Introduction & Background PCDH19 syndrome is a monogenic epilepsy related to PCDH19, which encodes for a protein important for brain development. This protein also seems to regulate the GABA-A receptor. The PCDH19 gene is located on chromosome Xq22.1; it consists of six exons and encodes for a calcium-dependent adhesion molecule called protein protocadherin-19 (PCDH19). The protein is expressed mainly in the central nervous system, the limbic system, and the cortex [1].