Department of Neurology (GT, RY, XZ), and Eye Center (NL), Beijing Tongren Hospital, Capital Medical University, Beijing, China.
Supported by the Beijing Healthcare High Level Academic Leaders Project, 2009-2-09.
The authors report no conflicts of interest.
Address correspondence to Xiaojun Zhang, MD, PhD, Department of Neurology, Beijing Tongren Hospital, No. 1 Dong Jiao Min Xiang, Dongcheng, Beijing 100730, China; E-mail: firstname.lastname@example.org
A 40-year-old man presented with painless sudden visual loss due to a central retinal artery occlusion (CRAO) in his right eye. Three months later, he had painless acute visual loss in his left eye with visual acuity of 20/100 and a swollen optic disc. After oral prednisone treatment, the disc swelling resolved and acuity recovered to 20/20. Five months later, the patient experienced another episode of vision loss in the left eye associated with optic disc edema. With steroid therapy, he regained 20/20 acuity once again. With a history of recurrent oral ulcers, fluorescein angiography showing obliterative retinal vasculitis in the right eye, and steroid responsive optic neuropathy in the left eye, we made the diagnosis of incomplete Behçet disease.
A 40-year-old man presented with sudden painless visual loss in his right eye for 10 hours. On examination, visual acuity was no light perception in the right eye and 20/20 in the left eye. The right pupil was amaurotic. Funduscopic examination of the right eye showed cloudy swelling of the nerve fiber layer throughout the posterior pole, a cherry-red spot in the macula, attenuated retinal arteries, and a swollen optic disc (Fig. 1). The left eye was normal. Fluorescein angiography of the right eye showed markedly delayed filling of the retinal arteries and optic disc vasculature with normal filling on the left eye (Fig. 2). The patient denied a history of transient monocular visual loss, diabetes, or hypertension.
An echocardiogram was normal while duplex carotid artery ultrasound showed atherosclerotic plaque formation without significant stenosis. Except for hypercholesterolemia, all blood tests, including antiphospholipid antibody, were normal. The patient was treated with aspirin and atorvastatin and regained light perception in the right eye.
Three months later, he awoke with painless blurred vision in his left eye. Visual acuity was now light perception in the right eye and 20/200 in the left eye. Anterior segment examination was normal. The right optic disc was pale with sheathing of the retinal vessels, and the left optic disc was swollen (Fig. 3). No other neurologic findings were present.
Laboratory data included a white blood cell count of 17.6 × 109/L (neutrophils 54.6%, lymphocytes 37.3%, and monocytes 6.2%), erythrocyte sedimentation rate of 3 mm/hour, C-reactive protein of 1 mg/L (normal, 0-8 mg/L), and rheumatoid factor of 59.7 IU/mL (normal, 0-20 IU/mL). Antistreptolysin O test, antinuclear antibody, rapid plasma reagin, HIV, hepatitis B virus, and hepatitis C virus antibodies were negative. Chest X-ray, cerebrospinal fluid examination, and brain and orbital MRI were normal.
The patient was treated with oral prednisolone 1 mg/kg/day for 7 days, followed by a tapering dose for another week. Visual acuity recovered to 20/40 in the left eye within 2 weeks and the optic disc swelling resolved. The patient quit smoking and continued on aspirin therapy.
Five months later, he reported blurred vision once again in his left eye and was found to have visual acuity of 20/50 with a swollen optic disc (Fig. 4). The patient now divulged a history of recurrent oral ulcers (Fig. 5). Hematologic studies for HLA-B51, angiotensin-converting enzyme, antiphospholipid antibody, c-antineutrophilic cytoplasmic antibody (ANCA), and p-ANCA were negative. The patient was diagnosed with incomplete Behçet disease (BD) and treated with intravenous methylprednisolone (1 g/day) for 3 days, followed by oral prednisone (1 mg/kg/day). Cyclosporin was also administered for long-term immunosuppression. Visual acuity in the left eye recovered to 20/20, with resolution of optic disc edema. During 1 year of follow-up, the patient has remained stable.
BD is a systemic inflammatory disease of unknown etiology. The incidence varies geographically, with one of the highest occurrence rates in China (1). BD can affect both the central nervous system and the eye. Neurologic abnormalities include parenchymal brain lesions, dural sinus thrombosis, arterial vasculitis, and aseptic meningitis (2). In the eye, BD most often manifests as a recurrent nongranulomatous uveitis and as a necrotizing obliterative retinal vasculitis (3).
Reports of central retinal artery occlusion (CRAO) and optic neuropathy in BD are uncommon (4). In a series of 140 BD patients published by Krause et al (5), only 1 case of CRAO and 1 case of papillitis were described. CRAO or inflammatory optic neuropathy due to obliterative vasculitis can also be caused by other systemic connective tissue diseases, such as systemic lupus erythematosus, Sjogren syndrome, and Wegener granulomatosis (6,7).
The diagnosis of BD can be divided into complete disease when 4 major criteria are present and incomplete disease when there are 3 major features, 2 major and 2 minor, or typical recurrent ocular symptom plus 1 major or 2 minor features (Table 1) (8). Our patient experienced a CRAO and recurrent papillitis and had recurrent oral ulcers. He is best classified as incomplete BD.
Treatment for BD is determined by which organ systems are affected and extent and severity. For eye involvement, local and systemic corticosteroids are recommended often in conjunction with other immunosuppressive agents, such as azathioprine, cyclosporine, or methotrexate.
The authors thank Dr. Jonathan Horton (Department of Ophthalmology, University of California, San Francisco) for his help in preparation of the manuscript.
1. Sakane T,
Takeno M, Suzuki N, Inaba G. Behçet's disease. N Engl J Med. 1999;341:1284-1291.
2. Mendes D,
Correia M, Barbedo M, Vaio T, Mota M, Goncalves O, Valente J. Behçet's disease—a contemporary review. J Autoimmun. 2009;32:178-188.
3. Deuter CM,
Kötter I, Wallace GR, Murray PI, Stübiger N, Zierhut M. Behçet's disease: ocular effects and treatment. Prog Retin Eye Res. 2008;27:111-136.
4. Nanke Y,
Kotake S, Goto M, Matsubara M, Ujihara H. A Japanese case of Behçet's disease complicated by recurrent optic neuropathy involving both eyes: a third case in the English literature. Mod Rheumatol. 2009;19:334-337.
5. Krause L,
Köhler AK, Altenburg A, Papoutsis N, Zouboulis CC, Pleyer U, Stroux A, Foerster MH. Ocular involvement in Adamantiades-Behçet's disease in Berlin, Germany. Graefes Arch Clin Exp Ophthalmol. 2009;247:661-666.
6. Smith CH.
Optic neuritis. In: Miller NR, Newman NJ, Biousse V, Kerrison JB, eds. Walsh & Hoyt Clinical Neuro-Ophthalmology, 6th edition. Philadelphia, PA: Lippincott Williams & Wilkins, 2005:293-348.
7. Costello F,
Gilberg S, Karch J, Burns B, Leonard B. Bilateral simultaneous central artery occlusions in Wegener granulomatosis. J Neuroophthalmol. 2005;25:29-32.
8. Suzuki Kurokawa M,
Suzuki N. Behcet's disease. Clin Exp Med. 2004;4:10-20.
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