DR. CHANDNI CHAKRABORTY

DR. SHOUMYA JYOTI DATTA MAZUMDER

Abstract

Lissencephaly is a rare gene linked Brain malformation commonly associated with ocular manifestations like abnormal VEP,Cortical Blindness,, Macular and Optic Nerve Hypoplasia, Optic Atrophy and Eso or Exotropia. Here we present a case of two and a half year old child Diagnosed as Lissencephaly (Agyria/Pachygyria) who presented with apparently cortical blindness and intermittent exotropia with history of abnormal VEP records at 11 month that showed evidence of normalisation by two and a half years.The child also showed evidence of improvement in functional vision with light stimulation.