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Show lournill of Clinical Neuro- ophthalmology 9( 1): 27- 32, 1989. Acute Sarcoid Optic Neuropathy with Spontaneous Recovery Steven Galetta, M. D., Norman J. Schatz, M. D., and Joel S. Glaser, M. D. © 1989 Raven Press, Ltd., New York Sarcoid optic neuropathy without fundus lesions is unusual. We present a case of optic neuropathy with spontaneous remission in one eye that paralleled the clinical course of demyelinative acute optic neuritis. The fellow eye had disc swelling with normal visual acuity but with a large blind spot and distended optic nerve sheaths seen on ultrasonography. The diagnosis of sarcoidosis was made by characteristic chest roentgenographic findings and confirmed by skin biopsy. Key Words: Demyelinative disease- Optic Neuropathy- Sarcoidosis. From the Bascom Palmer Eye Institute, Department of Ophthalmology, University of Miami School of Medicine, Miami, Florida, U. S. A. Address correspondence and reprint requests to Dr. J. S. Glaser, Bascom Palmer Eye Institute, P. O. Box 016880, Miami, FL 33101, U. S. A. 27 Sarcoidosis is a multisystem disease of unknown etiology, characterized by the formation of noncaseating granulomas in various organ systems. The acute form of the disorder has a sudden onset and a tendency to remit spontaneously; in comparison, the chronic form of sarcoidosis is usually insidious with a progressive course requiring immunosuppressive therapy ( 1). Central nervous system manifestations include meningeal infiltration, hydrocephalus, granulomatous mass lesions, and cranial neuropathies ( 2). Other than the facial nerve, the optic nerve is the second most commonly affected cranial nerve ( 3). Although it has been estimated that optic nerve involvement occurs in 5% of cases ( 4), the rarity of retrobulbar optic neuropathy complicating this disorder has been emphasized ( 5). We report here a case of sarcoid optic neuropathy simulating demyelinative retrobulbar optic neuritis in one eye and disc edema with normal function in the other. CASE REPORT A 31- year- old woman was well until Feb. 1, 1988, when she noted the onset of a painfully swollen left leg. Several days later, a painless and precipitous drop in the vision of the right eye occurred such that she could no longer discern a television set. On Feb. 16, 1988, her condition was medically evaluated for a presumptive diagnosis of thrombophlebitis of the left leg. Vision in the right eye was measured at hand motions only. The remainder of the ophthalmologic examination results were reportedly normal. Studies included an unsuccessful leg venogram and a computed tomography ( CT) scan of the head that was interpreted as normal. A chest roentgenogram was normal at that time. The patient was treated with i. v. heparin so- 28 S. GALETTA ET AL. dium for 5 days, and then therapy was switched to aspirin. During hospitalization, a nontender papular rash developed over the lower extremities and became progressively more prominent over the next several weeks. Following discharge, some improvement in the vision of her right eye was noted. When evaluated ophthalmologically on Feb. 29, 1988, her acuity was 3/ 200 00 and 20/ 20 as. There was a right afferent pupillary defect, and visual fields showed a central scotoma in that eye. The optic nerve head was normal on the right and swollen on the left. Evaluation at the Bascom Palmer Eye Institute on March 16, 1988, revealed a best corrected acuity of 20/ 60 + 2 00 and 20/ 15 - 1 as. There was a right afferent pupillary defect. Three of ten Ishihara color plates were identified with the right eye, and 10 of 10 were identified with the left. Visual fields showed a central scotoma and a superior arcuate defect in the right eye. In the left visual field, there was an enlarged blind spot. The slit- lamp examination was normal on the right, and 2+ vitreous cells were present on the left. The right optic nerve was normal except for a minimally blurred nasal margin, while the left optic nerve was moderately swollen ( Fig. 1). No other retinal or choroidal lesions were detected by extensive indirect ophthalmoscopy. Results of a complete neurologic examination were otherwise normal, but there was a prominent red papular rash over both lower extremities. Laboratory evaluation revealed a white blood cell count of 6,800; hemoglobin level, 11.6 g; hematocrit reading, 36.1 %; Ca level, 9.1 mg/ dl; serum lysozyme level, 16 J.. lg/ ml ( normal, 2.&- 8.0 J.. lg/ ml); angiotensin converting enzyme level, 60 lUlL ( normal 12- 36 lUlL); and erythrocyte sedimentation rate, 25 nun/ h. Antinuclear antibody, rheumatoid factor, RPR, and fluorescent treponemol antibody absorption test results were all negative. A purified protein derivative test result was negative, and anergy to mumps was present. Retinal fluorescein angiography in the late arteriovenous phase showed leaking capillaries on the surface of the left disc with diffuse leakage, and the right disc showed a small amount of leakage inferiorly ( Fig. 2). A chest roentgenogram revealed bilateral hilar adenopathy ( Fig. 3), but pulmonary function test results were normal. A gallium scan showed increased uptake in the parotid glands, lacrimal glands, and pulmonary hilum ( Fig. 4). Magnetic resonance imaging of the brain and orbits was normal. Standardized orbital echography ( 6) showed increased subarachnoid fluid in the optic nerve sheaths, with a bilaterally positive 30° test. A lumbar puncture revealed an opening pressure of 120 mm of cerebrospinal fluid ( CSF), no cells, a glucose level of 80 mg/ dl, and a protein level of 11 mg/ dl; oligoclonal bands were absent. Microbiological and cytologic examination results were also negative. A skin biopsy specimen of the papular lesions revealed noncaseating granulomas consistent with sarcoidosis ( Fig. 5). During this admission, the right visual acuity spontaneously improved to 20/ 20. Examination 6 weeks after a short course of corticosteroid therapy revealed no change except for a significant improvement of the optic disc swelling on the left. A repeat echogram showed resolution of the fluid previously seen in both optic nerve sheaths. 1( 1111 ' 1, ..... FIG. 1. The right disc ( left) shows a slightly blurred nasal margin. The left disc ( right) is edematous. with probable axonal debris inferiorly; note also the subretinal fluid temporally. t. · j ~ .. j ,\'" 7. 19x9 SARCOID OPTIC NEUROPATHY WITH SPONTANEOUS RECOVERY 29 FIG. 2. Fluorescein angiography of the right disc ( left) shows minimal dye leakage in the late arteriovenous phase. There is diffuse staining of the left disc ( right) in late phases. DISCUSSION The optic nerve may be involved by sarcoidosis in five major forms: granulomas of the nerve head; papillitis; retrobulbar neuritis; papilledema with raised intracranial pressure; and optic atrophy ( 7). Granulomas of the optic disc are the most frequently recognized form of sarcoid optic neuropathy ( 8). Sarcoid granulomas of the optic nerve head may occur as isolated fundus findings or be accompanied by the often characteristic chorioretinitis and periphlebitis retinae (" candle wax drip- FIG. 3. A chest roentgenogram shows prominent bihilar adenopathy. J Clill Neuro- ophthalmol, Vol. 9, No. L 1989 30 S. GALETTA ET AL. FIG. 4. A gallium scan of the skull demonstrates increased uptake in both lacrimal glands ( small arrows) and both parotid glands ( large arrows). pings"). Visual acuity in these cases ranges from 20/ 20 to no light perception ( 8). In 1972, Laties and Scheie ( 9) documented the spontaneous regression of an optic disc granuloma in a patient with normal acuity. They reviewed 10 previously reported cases of granulomatous infiltration of the optic disc. It was apparent that the size and location of the masses were critical factors in terms of visual outcome. Those granulomas that did not compromise the major retinal vessels were associated with a better outcome. Although sarcoid granulomas of the optic nerve head are usually unilateral, the may be bilateral ( 10). In 1976, FIG. 5. A skin biopsy specimen shows noncaseating necrosis with a large, multinucleated giant cell ( arrows). \, 1. J, 1~ 0~ Gass and Olson ( 11) reported consecutive involvement of the optic disc by sarcoid granulomas. Sarcoid optic neuropathy may also appear as a papillitis ( 8,12,13). Optic disc swelling rna? be a~ sociated with posterior uveitis, retinal penvascuhtis, or infiltration of the optic nerve by minute granulomas. Optociliary venous shunts have been rarely observed in granulomatous infiltration of the optic nerve by sarcoid ( 12,14,15). Mansour ( 15) has reported an excellent visual outcome in one patient with papillitis despite the presence of shunt vessels. Rush ( 5) pointed out the scarcity of retrobulbar neuropathy in sarcoidosis and presented a case that had a dramatic recovery with corticosteroid therapy. Beardsley et al. ( 8) noted significant visual improvement in three patients with retrobulbar optic neuropathy treated with systemic corticosteroids. One of these patients had an episode of visual loss that occurred over several months and spontaneously improved over months. Our patient had an acute onset of visual loss with complete and spontaneous recovery over 3 weeks. Orbital echography revealed the subarachnoid fluid that is typically seen with in£ lammatory optic neuritis ( 6). In the absence of a demonstrable discrete mass lesion, visual loss was likely the result of infiltration of the optic nerve septae by minute sarcoid granulomas ( Fig. 6). The clinical course was consistent with demyelinating optic neuritis. Left optic disc swelling was present with vitreous cells but was accompanied by normal visual function. A retrolaminar sarcoid granuloma seems likely. Occasionally, retrobulbar granulomas may mimic the clinical and neuroradiologic appearance of optic nerve meningiomas. Gudeman et al. ( 16) described three patients with progressive unilateral optic neuropathy and sarcoidosis. In two patients, the clinical and neuroradiologic data suggested an optic nerve meningioma; both patients showed contrast enhancing masses, with hyperostosis of the anterior clinoid process in one and thickening of the planum sphenoidale in the other. Biopsy specimens of the mass lesions in both cases revealed noncaseating granulomas. Sarcoid, with its propensity to involve the hypothalamus and pituitary region, frequently infiltrates the chiasm ( 7). As such, it represents one of the leading nontumoral causes of chiasmal visual loss. Tang et al. ( 17) described four patients with sarcoidosis who had visual field defects characteristic of a chiasmal lesion. All four showed profound visual loss in one eye with a temporal field defect in the contralateral eye that suggested an SARCOID OPTIC NEUROPATHY WITH SPONTANEOUS RECOVERY 31 FIG. 6. A pathologic specimen from a similar case demonstrating infiltration of the optic nerve septae by sarcoid granulomas. anterior chiasmal junction syndrome. In two patients, no discrete mass lesions were discovered on neuroradiologic imaging and even by craniotomy in one, implying diffuse infiltration of the chiasm by sarcoid. In the remaining two patients, pneumoencephalography suggested a suprasellar mass lesion, and at craniotomy, sarcoid granulomas involving the pituitary and hypothalamus were found. Decker et al. ( 18) described a patient who had severe progressive bilateral visual loss over a 2- week period. A CT scan revealed a suprasellar enhancing mass, and transsphenoidal resection of a large sarcoid granuloma was performed. Chiasmal sarcoidosis may be infiltrative or compressive. Cases of papilledema from raised intracranial pressure may result from a basal meningitis that blocks CSF resorption or from a granulomatous mass lesion ( 10). Sarcoid- induced optic atrophy may be the product of an optic nerve lesion or secondary to increased intracranial pressure ( 7,8). Graham et al. ( 19) have described five patients with sarcoid optic neuropathy. They divided patients into two groups: those with chronic cases with poor response to steroid therapy, and those with acute or subacute cases with a good response to steroid therapy. They pointed out that the three patients in the latter group became steroid dependent, and in none of their cases did the clinical profile resemble the optic neuritis associated with multiple sclerosis. This report serves to emphasize that a predominantly retrobulbar optic neuropathy similar to that seen in demyelinating optic neuritis may be observed in sarcoidosis. As with the optic nerve head granulomas previously reported, this form of optic neuropathy may also spontaneously remit. Thus, retrobulbar optic nerve involvement in sarcoidosis may simulate optic neuritis, an optic nerve tumor, or chiasmal compression. Optic disc swelling and vitreous inflammatory cells with normal acuity have been previously described. REFERENCES 1. Kerdel FA, Moschella SL. Sarcoidosis: an updated review. J Am Acad DermatoI1984; 11: 1- 19. 2. Stern BJ, Krumholt A, Johns C, et al. Sarcoidosis and its neurologic manifestations. Arch NeuroI1985; 42( 9): 909- 17. 3. Colover J. Sarcoidosis with involvement of the nervous system. Brain 1948; 71: 451- 75. 1Clin Neuro- ophthalmol. Vol. 9, No. 1, 1989 32 S. GALETTA ET AI. 4. Blair JG, Riley W, Logothetis J. Optic nerve manifestations of sarcoidosis. Arch Neurol 1965; 13: 307- 9. 5. Rush JA. Retrobulbar optic neuropathy in sarcoidosis. Ann OphthalmoI1980; 12: 390- 4. 6. Gans MS, Byrne SF, Glaser JS. Standardized A- scan echography in optic nerve disease. Arch Ophthalmol 1987; 105: 1232-- 6. 7. Ingestad R, Stigmar G. Sarcoidosis with ocular and hypothalamic- pituitary manifestations. Acta Ophthalmol 1971; 49: 1- 10. 8. Beardsley TL, Brown SU, Sydnor CF. Eleven cases of sarcoidosis of the optic nerve. Am JOphthalmoI1984; 97: 62- 77. 9. Laties AM, Scheie HG. Evolution of multiple small tumors in sarcoid granuloma of the optic disc. Am J Ophthalmol 1972; 74: 60- 7. 10. Jampol LM, Woodfen W, McLean EB. Optic nerve sarcoidosis. Arch OphthalmoI1972; 87: 355- 60. 11. Gass JDM, Olson CL. Sarcoidosis with optic nerve and retinal involvement. Arch Ophthalmol 1976; 94: 945- 50. JClill Neuro- ol'htltallllol. \,,,; 9. N". I. 1989 12. Kirkham TH. Neuro- ophthalmic presentations of sarcoidosis. Proc R Soc Med 1973; 66: 167- 9. 13. Hart WM, Burde RM. Optic disc edema in sarcoidosis. Am JOphthalmol 1979; 88: 769. 14. Krohel GB, Charles H, Smith RS. Granulomatous optic neuropathy. Arch OphthalmoI1981; 99: 1053- 5. 15. Mansour AM. Sarcoid optic disc edema and optociliary shunts. CIin Neuro OphthalmoI1986; 6( 1): 47- 52. 16. Gudeman SR, SelhorstJB, Susac JO, Waybright EA. Sarcoid optic neuropathy. Neurology 1982; 34: 597- 603. 17. Tang RA, Grotta JC, Lee KF, Lee YEo Chiasmal syndrome in sarcoidosis. Arch OphthalmoI1983; 101: 1069- 73. 18. Decker RE, Mardayat M, Marc J, Rasool A. Neurosarcoidosis with computerized tomographic visualization and trans- sphenoidal excision of a supra and intrasellar granuloma. JNeurosurg 1979; 50: 814- 6. 19. Graham EM, Ellis CJK, Sander MD, McDonald WI. Optic neuropathy in sarcoidosis. J Neurol Neurosurg Psychiatry 1986; 49: 756-- 63. |
