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Immunosuppressive Pulsed Treatment for Behçet’s Disease with Reversibly Extinct VEP and Reversibly Lacking Perception of Light

1Bialasiewicz A., 1Breidenbach K., 2Hassan M. O., 2Shenoy R.,
1Dept. of Ophthalmology and School of Ophthalmic Technicians, Sultan Qaboos University Hospital, University of Oman (Muscat)
2Dept. of Clinical Physiology, Sultan Qaboos University Hospital, University of Oman (Muscat)

Purpose: The reversal of an extinct VEP and confirmed lack of light perception has been documented in exceptionally rare cases.
Case report: A 62 year-old lady with a history of retinal vasculitis for 7 years had had a vitrectomy (04/2001) to remove a vitreous infiltrate abroad. Histology excluded NHL, and no further work-up was done. After 3 months she presented with a loss of vision OD: FC, OS: 0.6, IOP 12/12 mmHg. A complicated cataract OD, and a pale optic disc and retinal vasculitis OS were noted.
Results: A B-scan showed a vitreous infiltrate OU, the fluorescein angiography (OS) peripapillar and vessel leakage, focal central leakage spots, and non-perfusion areas. The VEP could not be elicited OD, and latency was increased and the amplitude reduced OS. Phaco and pcIOL implantation, ppv and endolaser treatment was performed (27.07.2001), and an occlusive hemorrhagic retinal vasculitis and optic neuritis were seen. Elevated systemic inflammation parameters, a positive HLA-B5, thrombocyte rosettes, and aphthous buccal lesions were found. The histology of vitreal cells was unremarkable. PostOP visual acuity dropped to OD NLP/EF+, and a VEP could not be elicited. A pulsed methylprednisolone 2g/d (for 3d) and continuing cyclosporin A treatment increased vision to OD: 0.5, OS: 0.6 (09/2001), and OU 1.0 (11/2001). The inflammation (Laser Cellmeter 7-10) had ceased, and no optic atrophy (VEP: normal latencies and amplitudes) could be observed until the last follow-up (03/2002).
Conclusions: It is suggested that all cases of occlusive retinal vasculitis should be evaluated to provide an appropriate therapy and a prevention of blindness. High-dosage immunosuppression may reverse acute blindness in posterior Behçet’s disease.

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