Roy, BMondal, GNanda, DDas, SDas, MK2026-05-202026-05-202013https://hdl.handle.net/20.500.14572/6153B Roy Dr. B.C. Roy Postgraduate Institute of Paediatric Sciences, Kolkata, West Bengal G Mondal Burdwan Medical College, West Bengal D Nanda Dr. BC Roy Postgraduate Institute of Paediatric Sciences, Kolkata, West Bengal S Das Institute of Hygiene, Kolkata, West Bengal MK Das Dr. B.C.Roy Postgraduate Institute of Paediatric Sciences, Kolkata, W.BAbstract: A 9 yr girl presented with bilateral ptosis and deafness of gradual onset for the last four years. Associated ophthalmoplegia and pigmentary retinopathy, heart block, raised CSF protein and serum lactate was suggestive of the diagnosis of Kearns-Sayre syndrome (KSS), a rare entity in the spectrum of the mitochondrial deletion syndrome. Search for endocrinopathy revealed no abnormality.en-USPtosisophthalmoplegiapigmentary retinopathyKSSmitochondrial deletionArticle