Hunt syndrome is characterized by ophthalmoplegia with unilateral severe retro. Diagnosis is based on clinical and imaging findings and the exclusion of other causes because there is no specific biomarker for the syndrome. Painful ophthalmoparesis or ophthalmoplegia is the hallmark of tolosahunt syndrome. Tolosa hunt syndrome is a rare disease, with an unknown etiology, characterized by hemicranial or periorbital pain, associated with cranial nerves paresis or paralysis as well as granulomatous inflammation of the cavernous sinus. It is characterized by painful ophthalmoplegia weakness of the eye muscles and is caused by an idiopathic granulomatous inflammation of the cavernous sinus. Tolosa hunt syndrome is a neurological disorder characterized by painful oftalmoplegia resulting from a granuloma of the anterior cavernous sinus, superior orbital fissure and orbital apex. The case of a 33 yearold feminine patient diagnosed with tolosahunt syndrome, which was preceded by facial palsy is reported. Premachandra skip to main content accessibility help we use cookies to distinguish you from other users and to provide you with a better experience on our websites. Migraine, tolosahunt syndrome and pleocytosis pdf free. Hunt syndrome ths is a rare disorder, especially in the paediatric population, characterized by unilateral painful ophthalmoplegia of, typically, a relapsing. Migraine, tolosa hunt syndrome chronic sarcoidosis, pleocytosis, csf meningitis. Tolosahunt syndrome with spontaneous remission and recurrence. Tolosa hunt syndrome ths diagnosis is based on clinical manifestations, rapid response to steroids, mri findings and exclusion of other causes of cavernous sinus cs involvement.
Tolosahunt syndrome is a diagnosis of exclusion, however magnetic resonance or computed tomographic scans can be used to detect inflammatory changes in the cavernous sinus or orbital fissure and a biopsy can be used to confirm the diagnosis. Reidels thyroiditis and tolosa hunt syndrome, a rare association volume 118 issue 2 m. The hallmark of the tolosahunt syndrome ths is a painful ophthalmoplegia that is steroid responsive. Computerized tomograms and mri, with and without contrast enhancement, of the cranium and orbits of patients fulfilling ihs criteria for the diagnosis of tolosa. Burkitts lymphoma presenting as tolosa hunt syndrome. Various combinations of these cranial nerve palsies may occur, localising the pathological process to the region of the cavernous sinussuperior orbital fissure. Tolosahunt syndrome is a rare, reversible and painful ophthalmoplegia characterized by recurrent unilateral orbital pain, ipsilateral oculomotor paralysis and.
Diagnosis and treatment of tolosahunt syndrome in the. Little consideration has been given to alternative therapies, probably due to the typical rapid response to glucocorticoids pain usually resolves within 24 to 72 hours after starting treatment. Pdf diagnosis and treatment of tolosahunt syndrome in the. In children with painful ophthalmoplegia, the diagnosis of tolosahunt syndrome or. Introduction tolosa hunt syndrome ths is an inflammatory granulomatosis of unknown etiology, with a chronic course and which involves the cavernous sinus and the superior orbital fissure. Conventional ct scan remains normal in about twothirds of these patients. Therefore, prothrombotic conditions with or without infections, such as trauma e. Protein s deficiency, lemierres syndrome, tolossa hunt syndrome and malignancies may manifest as mimics of gs. Highfield mri contribution in tolosahunt syndrome ths. Mri findings in tolosahunt syndrome ths bmj case reports.
Synonyms for tolosa hunt syndrome include painful ophthalmoplegia, recurrent ophthalmoplegia, ophthalmoplegia syndrome. Because of the tolosa hunt syndrome s classic quick response to steroids, limited attention has been made to explore other possible treatment options like tight glycemic control in patients with poorly controlled diabetes mellitus which in a way may have contributed to the resolution of the syndrome. We present a case study of tolosahunt syndrome, a rare idiopathic disease, that is characterized by painful ophthalmoplegia of strong intensity, generally. Systemic symptoms associated with tolosahunt syndrome, e. Gadoliniumenhanced mri is the imaging modality of choice to evaluate the ths and may demonstrate abnormal enlargement and enhancement of the cs extending through. Left untreated, symptoms may resolve spontaneously after an average of about eight weeks. Lymphoma of the orbit masquerading as tolosahunt syndrome neuroophthalmology shazly, ta and mitchell, eb and bonhomme, gr and schuman, js 2015 lymphoma of the orbit masquerading as tolosahunt syndrome neuro. Tolosahunt syndrome ths is an idiopathic condition included in the differential diagnosis of painful ophthalmoplegia. The tolosa hunt syndrome ths is a painful ophthalmoplegia caused by an idiopathic granulomatous inflammation of the cavernous sinus andor superior orbital fissure.
Tolosa hunt syndrome ths, also called steroidre sponsive painful ophthalmoplegia, was reported by hunt. It is characterized by painful ophthalmoplegia weakness of the eye muscles and is. Conclusion tolosa hunt syndrome is a chronic inflammatory disease of the cavernous sinus presenting with acuteonset retroorbital pain, paresis, or palsy of the orbital motor nerves in varying combinations with or without visual loss that typically responds promptly to corticosteroid therapy. Symptoms include excruciating burning, stabbing, or electrical headaches mainly near the eye and typically these sensations are only on one side of the body. Tolosa hunt syndrome is a rare disease, whose etiology is unknown. Department of neurology, universidade federal do triangulo mineiro, uberaba, mg, brazil. The diagnosis of tolosa hunt syndrome ths was based on the unilateral headache, preceding paresis of the ipsilateral cranial nerves, granulomatous enhanced lesion in the cavernous sinus on mri imaging, and absence of other clinical or radiographic signs or symptoms fulfilling the international classification of headache disorders. A rare case of tolosahuntlike syndrome in a poorly. Pubmed is a searchable database of medical literature and lists journal articles that discuss tolosa hunt syndrome. Clinical features and differential diagnosis with mr imaging jeong hyun lee, ho kyu lee, ji kang park, choong gon choi, dae chul suh. Tolosa hunt syndrome, ophthalmoplegia, cavernous sinus, magnetic resonance imaging, corticoster. Tolosahunt syndrome due to actinomycosis of the cavernous. The rare reports of facial palsy in patients with tolosa hunt syndrome suggest the inclusion of this disease in the socalled multiple cranial nerve palsy syndrome.
Cell count and differential, protein, glucose, fungal andor bacterial cultures, gram stain, cytology, and opening pressure of csf are helpful in eliminating conditions mimicking tolosa hunt syndrome. Tolosa hunt syndrome ths is an uncommon diagnosis with an incidence of nearly 1 to 2 cases per million hallmarked by the presence of painful ophthalmoplegia po due to a granulomatous inflammation gi. The signal intensity of tolosa hunt syndrome in this limited series was similar to that of orbital pseudotumor and is confined to a limited differential diagnosis, which includes meningioma. Report of a case of sphenoid fissure syndrome studied by orbital venography. Gadoliniumenhanced mri is the imaging modality of choice to evaluate the ths and may demonstrate abnormal enlargement and enhancement of the cs extending through the superior orbital fissure into. Glucocorticoids have long been the recommended treatment for tolosa hunt syndrome. Tolosahunt syndrome ths is one of the most common benign causes of painful ophthalmoplegia. Tolosahunt syndrome ths is a painful ophthalmoplegia caused by nonspecific inflammation of the cavernous sinus or superior orbital fissure. This case is a reminder that lymphoproliferative disorders often mimic other neurologic disorders and that tolosa hunt is a rare. Tolosahunt syndrome ths diagnosis is based on clinical manifestations, rapid response to steroids, mri findings and exclusion of other causes of cavernous sinus cs involvement. The patient presented clinical improvement of ophthalmoplegia and headache with corticosteroid therapy, but a month later developed back pain.
This disease usually has a dramatic response to steroids treatment. Painful ophthalmoplegia, tolosa hunt syndrome, criterias of international headache society, highfield mri 1. Chronic granulomatous inflammation of the apex of the orbit. The tolosahunt syndrome is a rare syndrome with an estimated annual incidence of one case per million per year 1. Summary the tolosahunt syndrome consists of a painful ophthalmoplegia, caused by unspecific inflammation of the superior orbital fissure or the cavernous sinus. Reidels thyroiditis and tolosahunt syndrome, a rare. The full text of this article is available in pdf format. The patient may complain of double vision worse at distance, headaches, dizziness, nausea, neck stiffness, photophobia, blurred vision, and a boring pain may be associated with the headache. Lymphoma of the orbit masquerading as tolosahunt syndrome. The etiology appears to be a nonspecific inflammation in the cavernous sinus and superior orbital fissure. Successful treatment of tolosahunt syndrome after a.
Pdf tolosahunt syndrome ths is a painful ophthalmoplegi a caused by nonspecific inflammation of the cavernous sinus or superior orbi tal fissure find. Tolosahunt syndrome ths is a rare disorder characterized by severe and unilateral headaches with orbital pain, along with weakness and paralysis ophthalmoplegia of certain eye muscles extraocular palsies. The painful ophthalmoplaegia had a prompt response to steroid therapy within 72 h and followup brain mri demonstrated signi. The tolosa hunt syndrome is a rare syndrome with an estimated annual incidence of one case per million per year. Tolosa hunt syndrome ths is a rare disorder characterized by severe and unilateral headaches with orbital pain, along with weakness and paralysis ophthalmoplegia of certain eye muscles extraocular palsies. Tolosa hunt syndrome ths is a rare condition characterized by painful ophthalmoplegia paralysis andor weakness of the eye muscles. Orphanet is a european reference portal for information on rare diseases and orphan drugs. The tolosa hunt syndrome consists of recurrent, unilateral painful ophthalmoplegia which responds dramatically to systemic steroid therapy. To present our experience in the neuroradiological diagnosis of six patients with tolosa. It is probable that tolosa hunt syndrome has an inflammatory pathogenesis.
Shortlasting unilateral neuralgiform headache with conjunctival injection and tearing sunct syndrome, is a rare headache disorder that belongs to the group of headaches called trigeminal autonomic cephalalgia tacs. Media in category tolosa hunt syndrome this category contains only the following file. In 1954, tolosa 1 reported a patient with left orbital. In 2004, the international headache society provided a definition of the diagnostic criteria which included granuloma. Tolosa hunt syndrome is usually idiopathic and is thought to be from nonspecific inflammation in the region of the cavernous sinus andor. Tolosa hunt syndrome, a painful ophthalmoplegia scielo. Tolosa hunt syndrome ths is described as severe and unilateral periorbital headache associated with painful and restricted eye movements. Diagnostically, the major ths challenges encountered are owing to the exclusion of other gi presenting conditions necessitating multispecialization consultations. Cavernous sinus mass in a woman presenting with painful ophthalmoplegia cavernous sinus syndrome. Th e patient declared as nonsmoker but acknowledged the habit of occasional. Tolosa hunt syndrome is a diagnosis of exclusion, however magnetic resonance or computed tomographic scans can be used to detect inflammatory changes in the cavernous sinus or orbital fissure and a biopsy can be used to confirm the diagnosis. It presents as a painful ophthalmoplegia of one or more oculomotor cranial nerves, which resolves spontaneously and responds well to treatment with corticosteroids.
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