Disease Information for Tolosa-Hunt syndrome

An idiopathic syndrome characterized by the formation of granulation tissue in the anterior cavernous sinus or superior orbital fissure, producing a painful ophthalmoplegia;--------(Adams et al, Principles of Neurology, 6th ed, p271)-------------Tolosa-Hunt syndrome (William Edward Hunt); Also known as: Tolosa-Hunt ophthalmoplegia; Cavernous sinus syndrome, cavernous sinus granulomatosis, ophthalmoplegia dolorosa, ophthalmoplegia pain, orbital apicitis, orbital pain, painful ophthalmoplegia, recurrent algetic ophthalmoplegia, recurrent ophthalmoplegia; Description: A syndrome characterized by recurrent, sharp unilateral retro-orbital pain with extraocular palsies, usually involving the third, fourth, fifth, and sixth cranial nerves; Other features include proptosis and sensory loss over the forehead, sluggish pupil reaction to light, and diminished corneal sensitivity with blurred vision up to complete blindness; It is attributed to a granulomatous infiltration or an invasive tumour at the apex of the orbit or in the region of the cavernous sinus; Males and females equally affected; The disease usually lasts several months to several years; Onset usually in the fifth decade------------[whonamedit-com website 2005]-------------Tolosa-Hunt Syndrome is a painful unilateral ophthalmoplegia caused by a granulomatous infiltration of nerves and muscles in the region of the superior orbital fissue; It is accompanied by partial or complete lesions of the third, fourth, fifth - ophthalmic division - and sixth cranial nerves; The ESR is usually raised; Treatment with steroids eg prednisolone is usually [quickly] effective

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