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Eric Sollenberger, Rebecca S Epstein, Grazyna Adamus, Alessandro Iannaccone; Clinical characteristics of autoimmune neuro-retinopathy (AINR). Invest. Ophthalmol. Vis. Sci. 2014;55(13):2509.
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To illustrate the clinical and functional presentation of a retrospective case series of 57 patients with AINR.
Age, onset modality, symptoms at onset, fundus features, Goldmann visual fields (GVF), flash electroretinogram (ERGs) and pattern-reversal visual evoked potentials (PVEPs) were reviewed. Anti-retinal and anti-optic nerve autoantibody (AAb) testing through western blots and immunohistochemistry (IHC) was performed in a diagnostic setting to confirm the suspicion of AINR.
Average patient age at first exam was 50±16.2 yo (SD) and as early as 4 yo. Patients were 63% female. Mean age of onset was 46.3±16.3 yo (SD). In most cases, onset was acute or subacute. Patients presented with as many as 6 anti-retinal and/or anti-optic nerve AAbs. Symptoms of night blindness (82%), decreased visual acuity (77%), photophobia (68%), and photopsia (49%) were common in AINR. On fundus exam, Papillary and juxtapapillary pigmentation and/or atrophy, disc elevation and/or hyperemia, temporal atrophy, and cupping were seen 93% of cases. Macular changes (epiretinal membrane, RPE changes, and focal macular atrophy) were common (85%). Peripheral punched-out retinal lesions (61%), bone spicule-like deposits (59%) and vascular attenuation, alongside vasculitic changes and sheathings (61%), were also frequently observed. PVEP delays were found in 87% of cases, also with 20/20 acuity. ERG abnormalities were found in 95% of the tested subjects. The most common findings were photopic ERGs delays (86%) and reduced amplitude (75%). Inter-ocular asymmetry seen by PVEP (85%), ERG (77%) and GVF (62%) criteria was another characteristic of AINR. All but one patient had asymmetry on at least one of these tests, while 56% had asymmetry in both PVEP and ERG. Combining PVEP and ERG data, we found that 90% of the tested patients had simultaneous retinal and optic nerve involvement.
AINR patients present with recognizable clinical and functional findings. Our findings indicate that the proportion of cases with simultaneous involvement of the retina and of the optic nerve, whether at the retinal ganglion cell (RGC)/ retinal nerve fiber layer (RNFL) and/or retrobulbar level, in patients with autoimmune retinopathy - hence, actually affected with AINR - is even higher than what we recently reported in a large, multi-center case series of patients (Adamus et al. J Ophthalmic Inflamm Infect 2011;1:111-21).
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