Hemimegalencephaly with microgyric – Agyric cortex

Abstract

A 2-month-old male child with normal birth history developed bilateral asymmetric tonic spasms on day four of life. The spasms occurred multiple times in a day. The right frontal prominence was noted on the examination. The patient had no cutaneous marker or focal neurological deficit. The electroencephalograpy showed asymmetric diffuse abnormalities of background more marked on right side. The cranial computed tomography showed right sided hemi megalencephaly with enlarged ventricle and prominent occipital sign. The marked abnormalities of gyration noted along with hemimegalencephaly including anterior microgyric and posterior agyric cortex. The microgyric cortex was also noted on the contra-lateral side. The basal ganglion complex was poorly demarcated (Figs 1–4). Hemimegalencephaly is a congenital malformation of cortical development that consists of a unilater