A Case of Lissencephaly Type 1 in a 11 Month Old Infant

Dr. Shrasta Soumya, Dr. Ipsita Vashishtha, Dr. Ravi Prakash Naulakha, Dr. Neelu Elon, Dr. Prithi Inamdar, Dr. Rajesh Kumar Rai

Abstract: Lissencephaly means smooth brain, i.e., brain without convolutions or gyri, rare and severe cortical malformation. It results from a defect in neuronal migration with four rather than six layers in cortex and the onset of this disorder is presumed to be before 9 weeks of gestation The spectrum ranges from complete absence of gyri (agyria) to broad gyri with reduced sulci (pachygyria), with an abnormally thick cortex. [1] Here, we report a 11 months old male babywith failure to thrive, spasticity, global developmental delay, microcephaly and cortical visual impairment. MRI brain was done which was suggestive of diffuse gyral thickening involving bilateral cerebral hemispheres with broad flat gyri and shallow sulci suggestive of Agyria ? Pachygyria complex (Lissencephaly) and mild dilatation of bilateral lateral ventricles and widening of bilateral sylvian fissures.

Keywords: Lissencephaly, neuronal migration, agyria, pachygyria, microcephaly, sylvian fissures