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 authors report on the case of a 32 year old woman treated with prednisone and diphenylsulfon for a multi-visceral sarcoidosis; headache and progressive oculomotor paralysis revealed an expansive lesion of the left cavernous sinus extending to the temporal fossa. C.T. scan, M.R.I., and angiographic data as well as the intra-operative findings evoked the diagnosis of meningioma. Pathological findings showed an epithelioid and gigantocellular granuloma without caseous necrosis, corresponding to a sarcoidosic lesion. After the operation, the increase of steroid doses was followed by a complete regression of the tumoral remnants, and total clinical recovery. In a patient having a previously diagnosed sarcoidosis with evidence of an intracranial tumor mimicking a meningioma, steroids should be first prescribed. Efficacy of steroids on sarcoid granulomas is often dramatic; surgical approach would be discussed in case of ineffective steroid therapy, of persisting questionnable diagnosis, and of tumoral threatening compression.
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PMID:[Sphenocavernous localization of meningeal neurosarcoidosis. Apropos of a case and review of the literature]. 824 16

Since Hunt et al's description (1961) Tolosa-Hunt syndrome has been a matter of controversies about its nosological identity and differential diagnosis. We report 7 cases diagnosed between 1979 and 1990. Four of them had a low-resolution CT and the diagnosis was made after a long follow-up, according to classical criteria of exclusion. The last 3 cases had a modern high-resolution CT or a MRI (2 cases) and the diagnosis was established in a few days. MRI has considerably simplified the differential diagnosis but it has not completely resolved it. Alterations of shape and signal of the cavernous sinus are similar to those of lymphoma, sarcoidosis and meningioma. When MRI is normal diabetic ophthalmoplegia, migrainous ophthalmoplegia and giant-cell arteritis must also be considered. We conclude that the diagnosis of the Tolosa-Hunt syndrome can generally be made in a few days, without a cavernous sinus biopsy. There is no reason to doubt that this syndrome is a specific nosological entity.
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PMID:[Should Tolosa-Hunt syndrome still be an exclusion diagnosis?]. 827 25

Direct surgery to cavernous sinus (CS) lesions has become one of the optimal treatments based on advancement in microsurgical anatomy and imaging modality, and the progress of microsurgical techniques. We have removed the CS or Meckel's cave tumors extradurally when they do not extend intradurally, using modified Al-Mefty's cranio-orbital zygomatic craniotomy. Three CS tumors; trigeminal neurofibroma, squamous cell carcinoma and chondrosarcoma, and a Meckel's cave meningioma were reported. Total removal was achieved in all but one (case 4). Postoperative complications were permanent ophthalmoplegia in one, transient ophthalmoplegia in one, subcutaneous CSF accumulation in two and trigeminal dysfunction in one. The extradural approach can be the first choice of methods for total removal of tumors when they are confined to the CS or Meckel's cave.
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PMID:[Cranio-orbital zygomatic extradural approach for cavernous sinus or Meckel's cave tumors]. 867

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

Intracranial meningiomas in children are rare, representing 1-4.2% of central nervous system tumors and 1.5-1.8% of all intracranial meningiomas. Meningiomas arising from the lateral wall of the cavernous sinus account for less than 1% of all intracranial meningiomas. To our knowledge, only one case of a meningioma arising from the cavernous sinus has been reported in childhood. A 6-year-old boy presented with left ophthalmoplegia. A slight drooping of the left eyelid was noted at the age of 1 year. Magnetic resonance imaging (MRI) with contrast administration revealed an enhancing mass lesion located in the left cavernous sinus. The tumor, arising from the lateral wall of the cavernous sinus, was totally removed and the oculomotor nerve was reconstructed with a sural nerve graft. MRI displayed total tumor removal 1 month after the surgery. The pathological diagnosis was of a psammomatous meningioma.
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PMID:Meningioma of the cavernous sinus in a child. 1006 14

A 23-year-old woman was admitted with headache, nausea, vomiting and blurred vision on the left side. Neurological examination showed ptosis with a complete internal and external ophthalmoplegia and a red fullness around the left orbita. Computed tomographic scanning of the brain revealed no abnormalities. As she improved on high doses of steroids a diagnosis of Tolosa-Hunt syndrome (THS) seemed to be indicated. However, magnetic resonance imaging (MRI) showed a lesion with intermediate signal intensity in the left cavernous sinus. Craniotomy was performed when symptoms of THS recurred. Histopathological examination revealed a meningioma with a papillary aspect and some mitoses. This case illustrates that: (1) THS is still a diagnosis by exclusion; (2) MRI and histopathological examination are important if there is any doubt about the diagnosis; and (3) also when there is no doubt, improvement after steroid therapy may be a diagnostic pitfall. Therefore, not only MRI but also orbital phlebography and angiography should seriously be considered.
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PMID:Meningioma presenting as Tolosa-Hunt syndrome. 1035 Jan 98

Dysthyroid orbitopathy (DO) is an autoimmune disorder usually associated with Graves' disease. The extra-ocular muscles are the target of the autoimmune attack. As a result, they become enlarged, producing restrictive ophthalmoplegia and proptosis. Other cardinal signs of DO include upper eyelid retraction and lag, conjunctival injection and chemosis, and periorbital edema. Visual loss may occur if the enlarged extra-ocular muscles compress the optic nerve in the orbital apex. These cardinal signs may mimic neurological conditions, including Parinaud's syndrome, sixth nerve palsy, carotid-cavernous fistula, and spheno-orbital meningioma. Treatment is directed at the cardinal signs and is largely palliative. Under certain clinical conditions, judicious use of corticosteroids, radiation therapy, or surgery designed to decompress the orbit, correct strabismus, or restore normal lid position may help to restore visual function.
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PMID:Dysthyroid orbitopathy. 1087 76

Unilateral internal and external ophthalmoplegia caused by an intracranial meningioma occurred in a 15-year-old Belgian Sheepdog. The dog initially presented with ventro-lateral strabismus of the left eye, and ptosis of the left upper eyelid. Anisocoria was present with the left pupil fixed and dilated. Both eyes were visual. Neuro-ophthalmic evaluation revealed a lesion located in the left oculomotor nerve. Pharmacological testing with dilute pilocarpine (0.1% in artificial tears) revealed evidence of parasympathetic denervation of the left eye. Further evaluation via magnetic resonance imaging (MRI) revealed a well-defined mass to the left of midline and lateral to the sella turcica. An attempt was made to excise/debulk the mass due to worsening conditions and the dog died the following day. Necropsy revealed a mass of randomly arranged bundles and streams of spindle cells. Immunohistochemistry demonstrated a strong avidity for vimentin and a negative response for S-100 protein. These findings suggest a diagnosis of meningioma.
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PMID:Unilateral external and internal ophthalmoplegia caused by intracranial meningioma in a dog. 1139 75

Meningiomas originating in Meckel's cave (MC) are uncommon lesions that represent 1% of all intracranial meningiomas. Innovations in skull base surgery have enabled resection of these lesions with less morbidity, but require an intimate knowledge of both lesional pathology and regional microneuroanatomy. To review the surgical and clinical considerations involved in the management of MC meningiomas, we retrospectively reviewed data from patients who underwent transpetrosal resection of primary MC meningiomas between 1984 and 1998. Of 146 patients who underwent transpetrosal removal of meningiomas, 7 were believed to have tumors originating in MC. All 7 patients presented with trigeminal dysfunction, facial pain, and/or headache. Complete tumor removal was achieved in 5 of the 7 patients. Facial hypoesthesia or anesthesia, paralysis of cranial nerve VI, and ophthalmoplegia were among the postoperative complications encountered. Meningiomas of MC represent treatable lesions whose diagnosis requires prompt imaging of patients with trigeminal dysfunction and symptoms of facial pain and headache.
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PMID:Meningioma involving Meckel's cave: transpetrosal surgical anatomy and clinical considerations. 1229 43

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.
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PMID:MRI findings in Tolosa-Hunt syndrome before and after systemic corticosteroid therapy. 1253 85


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