Introduction Painful ophthalmoplegia can be caused by a variety of conditions such as vascular, neoplastic, infiltrative, and infectious diseases affecting the cavernous sinus and causing unilateral orbital pain associated with paresis of one or more of cranial nerves (IV, III, and VI) and para- or sympathoplegia of the eye [1]. The term Tolosa-Hunt syndrome refers to a painful ophthalmoplegia due to non-specific granulomatous inflammation in the cav- ernous sinus or superior orbital fissure [2, 3, 4]. It is a rare condition whose incidence has been estimated to be of approximately one to two cases per million [5]. It is of unknown origin, responds dramatically to steroids, and often remains a diagnosis of exclusion. It is character- ized by typical MRI findings of enlargement of the clin- ically affected cavernous sinus by abnormal tissue with marked contrast enhancement [6]. The MR findings, although characteristic, may be subtle; therefore, great care must be taken in choosing the appropriate tech- nique and in evaluating neuroradiological findings in the light of a thorough clinical evaluation. Follow-up examinations with MR are recommended to confirm the diagnosis and to exclude malignant tumors since distinctive MRI criteria do not yet exist [4]. We report a case of a woman affected by systemic lupus erythematosus (SLE) who developed THS. Case report A 36-year-old woman was admitted to our hospital with a 2-week history of unremitting right periorbital pain that spread to the ip- silateral frontal and temporal regions, followed by onset of ptosis of the right eyelid and hypoaesthesia of the right side of the face. In 1997, after an episode of facial erythema and positive anti- nuclear antibodies titer, a diagnosis of SLE was made and con- firmed by skin biopsy. She was successfully treated with steroids and hydroxychloroquine with a subsequent clinical remission and normalization of the antibody titer. Examination on admission showed anisocoria, delayed light reflex, paralysis of extraocular muscles, hypoaesthesia, and pain in the distribution of the first branch of the fifth cranial nerve, all on the right side. Relevant laboratory data including hematology, erythrocyte sedimentation rate, serology, blood chemistry, anti- nuclear antibodies titer, and anti-DNA antibodies were normal. Eur. Radiol. (2002) 12:341±344 DOI 10.1007/s003300100960 NEURO Valentina Calistri Claudio Mostardini Patrizia Pantano Alberto Pierallini Claudio Colonnese Francesca Caramia Tolosa-Hunt syndrome in a patient with systemic lupus erythematosus Received: 24 November 2000 Revised: 4 April 2001 Accepted: 9 April 2001 Published online: 12 July 2001  Springer-Verlag 2001 V.Calistri ´ C.Mostardini ´ P.Pantano ´ A. Pierallini ´ F. Caramia ( ) ) Department of Neurological Sciences, University of Rome ªLa Sapienzaº, Viale dell' Università 30, 00185 Rome, Italy E-mail: francesca.caramia@uniroma1.it Phone: +39-06-49 91 47 19 Fax: +39-06-49 91 49 03 C. Colonnese ´ F. Caramia IRCCS Neuromed, 86077 Pozzilli (Isernia), Italy Abstract We report a case of To- losa-Hunt syndrome (THS) in a pa- tient with systemic lupus erythema- tosus studied with MRI. Magnetic resonance showed enlargement of the cavernous sinus and compres- sion of the carotid syphon by en- hancing tissue. In particular, fat- suppressed T1-weighted images be- fore and after contrast agent injec- tion and MR angiography showed extension of the abnormal tissue to the apex of the orbit and narrowing of the internal carotid artery. A pre- sumptive diagnosis of THS was made and steroid treatment was started with rapid relief of symp- toms. Follow-up MR study after steroid therapy demonstrated sub- total resolution of the neuroradio- logical findings. Neuroradiological findings in THS are quite typical but they may be subtle; furthermore, the presence of a systemic disease may suggest secondary involvement of the cavernous sinus. Utilization of the appropriate MR techniques and follow-up exams may contribute to the diagnosis of THS even in the presence of other systemic diseases. Keywords Tolosa-Hunt syndrome ´ Painful ophthalmoplegia ´ Systemic lupus erythematosus ´ MRI ´ MR angiography