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[Early infantile epileptic encephalopathy and glycine encephalopathy]
J González de Dios1, M Moya, C Pastore
1Servicio de Pediatría, Hospital Universitario San Juan, Alicante, España.
Revista De Neurologia
|April 7, 1998
Abstract
Introduction:
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.
Clinical Case:
We report a neonate with EIEE secondary to glycine encephalopathy, diagnosed by increased of LCR/plasma glycine index.