Fig. 2

a Patient 1 initial MRI at age 9 weeks showing poor myelination with absent dark T2 and bright T1 signal in white mater tracts, marked widening of the Sylvian fissure (black arrow), moderate widening of the frontal sulci (white arrow) and mild ventricular dilatation in favor of frontal and temporal cortical atrophy. Marked symmetrical thinning of the corpus callosum (arrow heads). b Patient (1) follow up MRI at the age of 23 weeks showing significant progress in myelin loss with absent white mater myelination on T2 and marked progression in the frontal and temporal cortical atrophy. There is marked reduction of the size and deformity of the hippocampus (black arrow) with loss of its normal ram horn like whirled appearance. There is focal restricted diffusion along the cortico-spinal tract in the brain stem (white arrow)