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Show Journal of Neuro- Ophthalmology 16( 4): 289- 290, 1996. © 1996 Lippincott- Raven Publishers, Philadelphia Feature Photo Painful Ophthalmoplegia Syndrome Secondary to Metastatic Renal Cell Carcinoma Thomas J. Mehelas, M. D. and Gregory S. Kosmorsky, D. O. A healthy 49- year- old Caucasian man was evaluated for persistent headaches and progressive ptosis and ophthalmoplegia. In June of 1994, he developed a constant right retrobular pain that spread into the forehead and temples ( at times graded at 10/ 10 in intensity) as well as photophobia, but without nausea or a stiff neck. He found some relief by lying in a darkroom. There were no previous headaches nor any family history of headaches. He was treated by his primary care physician with Elavil, Calan, and Ex-ecdrin without improvement. His symptoms persisted, and he presented to an emergency room in late July 1994; he was admitted for further evaluation. At that time, he was noted to have a mild horizontal diplopia and partial right blepharoptosis. A computed tomography ( CT) scan of the head was done and read as normal as was magnetic resonance imaging ( MRI) of the brain ( Figs. 1,2). Cerebral angiography was normal ( Fig. 3). Blood studies- complete blood count ( CBC), triglycerides, cholesterol, fasting blood sugar ( FBS), coagulation studies- were also normal. He was diagnosed as having migraines and treated with subcutaneous Imitrex, intravenous Reglan, and DHE, none of which improved his headaches. The patient was discharged on amitrip-tyline and cyprohepatadine. In early August 1994, he was sent for a neuro-ophthalmic evaluation that revealed 20/ 20 vision OD and OS, and 4 mm pupils with no relative Manuscript received October 16, 1995; accepted November 30, 1995. From The Cleveland Clinic, 9500 Euclid Avenue, Cleveland, Ohio 44106. Address correspondence and reprint requests to Dr. G. S. Kosmorsky, 9500 Euclid Avenue, Cleveland, Ohio 44106. afferent pupillary defect. Ocular motility revealed 10% supraduction, 20% infraduction, 0% abduction, and 100% abduction OD, and 100% ductions in all directions OD. Palpebral fissures were 5 mm OD and 15 mm OD. Rodenstock bases 108 were 21 OD and 20 OS. Conjunctivae were normal and there were no orbital cranial bruits. Resistance to globe retropulsion was normal OU. Facial sensation was intact. Biomicroscopy of the anterior segments, funduscopy, and Goldman perimetry ( when the right upper lid was elevated) were normal. The patient was tentatively diagnosed as a presumed Tolosa- Hunt syndrome ( pseudotumor) and given high- dose, tapered, oral corticosteroids ( 100 mg daily) without improvement. Unfortunately, on serial examinations, the right eye visual acuity progressed to no light perception and the patient developed complete ptosis, a " frozen globe" ( no ductions), and persistent headaches with his right cornea and face becoming anesthetic over a 3- month period. Repeat MRI and angiograms were read as normal. A second neuro- ophthalmic examiner interpreted the previous scans as showing a right cavernous sinus enlargement. A repeat high resolution scan concentrating on the cavernous sinus confirmed this suspicion ( Fig. 4). High- dose intravenous therapy did not improve his symptoms or signs. A metastatic workup was suggested. Upon return to his home, CT of the chest and abdomen were performed with the latter showing a large renal cell mass; a biopsy confirmed carcinoma ( Fig. 5). He received 6,000 cGy of radiation to the posterior orbit and cavernous sinus with relief of his headache, but no resolution of the motility defects. He has subsequently died of his disease. 289 290 T. J. MEHELAS AND G. S. KOSMORSKY FIG. 1. Axial CT scan at the level of the cavernous sinus which revealed no specific pathology. FIG. 2. Axial TA weighted image revealing mild enlargement of the right cavernous sinus. FIG. 3. Lateral internal carotid arterial injection revealing no abnormalities on the right. FIG. 4. Coronal T-, weighted MRI revealing enlargement of the right cavernous sinus. FIG. 5. CT scan of abdomen revealing a left renal mass. / Neuro- Ophthalmol, Vol. 16, No. 4, 1996 |