Ohtahara syndrome or Early Infantile Epileptic Encephalopathy (EIEE) with Suppression-
Burst, is the most severe and the earliest developing age-related epileptic encephalopathy.
Clinically, the syndrome is characterized by early onset tonic spasms associated with a
severe and continuous pattern of burst activity. It is a debilitating and early progressive
neurological disorder, resulting in intractable seizures and severe mental retardation.
Specific mutations in at least four genes (whose protein products are essential in lower …