TY - JOUR A1 - Naik, Anmol A1 - Ratra, Dhanashree A1 - Banerjee, Aniruddha A1 - Dalan, Daleena A1 - Jandyal, Sourabh A1 - Rao, Girish A1 - Sen, Parveen A1 - Bhende, Muna A1 - Jayaprakash, V A1 - Susvar, Pradeep A1 - Walinjkar, Jaydeep A1 - Rao, Chetan T1 - Enhanced S-cone syndrome: Clinical spectrum in Indian population Y1 - 2019/4/1 JF - Indian Journal of Ophthalmology JO - Indian J Ophthalmol SP - 523 EP - 529 VL - 67 IS - 4 UR - https://journals.lww.com/ijo/pages/default.aspx/article.asp?issn=0301-4738;year=2019;volume=67;issue=4;spage=523;epage=529;aulast=Naik DO - 10.4103/ijo.IJO_1480_18 N2 - Purpose: Enhanced S-cone syndrome (ESCS), a rare disorder, is often misdiagnosed as other forms of retinal degenerations, which have a poorer prognosis than ESCS. The aim of this study is to report the varied clinical features of ESCS and distinguish it from other similar disorders. Methods: We retrospectively scrutinized the records of patients with confirmed diagnosis of ESCS and analyzed the findings. Results: We included 14 patients (age range 4–39 years) who were confirmed to have ESCS according to pathognomonic electroretinography (ERG) showing reduced photopic, combined responses, and 30 Hz flicker with reduced L, M cone responses and supernormal S cone responses. The disease presented in the 1st decade with night blindness and was almost stationary or minimally progressive. Mid-peripheral fundus changes in form of nummular pigmentary alterations, yellow punctate lesions, and macular schisis were noted. The vision ranged from 6/6 to 6/36 with follow-up ranging from 1month to 22 years. Conclusion: ESCS shows varied clinical features ranging from unremarkable fundus to pigment clumping and atrophic lesions. It has good prognosis with patients mostly maintaining their vision. ERG is diagnostic. More awareness and knowledge about this entity can help to differentiate it from other forms of night blindness. ER -