González de Dios J, Moya M, Pastore C, Izura V, Carratalà F
Servicio de Pediatría, Hospital Universitario San Juan, Alicante, España.
Rev Neurol. 1997 Dec;25(148):1916-8.
Early infantile epileptic encephalopathy (EIEE) with suppression burst activity in EEG (Ohtahara syndrome) is a rare type of epileptic encephalopathy in infancy and represents the earliest type of age-related symptomatic generalized epilepsy. The main etiologic factors associated to EIEE are cerebral dysgenesia and metabolopathies, principally nonketotic hyperglycinemia.
We report a neonate with EIEE secondary to glycine encephalopathy, diagnosed by increased of LCR/plasma glycine index.
