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Acute zonal occult outer retinopathy: towards a set of diagnostic criteria

¹ Institute of Ophthalmology, UCL, 11-43 Bath Street, London, UK
² Moorfields Eye Hospital, 162 City Road, London, UK

Correspondence to:
Correspondence to:
Graham E Holder PhD
Department of Electrophysiology, Moorfields Eye Hospital, 162 City Road, London EC1V 2PD, UK; Graham.Holder{at}moorfields.nhs.uk

Background: Individuals with acute zonal occult outer retinopathy (AZOOR) present with initially progressive scotomata and photopsia. Characteristically, the extent of the visual field defect is unexplained by fundal examination, but there is marked retinal dysfunction evident electrophysiologically. It is the authors’ experience that a group of patients exhibit characteristic clinical and electrophysiological abnormalities, which serve as criteria for a working diagnosis.

Methods: A retrospective observational case series of 28 patients were identified with the clinical diagnosis of AZOOR who shared similar abnormal electrophysiology. Details of the history and ophthalmic findings were obtained from the case notes.

Results: Electrophysiology demonstrated a consistent pattern of dysfunction both at the photoreceptor/retinal pigment epithelial complex but also at inner retinal levels, essentially comprising a delayed 30 Hz flicker ERG and a reduction in the EOG light rise.

Conclusion: This study determines diagnostic criteria applicable to a group of patients with AZOOR, typically those with classic symptomatology. Electrophysiological testing can help avoid lengthy, costly, and potentially invasive investigations, and the unnecessary use of immunosuppressive therapy.

Abbreviations: AIBSE, acute idiopathic blind spot enlargement syndrome; AMN, acute macular neuroretinitis; AZOOR, acute zonal occult outer retinopathy; EOG, electro-oculography; ERG, electroretinogram; MCP, multifocal choroiditis with panuveitis; MEWDS, multiple evanescent white dots syndrome; MIC, multifocal inner choroiditis; PERG, pattern ERG; PIC, punctate inner choroiditis; P-POHS, pseudo-presumed ocular histoplasmosis syndrome

Keywords: AZOOR; electrophysiology; MEWDS; punctate inner choroiditis

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