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Show "~ 1986 Raven Press, New York Ischemic ("Diabetic") Cavernous Sinus Syndrome Gregory S. Kosmorsky, D.O., and Robert L. Tomsak, M.D., PH.D. A 65-year-old diabetic woman presented with an acute cavernous sinus syndrome involving the right third, fifth, and sixth cranial nerves. The complete neuroophthalmic workup included high-resolution computed tomography, angiography, lumbar puncture, and laboratory studies, all of which were unrevealing. The cranial nerve palsies spontaneously resolved in 10 weeks, consistent with an ischemic etiology. From the Cleveland Clinic Foundation, Cleveland, Ohio. Address correspondence and reprint requests to Dr. G. S. Kosmorsky at Cleveland Clinic Foundation, 9500 Euclid Ave., Cleveland, OH 44106, U.S.A. 96 The onset of a cavernous sinus syndrome should prompt further evaluation. The etiologies are legion but some are treatable. Diabetes as a cause of multiple simultaneous cranial nerve palsies is very unusual and must therefore remain a diagnosis of exclusion. CASE REPORT On April 1, 1985, a 65-year-old Caucasian woman developed a painful right eye followed immediately by variable (horizontal or vertical) diplopia. The pain diminished over a few days and took on a "toothache-like" character. There were no facial dysesthesias. Twelve days later she developed ptosis on the right, with no change in pain or diplopia. She was referred to the Neuroophthalmology Section at the Cleveland Clinic Foundation on April 23, 1985. Her past medical history was significant for diabetes mellitus for 19 years, treated with oral medication. She had no known diabetic retinopathy. She denied a history of high blood pressure or any other medical condition. Examination showed the patient's best corrected visual acuity to be 20/30 and J1 with each eye. The pupils measured 4 mm, with normal reaction to light and near, and no afferent pupillary defects. Four millimeters of ptosis was noted on the right. Motility examination revealed a partial right third nerve paresis involving mainly the medial rectus and the superior rectus muscles. The right inferior rectus muscle was involved to a lesser degree. In addition, there was mild paresis of abduction (Fig. 1). Corneal sensation was depressed on the right but facial sensation was otherwise normal. Backgr~ und diabetic retinopathy was present without eVidence of neovascularization. The visual fields were normal. The diagnosis of acute cavernous sinu~ syndrome was made, and the patient was admitted to the Cleveland Clinic Foundation Hospital on April 23, 1985. ISCHEMIC CAVEl~NOUS SINUS SYNDROME FIG. 1. April 23. 1985. Note ptosis 00. with equal pupils. There is limitation 00 of supraduction and adduction. and mild abduction weakness 00 also. 97 A neurologic examination revealed no other abnormalities. A computed tomographic scan with and without contrast enhancement was unremarkable and included overlapping thin sections of the cavernous sinus region and orbit apex. Lumbar puncture showed a glucose level of 123 mg/dl (simultaneous serum glucose level of 233 mg/dl), and a protein level of 47 mg/dl. CSF white blood count was 7/mm3 (all lymphocytes). Cytology for malignant cells was negative. A CSF-VDRL was nonreactive. An intraarterial digital subtraction angiogram showed no evidence of an intracavernous aneurysm or the typical intracavernous narrowing frequently seen with the Tolosa- Hunt syndrome. There was, however, a smooth narrowing of the supraclinoid portion of the right internal carotid artery consistent with atherosclerosis. Laboratory studies included a Westergren sedimentation rate of 62 mm/h. Collagen vascular studies (antinuclear antibody, rheumatoid factor, and complement) were normal. An FTA-ABS was nonreactive. A temporal artery biopsy was performed and showed only mild intimal fibroplastic changes without evidence of giant cell arteritis. Because of the possibility of inflammatory pseudotumor of the cavernous sinus (Tolosa-Hunt syndrome) or temporal arteritis, the patient was begun on a regimen of 60 mg prednisone per day on April 26, 1985, and discharged from the hospital. On May 3, 1985, she stated that her headaches were gone but that she had had neck and shoulder pains for the preceding 6 days. Ophthalmoplegia and ptosis were unchanged but corneal sensation was equal. The pupillary examination was unchanged. Steroids were discontinued at this time. On May 10, 1985, only 2 mm of ptosis was found. Ocular motility was improved. Examination on June 4, 1985, showed an almost complete recovery of the third and sixth nerve paresis. By July 9, 1985, the patient was asymptomatic, with normal ocular movements and lid function (Fig. 2). DISCUSSION We believe this case represents an unusual complication of adult-onset diabetes mellitus, that is, simultaneous ischemic cranial nerve palsies on one side presenting as an acute cavernous sinus syndrome. While separate multiple cranial nerve palsies secondary to diabetes mellitus are not unusual (1-4), simultaneous involvement of the third, fourth, fifth, or sixth nerves secondary to diabetes mellitus is rare. In a series of 1,000 consecutive cases of cranial nerve palsies, Rucker (5) demonstrated that the combination of third and sixth nerve involvement was present in only 7.6%. Of these, only three cases were ascribed to an ischemic cause. In a follow-up study utilizing computed tomography, Rush and Younge (6) found no significant change in the frequency of multiple cranial nerve palsies secondary to isch- I Clill Neuro-ophthalmol, Vol. 6, No.2. 1986 98 G. S. KOSMORSKY AND R. L. TOMSAK FIG. 2. July 9, 1985. Note restora-tion of normal ocular motility 00. emia in comparison with Rucker's study. They further showed that 71% of the patients with vascular disorders (i.e., diabetes mellitus, atherosclerosis, or hypertension) recovered fully regardless of the cranial nerve involved. Sergott et al. (7) have recommended that a diabetic patient with multiple simultaneous cranial nerve involvement on one or both sides should be evaluated for a cause other than ischemia. The vascular anatomy of the cavernous sinus has been well described by Parkinson (8). He demonstrated that in 80% of autopsy specimens the third, fourth, and sixth nerves and the gasserian ganglion are fed by an artery called the "artery of the inferior cavernous sinus," which originates from the inferior lateral aspect of the internal carotid. A single lesion in this artery could, therefore, cause a large amount of ischemic damage to the nerves of the cavernous sinus simultaneously. This, however, is not seen clinically. That simultaneous ischemic cranial nerve deficits do not commonly occur indicates that either (a) this artery is not very susceptible to atherosclerotic or arteriosclerotic changes, (b) more distal lesions of the terminal branches are responsible for the cranial nerve palsies with or without more proximal stenosis of the artery of the inferior cavernous sinus, or (c) collateral flow exists. Dreyfus et al. (9) showed diffuse vascular changes in the cavernous sinus of a diabetic patient who had only a third nerve palsy. These changes occurred in the vasculature ultimately supplying the frontal, trochlear, abducens, and short ciliary nerves as well as the 1 nill Nt·uro-ophl/ul/mol. Vol. 6. No.2. 1986 ciliary ganglion. These changes consisted of increased collagen, perivascular lymphocytes, and thickening, hyalinization, and proliferation of endothelial cells in the intima. Although the lumina of these vessels were greatly reduced, no occlusion was observed. Perhaps slow flow through the artery of the inferior cavernous sinus secondary to these same vascular changes, in addition to more distal narrowing, could produce selective cranial nerve ischemia without total occlusion of the artery. The possibility that our patient had the TolosaHunt syndrome was seriously considered. In a review of the Tolosa-Hunt syndrome, Kline (10) stated that patients with the Tolosa-Hunt syndrome have (a) eye pain that is "boring" or annoying in character, (b) cranial nerve involvement of any cavernous nerve, (c) symptoms lasting days to weeks, (d) spontaneous remissions, (e) attacks recurring at intervals of months to years, (f) no involvement of structures outside of the cavernous sinus. Our patient's eye pain was initially in character with the Tolosa-Hunt syndrome but was short-lived and did not recur. Her symptoms lasted for 12 weeks, which is consistent with either the Tolosa-Hunt syndrome or diabetes mellitus. Our patient has not experienced a recurrence in the ensuing 6 months. Against the diagnosis of the Tolosa-Hunt syndrome was the fact that the patient had a long history of diabetes mellitus, which predisposed her to vascular changes and subsequent cranial nerve ischemia. Additionally, typical intracavernous narrowing of the carotid ar- ISCHEMIC CAVERNOUS SINUS SYNDROME 99 tery was not seen in our patient, who simply had minimal supraclinoid narrowing consistent with atherosclerotic disease. That our patient had a pupillary- sparing third nerve paresis, which is characteristic of ischemic paresis of this nerve (9) was also helpful. Patients with the Tolosa - Hunt syndrome demonstrate involvement of the pupil about 22% of the time (10). Other causes of the cavernous sinus syndrome, i.e., metastatic disease, temporal arteritis, aneurysm, carotid cavernous fistula, myasthenia gravis, and infection Wt're excluded by appropriate testing and follow-up. REFERENCES 1. larson, D. L., and Auchindoss. H. J.: Multiple svmmetric bilateral cranial nerve palsies in patients with unregulated diabetes mellitus. Arch. 1111<'",. Med. 85: 265-271, 1950. 2. Weinstein, E. A., and Do!ger, H.: External ocular muscle palsies occurring in diabetes mellitus. Arch. Neural. PSYc!II/ Iln/60: 597-603,1948. 3. Symonds, C P.: Recurrent multiple cranial nerve palsies. J. Nell",t. Nell"'5I/rs. Psychialry21: 95-100, 1958. 4. Ross. A. T.: Recurrent cranial nerve palsies in diabetes ITIl'lIitus. Nel/l'lllosy 12: ISO-IS5. 1962. 5. Rucker, C W.: Paralysis of the third, fourth, and sixth cranial nl·rVl'S. Alii. J. 01'111111/111101. 46: 787-794, 1958. 6. Rush, J. A., and Youngl', B. R.: Paralysis of cranial nerves III, IV, and VI. Arch. Opl1lhalllwl. 99: 76-79, 1981. 7. Sergott, R. C, GlasL'r, J. S., and Berger, L. J.: SimultaI1t~ OUS bilateral diabl'lic ophthalmoplegia. Ophtiratlllology 91: IIl-22, 19114. Il. Parkinson, D.: Anatomv of thl' cavernous sinus." In Neuroophlhalnwtosy SI/lIIp05il/;1I or Ihe Ullil'CT5ily or Miallli alld BasCOlli PallllCT E,/e IlIslill/le, Vol. 6, J. L. Smith, Ed. C V. Mosby, SI. Ll;uis, 1972, pp. 73-101. 9. Dreyfus, P. M., Hakim, S., and Adams, R. D.: Diabetic ophthalmoplegia. Art'll. Nel/"'/. PSlft'lrialrlf 77: 337-349, 1957. 10. Kline, L. B.: The Tolosa-Hunt syndrome. Surl'. OphtllI/ llllol. 27: 79-95, 1982. I Clill Neuro-oplltl1almol. Vol. 6, No.2, 1986 |
