Here, we a report a case of presumed AIR and the diagnostic challenges this condition poses. Case Report A 44-year-old female patient presented to our institute with complaints of loss of vision in both eyes since 4 years. are clinically similar to paraneoplastic AIRs but occur in the absence of underlying malignancy. Many np-AIR patients may also have cystoid macular edema. AIRs are commonly seen in the fourth to fifth decades and have a slight female preponderance. Commonly seen clinical manifestations include acute or subacute loss of vision in one or both eyes, along with complaints of night blindness, photopsias, abnormal color vision, and visual field defects. This is accompanied by a normal looking fundus or attenuated retinal vasculature, retinal pigment epithelium (RPE) atrophy or mottling, Rabbit polyclonal to AKR1A1 few or no inflammatory cells, and waxy disc pallor. Constricted visual fields, central or paracentral scotomas are usually seen on visual field testing. Electroretinogram (ERG) shows diminished rod and cone responses. Moreover, they demonstrate the presence of circulating anti-retinal antibodies. There is usually a history of autoimmune disease in the family, but no history of retinitis pigmentosa (RP). No established guidelines exist for the treatment of this group of disorders. Here, we a report a case of presumed AIR and the diagnostic challenges this condition poses. Case Report A 44-year-old female patient presented to our institute with complaints of loss of vision in both eyes since 4 years. It was subacute in onset, worsened gradually over a period of 1 1 1 year and then stabilized. The patient had a history of bronchial asthma and hypothyroidism and was on treatment for the same. The systemic examination was within normal limits. She had been evaluated elsewhere before presenting to our institute. Old reports revealed a central scotoma in both her eyes, optical coherence tomography (OCT) showed foveal thinning, with foveal thickness being 107 in the right eye Desmopressin Acetate and 88 microns in the left eye. Visually evoked potential test and computed tomography scan brain-orbits were normal. Her visual acuity at the time of presentation was right eye 6/24, N10 and left eye 6/36, N12; not improving with glasses or pinhole. Rest of the anterior segment evaluation was within normal limits. Fundus showed attenuation of vessels and an altered foveal reflex along with small macular pigment epithelial detachment in both the eyes [Figure 1]. Color vision Desmopressin Acetate as checked by the Ishihara chart was 12/21 and 13/21 in the right and left eyes, respectively. At this stage, no intervention was made, and she was advised regular follow-up. A year after the initial presentation, she was seen in the uvea clinic for complaints of floaters and diminished vision in both eyes with persisting headaches. Visual acuity had dropped to 6/60, N12 in both eyes. Examination revealed the presence of few cells in the anterior vitreous and internal limiting membrane (ILM) folds in both eyes. An epiretinal membrane (ERM) also was noted in the right eye [Figure 2]. Ultrasound-B scan showed increased peripapillary choroidal thickness (1.4 and 1.5 Desmopressin Acetate Desmopressin Acetate mm in the right and left eyes respectively). Fundus fluorescein angiography was done and showed few retinal pigment epethelial window defects and late staining of the optic disc in both eyes [Figure 2]. OCT showed foveal thinning in both eyes (foveal thickness 24 in right eye, and 40 in the left eye) with RPE atrophy and few cystic spaces, ERM, and ILM striae [Figure 3]. Noting the progressive foveal thinning seen on OCT scans and visual acuity not corresponding to the visible fundus changes, visual field analysis was repeated and showed centrocecal defects in both the eyes. A multifocal ERG was also done and it showed nonrecordable foveal ring responses, reduced parfaoveal ring responses, and minimally reduced perifoveal ring responses in both eyes [Figure 4]. At this stage, a probable diagnosis of AIR was made. An internist was consulted, and any underlying occult malignancy was ruled out. She was advised a trial of a short course of oral steroids for 4 weeks, 1 mg/kg/day, in tapering doses. Open in a separate window Figure 1 Fundus photograph of both.