A role for methotrexate in the management of non-infectious orbital inflammatory disease.
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AIM: To evaluate the clinical usefulness of methotrexate for patients with non-infectious orbital inflammatory disease who fail to respond to systemic corticosteroids and/or orbital irradiation. METHODS: The medical records of patients with non-infectious orbital inflammatory disease who were treated with methotrexate at Oregon Health Sciences University between June 1993 and June 2000 were examined. Methotrexate was administered at a median maximum dose of 20 mg per week (range 15-25 mg per week) in conjunction with folate supplementation. Patients were followed with regular ophthalmic examinations, as well as serum liver enzyme levels and blood cell counts. Clinical signs of regression of the orbital inflammation, visual acuity, dosage and duration of methotrexate therapy, requirement for concurrent corticosteroid administration, and adverse drug reactions were recorded. RESULTS: The study cohort included 14 patients (24 eyes) with diagnoses including non-specific orbital inflammation (n=7), Tolosa-Hunt syndrome (n=1), thyroid orbitopathy (n=3), Wegener's granulomatosis (n=1), sarcoidosis (n=1), and Erdheim-Chester disease (n=1). In all cases, methotrexate was commenced as a corticosteroid sparing agent. 10 patients (71%) completed a 4 month therapeutic trial of methotrexate. Median duration of treatment for the nine (64%) patients who experienced clinical benefit was 25 months (range 10-47 months). Six responders were ultimately able to cease methotrexate, including the single patient who required concurrent long term corticosteroid therapy. Complications included fatigue, gastrointestinal disturbance, hair thinning and mild, reversible serum liver enzyme elevation. Two patients (14%) discontinued treatment because of adverse effects. CONCLUSION: Methotrexate is a well tolerated immunosuppressive medication which may benefit patients with recalcitrant non-infectious orbital inflammatory disease. (+info)
Granulomatous pachymeningitis around the sella turcica (Tolosa-Hunt syndrome) involving the hypophysis--case report.
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A 50-year-old female presented with right painful abducens nerve palsy persisting for 4 months and mild panhypopituitarism with diabetes insipidus for 6 months. T(1)-weighted magnetic resonance (MR) imaging of the sellar region showed a homogeneously enhanced mass lesion in the right cavernous sinus which seemed to extend from the swollen pituitary gland. T(2)-weighted MR imaging clearly showed the mass in the right cavernous sinus and the thickened dura mater of the sellar floor as hypointense, and the enlarged pituitary gland as isointense. Biopsy of the thickened dura mater and swollen pituitary gland was performed via the transsphenoidal approach. Histological examination revealed inflammation and collagen fiber formation in these regions. The diagnosis was secondary panhypophysitis resulting from granulomatous pachymeningitis involving the cavernous sinus (Tolosa-Hunt syndrome). Corticosteroid therapy was begun after the biopsy. Her periorbital pain and diplopia were relieved, but diabetes insipidus persisted. Follow-up MR imaging showed a decrease in the volumes of the pituitary gland and the mass in the cavernous sinus. (+info)
Metastatic skull base tumor from thymic carcinoma mimicking Tolosa-Hunt syndrome.
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A 39-year-old male without contributory medical history had sustained progressive double vision, ptosis, and trigeminal pain for 2 weeks. Physical examination revealed total ophthalmoplegia and visual field defect with normal blood examination and chest radiography. Cranial computed tomography revealed a hyperdense mass in the left frontotemporal fossae with bony erosion. Magnetic resonance imaging confirmed a broad-based, intensely enhanced extraaxial tumor of 4x4x4 cm diameter with dural tail sign. Cerebral angiography demonstrated insignificant blood supply both from the internal carotid and middle meningeal arteries. Nearly total tumor resection was achieved via orbitofrontotemporal craniotomy. Intraoperative findings revealed the extraaxial tumor with broad attachment to the dura mater and invasion to the optic and oculomotor nerves. Histological examination revealed hypercellular tumor with significant cell atypism, mitotic activity, and focal necrosis. Immunohistochemical staining was positive for AE1/3 and c-kit, but negative for glial fibrillary acidic protein. Systemic examination performed postoperatively revealed a thymic tumor without additional remote lesions. The final diagnosis was metastatic brain tumor from thymic carcinoma. Rapid progression of neurological impairment inconsistent with a benign extraaxial tumor needs prompt surgical intervention. (+info)
Isolated trochlear nerve palsy in Tolosa-Hunt syndrome.
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A 67-year-old Japanese woman without contributory medical history developed acute onset of left-sided trochlear nerve palsy (TNP) with persistent and severe periorbital pain. There were no other neurological abnormalities. Funduscopic findings were normal. Cranial and orbital magnetic resonance (MR) imaging, and cranial MR angiography demonstrated no abnormalities. By administration of prednisolone 40 mg/day from the day after onset, periorbital pain was resolved within 24 hours, and TNP within 5 days. Thereafter, prednisolone was gradually tapered off. She remained asymptomatic under no medication. In the English language literature, this is the first reported case of Tolosa-Hunt syndrome presenting with isolated TNP. (+info)
Utility of thallium-201 scintigraphy in Tolosa-Hunt syndrome.
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Tolosa-Hunt syndrome (THS) is a rare disorder, especially in the pediatric population, characterized by unilateral painful ophthalmoplegia with a relapsing-remitting course. Because the diagnosis of THS is based on the exclusion of other causes of painful ophthalmoplegia, attention should be paid to possible alternative diagnoses. Thallium-201 chloride ((201)Tl) scintigraphy has been used to evaluate tissue histology in clinical oncology with a marker, the retention index (RI). A higher value indicates histological malignancy. Although its utility in pediatric THS has not been discussed, we suggest that (201)Tl scintigraphy may be informative as a marker in the diagnosis. We present an 11-year-old boy with THS who was evaluated with (201)Tl scintigraphy before treatment with corticosteroids, when he had headache, photophobia, and diplopia. The RI of (201)Tl indicated that the lesion would be benign. Although his clinical symptoms did not fulfill the THS criteria completely, his eye symptoms disappeared 2 weeks after corticosteroid treatment, which was not within the 72 h as in the diagnostic criteria of THS. He has been symptom-free for more than 2 years with only an initial 4-week corticosteroid therapy. This report not only shows the potential of (201)Tl scintigraphy to contribute to the correct diagnosis of pediatric THS but also suggests the possibility that the diagnosis of THS could be supported uniquely even in a pediatric THS-suspicious patient who did not fulfill the current THS criteria completely. In conclusion, we suggest that (201)Tl scintigraphy may be useful for making the diagnosis of THS, especially in pediatric patients. (+info)
Tolosa-hunt syndrome associated with cytomegalovirus infection.
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We herein present the case of a 38-year-old woman with left-sided oculomotor paralysis with ocular pain that developed after a respiratory infection. Her serum was positive for IgM against GM2 and GalNAc-GD1a gangliosides and cytomegalovirus. Thin-slice magnetic resonance imaging revealed enhanced abnormal tissue located primarily in the superolateral part of the left-sided cavernous sinus, which corticosteroids subsequently obscured with immediate resolution of the patient's ocular symptoms. These clinical features were consistent with those of Tolosa-Hunt syndrome (THS). Our findings in the present patient suggest that cytomegalovirus may provoke granuloma formation in the cavernous sinus, as reported in other various organs, thereby leading to the development of THS. (+info)
Primary retroperitoneal diffuse large B-cell lymphoma presenting with numb chin syndrome and painful ophthalmoplegia.
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Numb chin syndrome (NCS) and painful ophthalmoplegia (PO) are neurological syndromes associated with the disturbance of certain cranial nerves and their downstream nerves. These syndromes are caused by various diseases, and, in rare cases precede the diagnosis of systemic malignant lymphoma. We herein present the case of a 59-year-old man diagnosed simultaneously with NCS and PO caused by a tumor located around the cavernous sinus and with diffuse large B-cell lymphoma that was identified via biopsy of a large retroperitoneal tumor. He was successfully treated with a standard rituximab-containing chemotherapy combined with high-dose intravenous methotrexate and intrathecal chemotherapy. (+info)