The strong association of PCDH19 with epilepsy inheritance is also stressed by genetic screening demonstrating that 11 out of 45 patients suffering from Dravet syndrome (severe myoclonic epilepsy of infancy) carried mutations in PCDH19 (Depienne et al., 2009). This evidence concerns the gene PCDH19 and encephalopathy, progressive, early-onset, with brain edema and/or leukoencephalopathy.