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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The differential diagnosis of painful ophthalmoplegia is extensive and consists of numerous sinister etiologies, including neoplasms (ie, primary intracranial tumors, local or distant metastases), vascular (eg, aneurysm, carotid dissection, and carotid-cavernous fistula), inflammatory (ie, orbital pseudotumor, giant cell arteritis, sarcoidosis, and Tolosa-Hunt syndrome), and infectious etiologies (ie, fungal and mycobacterial), as well as other miscellaneous conditions (ie, ophthalmoplegic migraine and microvascular infarcts secondary to diabetes). A systematic approach to the evaluation of painful ophthalmoplegia can lead to prompt recognition of serious disorders that can be associated with significant morbidity or mortality if left untreated. Inflammatory conditions such as Tolosa-Hunt syndrome and orbital pseudotumor are highly responsive to corticosteroids but should be diagnoses of exclusion.
Curr Pain Headache Rep 2007 Aug
PMID:An approach to the patient with painful ophthalmoplegia, with a focus on Tolosa-Hunt syndrome. 1768 98

Tolosa Hunt syndrome is a rare disorder caused by nonspecific inflammation in the cavernous sinus/superior orbital fissure and/or orbital apex. It is clinically characterized by alternating remissions and exacerbations, and manifested as diplopia associated with unilateral periorbital hemicranial headache. The symptoms include blepharoptosis, which is usually mild if present, bulbomotor paresis involving the pupil, and loss of sensation in the area supplied by the first division of the trigeminal nerve. Therapy for Tolosa-Hunt syndrome are systemic steroids. The course of disease in a 25-year-old man hospitalized for painful ophthalmoplegia and diplopia is presented. The history included severe pain on rightward eye movement and parabulbarly on the right, considerable defect in the area supplied by the first division of the trigeminal nerve, right hemicrania, and diplopia on looking to the left, right, upward and downward that developed after four days. A month before, the patient was observed at neurology department for severe right hemicrania. Current status included severe pain parabulbarly on the right, discrete proptosis with mild ptosis on the right eye, restricted right eye bulbar motoricity on looking nasally, nasally upward and downward, and loss of sensation in the area supplied by the first division of the trigeminal nerve. Pupilar motoricity was normal. Upon admission, neuroradiologic examination (orbit CT) and brain MR were performed, and therapy with systemic corticosteroids was initiated ex iuvantibus, in consultation with a neurologist. At 24 hours of corticosteroid therapy, the pain subsided, whereas diplopia disappeared almost completely after 5 days, now being only occasionally recorded on looking to the left and upward.
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PMID:[Painful ophthalmoplegia--Tolosa-Hunt syndrome]. 1804 75

A 19-year-old immune-competent patient developed right-sided headache and, subsequently, subacute diplopia. On clinical examination he had incomplete right oculomotor palsy. Cranial MRI showed pathologic contrast enhancement of the right oculomotor nerve at its exit point from the mesencephalon, and the CSF displayed slight pleocytosis. The following relevant differential diagnoses were not supported by additional examinations: neurosarcoidosis, Lyme neuroborreliosis, neurosyphilis, tuberculous meningitis, viral meningitis (HIV, VZV, CMV), CNS lymphoma, vasculitis associated with rheumatic disease, Tolosa-Hunt syndrome, and diabetic neuropathy. However, on the basis of blood lymphocytosis, positive heterophile antibody test (Paul-Bunnell test), the presence of IgM antibodies against Epstein-Barr virus capsid antigen, and elevated transaminases, infectious mononucleosis was diagnosed. Isolated neuritis of the oculomotor nerve is a rare parainfectious manifestation of infectious mononucleosis.
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PMID:[Isolated neuritis of the oculomotor nerve in infectious mononucleosis]. 1805 80

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.
Cephalalgia 2008 Jun
PMID:ICHD-II diagnostic criteria for Tolosa-Hunt syndrome in idiopathic inflammatory syndromes of the orbit and/or the cavernous sinus. 1838 13

Idiopathic Tolosa-Hunt syndrome (ITHS) is a very rare cause of painful ophthalmoplegia characterized by unilateral orbital pain, ipsilateral oculomotor paralysis and prompt response to steroids. In this paper we report 4 additional cases of ITHS. This rare cause of painful ophthalmoplegia effects the cranial nerves due to a granulomatous lesion of unknown etiology in the cavernous sinus or superior orbital fissure. The International Headache Society redefined the diagnostic criteria for ITHS but it is still mostly a diagnosis of exclusion. Careful evaluation and follow-up is essential for diagnosis. Optimal therapy duration and dosage and prophylactic treatment in recurrent cases needs further research.
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PMID:Idiopathic Tolosa-Hunt syndrome: four additional cases. 1876 81

Tolosa-Hunt syndrome (THS) is a very rare, relapsing, and remitting painful ophthalmoplegia caused by nonspecific granulomatous inflammation in the cavernous sinus. To our knowledge, bilateral complete, simultaneous palsies of all 3 cranial nerves associated with extraocular movement have not been reported. We describe the first such patient with bilateral THS that responded quickly to corticosteroid therapy. A 54-year-old man presented with a periorbital and frontal headache with acute bilateral severe blepharoptosis and fixed eyes, which dramatically responded to corticosteroid therapy. He had diabetes mellitus type II. Brain MRI showed granulomatous inflammation in both cavernous sinuses and thickening of the surrounding dura mater of the cranial base, suggesting the coexistence of focal hypertrophic cranial pachymeningitis. Our experience indicates that steroid therapy with strict control of blood sugar should be considered in patients with THS complicated by diabetes. MRI is a valuable tool for serially monitoring the response of lesions to treatment in THS.
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PMID:Serial neuroimaging in tolosa-hunt syndrome with acute bilateral complete ophthalmoplegia. 1945 27

Tolosa-Hunt syndrome (THS) is characterized by unilateral painful ophthalmoplegia with oculomotor paresis, associated with an idiopathic granulomatous inflammation involving the cavernous sinus, with a typical relapsing-remitting course. We report a case of an 8-year-old girl who was admitted because of an ophthalmoplegia with exotropia and ptosis of the left eyelid, accompanied by diplopia and left sovraorbital pain. The clinical data, neuroradiological findings and response to steroid treatment suggested THS, as defined by the 2004 International Classification of Headache Disorders (ICHD)-II criteria. THS must be considered a possible cause of painful ophthalmoplegia in childhood, as well as in adults, and confirmed with a focused neuroradiological investigation. The few paediatric cases described in the literature that meet the 2004 ICHD-II criteria are not sufficient to identify possible differences between the paediatric and the adult forms. Every new paediatric case should therefore be reported in order to gather and compare further information.
Cephalalgia 2009 Nov
PMID:The Tolosa-Hunt syndrome in children: a case report. 1955 37

Tolosa-Hunt syndrome (THS) consists of a painful ophthalmoplegia with typical features in magnetic resonance imaging (MRI). The recurring nature of this affliction has been known since its first description. However, compromise of the contralateral cavernous sinus, known as alternating THS, is very rare and has never been examined using MRI. We report clinical data, laboratory data and imaging features of a patient with alternating THS. According to our literature review, this is the first MRI study of THS.
Cephalalgia 2010 Sep
PMID:A case review of the MRI features in alternating Tolosa-Hunt syndrome. 2071 64

We report on a previously healthy 11-year-old boy with unilateral periorbital mild headache and facial nerve palsy, followed during the next 5 months by recurrent unilateral headaches and subsequent extrinsic paresis of the third cranial nerve and paresis of the sixth cranial nerve, each of which improved with steroids. Cranial magnetic resonance imaging revealed increased thickening of the left cavernous sinus and adjacent structures, with marked gadolinium enhancement. Eight months after the initial signs, he developed left retro-orbital headache that lasted 16 days and was relieved 24 hours after resuming steroid treatment. Recurrent multiple cranial neuropathies, neuroimaging findings, and long-lasting headache that responded to steroids indicated Tolosa-Hunt syndrome, further confirmed by extensive investigation and a long follow-up to exclude other causes. This patient illustrates the complexity of disorders with multiple cranial nerve palsies, and adds to the sparse literature on Tolosa-Hunt syndrome in children, describing the first pediatric case preceded by facial palsy.
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PMID:Tolosa-Hunt syndrome preceded by facial palsy in a child. 2155 63

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.
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PMID:Unusual clinical course in pediatric Tolosa-Hunt syndrome. 2155 61


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