TY - JOUR T1 - Batten’s disease presenting as visual loss in twins JF - Archives of Disease in Childhood JO - Arch Dis Child SP - 843 LP - 843 DO - 10.1136/archdischild-2018-314911 VL - 104 IS - 9 AU - Amun Sachdev AU - Stephen Scotcher Y1 - 2019/09/01 UR - http://adc.bmj.com/content/104/9/843.abstract N2 - Identical twin boys, aged 7, presented with difficulty seeing at school. Examination revealed bilateral reduced visual acuity (6/60 in one twin, 6/48 in the other) and bilateral maculopathy on funduscopy. Electrodiagnostic testing suggested an inner retinal problem, demonstrating an absent b wave and relatively normal a wave; rod-specific and flash-photopic electroretinograms (ERG) had an electronegative maximal response, cone-flicker ERG was reduced/delayed and pattern ERG was undetectable (figure 1). A diagnosis … ER -