4-week history previously diagnosed as tolosa-hunt syndrome. MRI is the investigation of choice. Surgical resection can result in excellent recovery of preoperative deficits of cranial nerves (CN) III through VI.
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7/13. 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.
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8/13. 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.
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9/13. Reidel's thyroiditis and tolosa-hunt syndrome, a rare association.

    A previously undescribed association of Reidel's thyroiditis with tolosa-hunt syndrome is reported. A 33 year-old Caucasian female presented with a two-year history of painful progressive goitre, histologically a Reidel's thyroiditis. She underwent left partial thyroidectomy. Post-operatively she developed severe right retro-orbital pain, right temporal headache and a partial right occulomotor nerve palsy. A diagnosis of tolosa-hunt syndrome was made. Some important aspects of this case are discussed.
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10/13. Paediatric tolosa-hunt syndrome.

    tolosa-hunt syndrome is characterized by a dull, persistent pain around the affected eye, ophthalmoplegia and, sometimes, involvement of other cranial nerves passing through the cavernous sinus. Corticosteroid administration is valuable in the treatment and frequently has a dramatic effect. We report a boy with tolosa-hunt syndrome who fails to respond to the initial steroid treatment. The role of the MRI in the management of this condition is discussed.
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