Tolosa-Hunt syndrome: one cause of painful ophthalmoplegia

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✓ The author reports 12 cases of Tolosa-Hunt syndrome, a benign steroid-resistant cryptogenic granuloma that presents as painful ophthalmoplegia. This syndrome is differentiated from other causes of painful ophthalmoplegia including tumors, aneurysms, collagen disease, specific infections, mucoceles, and benign granulomas of unknown etiology. These other conditions should be excluded by appropriate tests. Surgical exploration is not necessary if there is a prompt remission on steroid therapy.

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Address reprint requests to: William E. Hunt, M.D., N-907 University Hospital, 410 West 10th Avenue, Columbus, Ohio 43210.

© AANS, except where prohibited by US copyright law.

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Figures

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    Photomicrographs of the autopsy specimen, by courtesy of Dr. Tolosa. Left: Granulomatous lesion fusing the adventitia of the carotid artery to the abducens nerve. H & E, × 10. Right: Granulomatous tissue showing scarcity of vessels, numerous lymphocytes, and plasma cells. H & E, × 200.

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    Studies in a patient later proved to have a metastatic malignancy. Left: Skull film showing erosion of the right anterior clinoid process and the lateral wall of sphenoid sinus with mass protruding into the sinus. Right: Orbital venogram. The right cavernous sinus is patent but the third segment of the right ophthalmic vein is stenotic with distended first and second segments and collateral dilatation suggestive of extrinsic pressure.

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    Photomicrograph of an orbital pseudotumor biopsy, by courtesy of Dr. Makley and Dr. Lubow. Numerous lymphocytes and plasma cells with few vessels are seen. H & E, × 40.

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