Most recently, ERG findings compatible with iCSNB were also described in patients with biallelic loss-of-function variants in RIMS2. All RIMS2 patients had optic disc pallor with most demonstrating retinal vascular attenuation; affected individuals also displayed neurodevelopmental disease and infrequently abnormal glucose homeostasis, making this a syndromic form of iCSNB [15]. Here, RIMS2 is linked to Optic disc pallor.