Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029089 (ophthalmoplegia)
 3,338 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The so-called Tolosa-Hunt syndrome consists of painful ophthalmoplegia caused by chronic nonspecific inflammation of the cavernous sinus and/or superior orbital fissure, responsive to steroid therapy. We present a case with the unusual feature of sellar erosion, in which angiography. CT and MRI suggested this idiopathic condition.
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PMID:Tolosa-Hunt syndrome with sellar erosion: case report. 837 19

The authors refer to a case report in which two rare clinical syndromes, the Tolosa Hunt Syndrome (THS) and the polyglandular auto-immune syndrome (PGA) co-exist in the same patient and make a detailed analysis of the literature regarding this kind of diseases. The THS is a rare clinical condition characterized by a painful ophthalmoplegia due to an involvement of the cranial nerves and of the sympathetic nerve-fibres going through the cavernous sinus and the superior orbit cavity. Nowadays the diagnosis of TSH is made by high resolution computerized tomography aimed at the cavernous sinus and the upper orbital cavity. This method allows to notice if at the basis of this syndrome there are vascular, neoplastic or inflammatory diseases or if, as in e case here presented, by exclusion, we must think of an inflammatory process with unknown pathogenesis. In this case report the patient under observation process to be affected by THS and also by auto-immune polyglandular syndrome type III, as she was affected by diabetes type I, Basedow disease and alopecia. The authors believe that an auto-immune mechanism can be at basis of the THS, and this fact has already been pointed out in the literature in two previous works. An important feature of this case report has been the definitive improvement of the symptoms by an antithyroid effect and, also, by an immune suppressor activity. The Authors hope that there will be reported a greater number of cases pointing to the coexistence of the THS with autoimmune diseases.
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PMID:[Tolosa Hunt syndrome and autoimmune polyglandular syndrome. A rare case report]. 853 98

Two patients with Tolosa-Hunt syndrome (THS) who had atypical lesions in the intrasellar and juxtasellar regions are reported. They manifested with painful ophthalmoplegia. Magnetic resonance imaging (MRI) commonly showed an increase in the volume of the cavernous sinus occupied by homogeneously well-enhanced lesions in both cases. These lesions extended to the intrasellar and juxtasellar regions with meningela enhancement. Follow-up MRI after steroid treatment demonstrated normalized or decreased size of the cavernous sinus. These findings suggested nonspecific inflammatory granulomatosis with atypical extension.
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PMID:Tolosa-Hunt syndrome with atypical intrasellar and juxtasellar lesions--two case reports. 875 20

We report a case of painful ophthalmoplegia (Tolosa-Hunt syndrome), which was complicated with diabetes insipidus (DI) and pituitary-adrenal axis hypofunction. A 42-year-old man hospitalized for left orbital pain and impairment of left cranial nerves III, IV, V, VI, developed diabetes insipidus during the corticosteroid treatment. Neuroimaging studies disclosed a thickened, highly contrast-enhanced pituitary stalk, swollen pituitary gland and widened left cavernous sinus up to the superior orbital fissure, which were accompanied by diabetes insipidus and hypofunction of the pituitary-adrenal axis. These were indicative of an extension of granulomatous inflammation of the cavernous sinus to the pituitary portal system and the gland itself.
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PMID:Inflammatory involvement of the hypophysis in Tolosa-Hunt syndrome. 877 71

Tolosa-Hunt syndrome (THS) is characterized by painful ophthalmoplegia due to idiopathic granulomatous inflammation of the cavernous sinus. Steroid therapy dramatically reverses the clinical signs and symptoms of THS. We report a typical case of THS, in which MRI showed a mass lesion in the right cavernous sinus with intermediate signal intensity on T1-weighted images, hypointensity on T2-weighted images and positive gadolinium enhancement. This 24-year-old man had three episodes of painful opthalmoplegia in a period of 9 months. The side of the involved cavernous sinus was different in the first and the second episodes of THS. The symptoms lasted for 1 month and the patient recovered spontaneously from these two episodes. In the third episode, the right pupil was also involved and the light reflex was lost. After steroid therapy, the ocular symptoms improved rapidly within 48 hours and the mass lesion in the right cavernous sinus on MRI was markedly diminished by the 7th day of treatment.
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PMID:Magnetic resonance images of Tolosa-Hunt syndrome before and after steroid therapy. 884 Jul 63

We describe a 60-year-old man with a history of Tolosa-Hunt syndrome associated with intermittent painful ophthalmoplegia and a visual disturbance on the left side, who presented with signs and symptoms of severe hypoadrenalism and diabetes insipidus. Magnetic resonance imaging demonstrated enlargement of the hypophysis and infundibulum and left cavernous sinus. An endocrinologic study revealed anterior pituitary dysfunction and diabetes insipidus. The patient underwent a transsphenoidal biopsy which revealed chronic inflammation in the hypophysis, mucosa of the sphenoid sinus, and dura mater. The patient was treated with steroids that decreased the size of the hypophysis and infundibulum, but the symptoms of anterior pituitary insufficiency and diabetes insipidus have persisted. The chronic inflammation of the hypophysis and infundibulum is thought to have spread from the cavernous sinus.
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PMID:Parasellar chronic inflammatory disease presenting Tolosa-Hunt syndrome, hypopituitarism and diabetes insipidus: a case report. 898 Aug 89

Unexpected autopsy findings are presented of a patient who died suddenly after a 6-month history of progressive headaches, nausea, and vomiting. A ruptured Rathke's cleft cyst (RCC) was identified within the adenohypophysis, with a chronic inflammatory reaction in surrounding pituitary and overlying hypothalamus. A brisk lymphoplasmacytic response was also seen in the cavernous sinuses bilaterally, identical to the pathology reported for idiopathic painful ophthalmoplegia, also called Tolosa-Hunt syndrome (THS). The pathogenesis of THS has not been elucidated; based on the findings in this report, it is suggested that some THS cases may result from a hyperimmune response to RCC rupture with extension into one or both cavernous sinuses. Although prompt alleviation of symptoms with corticosteroid treatment is generally encountered with THS, recurrence of symptoms is not uncommon. A careful search for a ruptured RCC should be undertaken in atypical cases of THS, with possible consideration of surgical intervention.
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PMID:Ruptured Rathke's cleft cyst: a possible cause of Tolosa-Hunt syndrome. 910 Nov 12

We report a case of Tolosa-Hunt syndrome (THS) in which the lesion has been demonstrated by magnetic resonance imaging (MRI), computed tomography (CT), and angiography. A healthy 23-year-old man developed an acute painful ophthalmoplegia on the right side. CT and MRI scans revealed asymmetric enlargement of the right cavernous sinus with contrast enhancement extending down to the region of trigeminal ganglion. MRI further delineated the detailed anatomical structures of the region and excluded any infiltration of the surrounding tissues by a mass lesion. Cerebral angiography showed a significant decrease in the calibration of petrous segment and a mild decrease in the calibration of cavernous segment of the ipsilateral internal carotid artery. The patient was treated with oral prednisone, 100 mg daily. Neurological findings totally subsided after 2 weeks on corticosteroid and MRI showed resolution of the lesion in the cavernous sinus. The patient was symptom-free for 6 months after discharge. Our findings have suggested that MRI is the most valuable imaging technique for demonstration and follow-up of lesions in the cavernous sinus that are directly responsible for the symptoms of THS and the lesions can be more extensive than was currently believed.
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PMID:The value of MRI in a case of Tolosa-Hunt syndrome. 921 63

The oculomotor nerve of a 6-year-old boy with recurrent headache and recurrent ophthalmoplegia was contrast-enhanced on a magnetic resonance imaging (MRI) scan during an episode. The boy exhibited dramatic response to steroid treatment. The clinical features of ophthalmoplegic migraine and Tolosa-Hunt syndrome overlapped in this patient. We suggest that a positive MRI finding can be included as one of the diagnostic criteria in the classification of ophthalmoplegic migraine and that a trial of steroid is worthwhile in the presence of enhancement of the oculomotor nerve since ophthalmoplegic migraine may be noninfectious but inflammatory in etiology.
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PMID:Enhancement of oculomotor nerve: a diagnostic criterion for ophthalmoplegic migraine? 930 81

The Tolosa-Hunt syndrome (THS) consists of a painful ophthalmoplegia related to granulomatous inflammatory process in the cavernous sinus. According to recent concepts, the diagnosis is established only when other causes of painful ophthalmoplegia are ruled out. A typical pattern of response to corticosteroids associated with a benign evolution may reinforce this possibility. Tumors such as lymphoma and meningioma and orbital pseudotumors can make difficult the differential diagnosis because they also may respond to steroids. Thus it is always necessary to make an extensive ancillary investigation. We performed a clinical, laboratory and radiologic study of five patients with THS. Prednisone was used in all, with dosages ranging from 40 to 80 mg/day. In four patients there was a dramatic analgesic effect in less than 48 hours. Improvement of the ophthalmoplegia was not so fast but occurred in all with a complete remission in 4 to 45 days.
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PMID:[Tolosa-Hunt syndrome. Troubles in diagnosis and pattern of response to prednisone]. 933 68


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