A New Etiology for Visual Impairment and Chronic Headache: The Tolosa-Hunt Syndrome may be Only One Manifestation of Venous Vasculitis

Abstract

The Tolosa-Hunt syndrome is characterized by recurrent periods of painful ophthalmoplegia, dramatically ameliorated by steroid treatment. In half of the cases orbital phlebography shows characteristic changes. Ninety-six patients with orbital pain characteristic of the Tolosa-Hunt syndrome were submitted to orbital phlebography regardless of the existence of other symptoms. A pathologic phlebogram with changes typical of the Tolosa-Hunt syndrome was found in 50 patients. In 17 of these 50 patients the symptoms consisted of orbital pain only and no ophthalmoplegia. Twenty patients had pain and ipsilateral decrease of vision but no ophthalmoplegia, and 13 had painful ophthalmoplegia. Of 41 patients treated with steroid medication 39 responded dramatically with regard to the pain. Earlier findings indicate that the phlebographic changes are due to venous vasculitis, which thus may cause irreversible visual impairment but also chronic headache without ophthalmoplegia. It is concluded that the Tolosa-Hunt syndrome may be only one manifestation of a not uncommon disease.