title: [Blind spot enlargement syndrome in acute zonal occult outer retinopathy with detection of autoantibodies against the retinal antigens CRALBP and S-Ag].
abstract: Acute zonal occult outer retinopathy (AZOOR) is a rare disease and is part of the white dot syndrome occurring bilaterally and often asymmetrically in young healthy myopic women. Characteristic findings are distinct focal lesions of the outer segments (OS) of the photoreceptor (PR) layer and abnormalities in fundus autofluorescence (FAF) within the lesions. Currently there is a lack of defined disease criteria, such as specific laboratory findings. Also no effective therapy is known which makes it difficult to diagnose, differentiate and treat AZOOR. Supplementation of antioxidants may become part of therapeutic options in AZOOR. A 19-year-old myopic woman presented with unilaterally reduced visual acuity. Due to the clinical features and with the help of FAF, spectral domain optical coherence tomography (SD-OCT) and perimetry the diagnosis of blind spot enlargement syndrome in AZOOR was made. Identification of autoantibodies specific for two retinal antigens (CRALBP and S-Ag) supports the concept of an autoimmunological origin of the disease. Systemic steroids were given but stopped almost 6 weeks later as no improvement was seen. In follow-up controls over 12 months the clinical picture remained unchanged without any further therapy.
authors: Klinik und Poliklinik für Augenheilkunde, Justus-Liebig-Universität Gießen, UKGM GmbH, Standort Gießen, Gießen, Deutschland. ramona.neutzner@augen.med.uni-giessen.de
authors: R V
authors: RV
authors: Neutzner
authors: M
authors: M
authors: Jäger
authors: C
authors: C
authors: Friedburg
authors: C A
authors: CA
authors: Deeg
authors: B
authors: B
authors: Lorenz
mesh_terms: D000328:Adult; D019393:Arrestin / Q000276:immunology*; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000276:immunology*; D002352:Carrier Proteins / Q000276:immunology*; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D009898:Optic Disk / Q000276:immunology / Q000473:pathology; D012164:Retinal Diseases / Q000175:diagnosis* / Q000276:immunology*; D014794:Visual Fields
chemical_list: C020932:11-cis-retinal-binding protein; D019393:Arrestin; D002352:Carrier Proteins