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Show JOUTTIilI of Clinical Neuro- ophlhalmology 9( 41: 277- 280. 1989 Optic Neuropathy In Beh~ et's Disease Tulay Kansu, M. D., Pinar Kirkali, M. D., Emin Kansu, M. D., and Turgut Zileli, M. D. © 1989 Raven Press, Ltd., New York Optic neuropathy in Beh<; et's disease is rare, despite wide ocular and neurological involvement. Progressive atrophy of the optic disc and severe visual loss is not uncommon in Beh<; et's disease; however, visual loss due to acute optic neuropathy is less well known. We report three cases of optic neuropathy in Beh<; et's disease. The clinical picture was variable in our patients, presenting either as acute retrobulbar optic neuritis or anterior optic neuropathy. It is interesting to note that although the neurological picture resembles multiple sclerosis, there seems to be less predilection to optic nerve involvement in Beh<; et's disease. Key Words: Beh<; et's disease- Optic neuropathy. From the Department of Neurology, Neuro- Ophthalmology Unit ( T. K., P. K., T. Z.) and Department of Internal Medicine ( E. K.), Hacettepe University Hospitals, Ankara, Turkey. Address correspondence and reprint requests to Tulay Kansu, M. D., Hacettepe University HospItals, Department of Neurology, Neuro- Ophthalmology Unit, Ankara, Turkey 06100. 277 Beh<; et's disease is a systemic disorder with ocular, oral, and genital manifestations first described by Hulusi Beh<; et in 1937 ( 1). Since the original description, numerous other manifestations and a wide systemic spectrum of the syndrome have been reported. Despite the wide range of ocular involvement in Beh<; et's disease, isolated optic nerve disease associated with neither intraocular inflammatory nor extensive disease of the central nervous system is rare. We report here data from three cases of optic neuropathy associated with Beh<; et's disease. Our aim is to emphasize this rare condition and to discuss possible mechanisms of optic nerve involvement. CASE REPORTS Patients were drawn from the records of the Beh<; et's disease clinic of the Hacettepe University Hospitals. Two hundred patients had registered over a period of 8 years, and 65 of them had neurological manifestations. Optic neuropathy was found in only three patients. The diagnosis of Beh<; et's disease was made when two of three major criteria were present ( oral and/ or genital ulcers and eye involvement) and minor features were apparent ( skin, joint, vascular, and nervous system manifestations). Patients with optic atrophy who had active inflammation or papilledema prior to the detection of optic neuropathy were not included. Case 1 A 32- year- old man presented in November, 1982, complaining of acute loss of vision in his left eye. He had a history of recurrent oral and genital ulcers, arthralgia, and thrombophlebitis during the past 4 years; no diagnosis had been made. On examination, visual acuity ( VA) was 20/ 20 aD and only light perception as. There was an 278 T. KANSU ET AL. afferent pupillary defect in the left eye. The vitreous was clear in slit lamp examination. Ophthalmoscopic examination revealed normal findings in the right eye. In the left eye the optic disc was pale, the macula was edematous, surrounded with small exudates, and sheathing was observed in retinal veins. Three months later, he developed visual blurring in the right eye. VA was 20/ 100 00 and light perception OS. A central scotoma in the right eye was detected with confrontation and Amsler Grid cards. Ophthalmoscopic examination revealed optic disc edema with peripapillary exudates and hemorrhages 00 and optic atrophy OS. Serum VORL was negative. Lumbar puncture ( LP) pressure was normal, and protein was elevated ( 55 mg%) in cerebrospinal fluid ( CSF). No cells were detected under microscopic examination. A diagnosis of Beh<; et's disease was made on the basis of clinical findings; the treatment with prednisolone 60 mg daily and cyclophosphamide 50 mg twice daily was started. One month later the right disc was pale, and VA was 20/ 50 in the right eye. No follow- up examination was available until 4 years later when he presented with abdominal pain, which was thought to be related to a peptic ulcer. He had no further visual complications but had recurrent aphthous lesions in the mouth. VA was 20/ 30 00 and hand motion OS. Comments This patient had findings of a pale disc and retinitis OS and papillitis 00 3 months apart without active inflammation in the eye. His past history and elevated protein in the CSF was compatible with Beh<; et's disease. Case 2 A 25- year- old man was first seen in September, 1980, with right peripheral facial palsy, bilateral abducens nerve paresis, and swollen optic discs. VA was 20/ 40 00 and 20170 OS. There was a paracentral scotoma in the right eye and a small central scotoma on the left on perimetric examination. Computerized tomography of the brain was normal. LP pressure was 170 mm H20, and there were only 2 mononuclear cells. Serum VORL was negative. He recovered completely 1 month later. He was thought to have multiple sclerosis ( MS) with bilateral optic neuritis. In April, 1981, he was admitted again with blurred vision, gait disturbance, and urinary hesitancy. Physical examination disclosed oral aphthae I Clin Neuro- ophtha/ mol. Vol. 9. No. 3. 1989 and a scar of scrotal ulceration. He was ataxic. There was a right sixth nerve palsy, and deep tendon reflexes were increased. Bilateral plantar reflexes were extensor. He was clumsy in cerebellar tests. VA was 20170 00 and 20170 OS. Visual field examination was similar to the previous fields. In the color vision test he was able to read only 5 of 12 Ishihara plates OU. Optic discs were pale bilaterally. There were no signs of ocular inflammation. Beh<; et's disease was considered as a diagnosis, and corticosteroid treatment was given. Visual symptoms remained unchanged, but cerebellar findings improved slightly. In December, 1981, he was seen again because of a generalized convulsion. He was stable until 1983, when he developed blurred vision in the left eye. On examination he had aphthous lesions in the mouth and papular lesions on the scrotum. VA was 20/ 40 00 and 20/ 200 OS. Slit lamp examination revealed cells in the vitreous, posterior synechia, and uveal pigmentation in the lens of the OS. Optic discs were both pale, suggesting atrophy. Phenytoin and cyclophosphamide were added to the treatment, along with the topical treatment for uveitis. His vision returned to a 20170 level after the inflammation subsided. Comments This patient had optic disc edema with decreased vision and normal LP pressure. He was thought to have bilateral optic neuritis with simultaneous onset, resulting in atrophy. He developed uveitis 3 years later, completing the three major criteria for the diagnosis of Beh<; et's disease. Case 3 A 22- year- old woman was first seen in April, 1977, with acute visual loss in the right eye. VA was 20170 00 and 20/ 20 OS. There was a central scotoma in the right eye. Ophthalmoscopic examination was normal. Retrobulbar neuritis was considered, and she was given oral corticosteroids. One month later her vision recovered completely. She was seen again 2V2 months later because of paresthesias in her feet, but her neurological examination was normal. In March, 1978, she was seen again because of visual loss in the left eye. The diagnosis of retrobulbar neuritis was made by the ophthalmology department, but details of her eye examination were not available. On physical examination, she had oral aphthous lesions and genital ulcerations in the labia major. She was thought to have Beh<; et's disease. Prednisolone OPTIC NEUROPATHY IN BEHCET'S DISEASE 279 and cyclophosphamide were started, and 1 month later vision returned to normal levels. Her last examination was 1 year later, and she had no visual complaints. Comments A 22- year- old woman with two major findings of Beh.; et's disease had retrobulbar neuritis involving both eyes 1 year apart and recovered completely. DISCUSSION Beh.; et's disease is characterized by three primary components: iridocyclitis ( historically with hypopyon), aphthous lesions in the mouth, and ulceration of the genitalia. Erythema nodosum, arthropathy, and thrombophlebitis often accompany these manifestations. The etiology and pathogenesis of Beh~ et's disease is not well understood, although immunologic mechanisms have been suspected to be pathologically important because of its vasculitic nature and the cellular characteristics ( 2). Involvement of the central nervous system occurs in approximately 10% of patients, and the frequency of ocular manifestations in Beh.; et's disease is between 70 and 85% ( 2). The underlying disease in all organ systems is an occlusive vasculitis, and the most common ocular finding is anterior uveitis ( iridocyclitis). Posterior segment manifestations include localized retinal edema, macular edema, perivascular sheathing, and occlusion of retinal vessels. The presence of necrotizing retinal vascular lesions is well known and is often obscured by the severity of the anterior reaction. Optic nerve damage can follow the extension of an inflammation from the uvea to the mesenchymal tissue surrounding the nerve fibers ( uveopapillitis). Papilledema in the absence of uveitis is also a wellrecognized feature of the disease as a manifestation of benign intracranial hypertension ( 3). In our experience such cases are seen more often than are cited in the literature. Of our 65 patients with Beh.; et's disease and neurological manifestations, 20 had benign intracranial hypertension ( unpublished data). However, optic nerve disease without intraocular inflammation or papilledema is rare. There is only one well- documented report of optic neuropathy in Beh.; et's disease ( 4). Scouras and Koutroumanos ( 4) described typical anterior ischemic optic neuropathy in two patients with Beh~ et's disease, presumably on the basis of posterior ciliary arteritis. Cotticelli et al. reported an unusual case of Beh.; et's disease with bilateral obliterating retinal panarteritis and ischemic optic atrophy ( 5). Colvard et al. ( 6) reviewed the ocular manifestations of Beh.; et's disease in 32 patients. Inflammatory involvement of the optic nerve in the form of papillitis is reported in three patients; however, no details of the optic nerve process were given. In another review, Atmaca ( 7) reported edema in 51 and atrophy in 17 eyes of a total 148 eyes of 83 patients with Beh.; et's disease, but the clinical details were scanty, and the possibility of optic atrophy due to severe retinal vascular disease was not addressed. Histopathological studies of the optic nerve and retina based on three cases have shown that changes in the optic nerve developed secondary to principal changes in the retina in two cases ( 8). Findings in the third case showed that such changes may start primarily in the optic nerve, manifested by fibrous astrocyte substitution for the axonal portion of the optic nerve. The capillary network was still intact despite extensive narrowing of the central retinal artery ( 8). Syphilis was considered in the differential diagnosis of our patients. Serum VORL was negative in patients 1 and 2 and not done in patient 3. The incidence of syphilis is very low in Turkey, and the fluorescent treponema antibody absorption ( FTAABS) test is not available. Treponema pallidum immobilization ( TPI) is done if the suspicion is high. Our clinical impression was that these patients did not have syphilis, although we do not have definite serological proof. The similarity of the clinical picture in multiple sclerosis and Beh.; et's disease is well known. Clinical comparative studies for these two diseases showed a very low incidence of optic nerve involvement in Beh.; et's disease, in spite of a high incidence of visual impairment due to optic neuritis in multiple sclerosis ( 9). Our cases as well as those in the literature suggest that optic neuropathy in Beh.; et's disease can be caused by ( a) ischemic process ( 4), ( b) demyelinization ( 2), ( c) inflammation ( 6), or ( d) secondary invasion of the optic nerve from the processes that have involved the uvea and retina ( 8). We believe that the pathogenesis is occlusion of the small vessels of the optic nerves and demyelinization on the basis of mild ischemia. Axonal damage and necrosis occur in more severe cases. In conclusion, we have reported data from three cases of optic neuropathy in Beh.; et's disease. The optic nerve appears to be less involved. While not common, optic neuropathy in Beh~ et's disease can 1Clin Neuro- ophthalmol. Vol. 9, No. 4, 1989 280 T. KANSU ET AL. present as retrobulbar neuritis or anterior optic neuropathy. REFERENCES 1. Behc; et H. Ueber rezidivierende aphthouse durch ein virus verursachte geschwuere am mund, am auge und an der genitalien. Dermato Monatsschr Wochenschr 1937; 105: 1152- 7. 2. Michelson JB, Chisari FV. Behc; et's disease [ Review). Surv Ophthalmol 1982; 26: 190- 203. 3. Parnir N, Kansu T, Zileli T, Erbengi A. Papilledema in Beheet's syndrome. Arch Neurol 1981; 38: 643- 5. 4. 5couras 1. Koutroumanos J. Ischemic optic neuropathy in Behc; et's syndrome. Ophthalmologica 1976; 173: 11-- 8. 5. Cotticelli L, Apponi- Battini G, Federico A, Cotrufo R, An- I Clin Neuro- ophthalmol, Vol. 9, No. 3, 1989 nunziata P, Dilorio G. Behc; et's disease: an unusual case with bilateral obliterating retinal panarteritis and ischemic optic atrophy. Ophthalmologica 1980; 180: 328- 32. 6. Colvard M, Robertson OM, O'Duffy JD. The ocular manifestations of Behc; et's disease. Arch Ophthalmol 1977; 95: 1813- 7. 7. Atrnaca LS. Fundus changes in Behc; et's disease. In: Saylan T, Ovul C, eds. Second meeting on Beh~ et's disease. Istanbul: University of Istanbul, 1984: 14- 20. 8. Uga 5, Ishikawa 5, Wakakura M. Histopathology of the optic nerve and retina in Behc; et's disease. In: Dilsen N, Konice M, Ovul C, eds. Proceedings of an international symposium on Behfet's Disease. Amsterdam: Exerpta Medica, 1977: 85- 7. 9. Motomura 5, Tabira T, Kuroiwa Y. A clinical comparative study of multiple sclerosis and neuro- Behc; et syndrome. 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