[Early infantile epileptic encephalopathy and glycine encephalopathy].
BACKGROUND
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.


METHODS
We report a neonate with EIEE secondary to glycine encephalopathy, diagnosed by increased of LCR/plasma glycine index.
González de Dios
J
J
Servicio de Pediatría, Hospital Universitario San Juan, Alicante, España.
Moya
M
M
Pastore
C
C
Izura
V
V
Carratalà
F
F
D001927:Brain Diseases / Q000209:etiology* / Q000503:physiopathology*; D004569:Electroencephalography; D004829:Epilepsy, Generalized / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology*; D017809:Fatal Outcome; D005260:Female; D005998:Glycine / Q000134:cerebrospinal fluid*; D006801:Humans; D007231:Infant, Newborn; D008661:Metabolism, Inborn Errors / Q000134:cerebrospinal fluid* / Q000150:complications*; D013577:Syndrome; D013997:Time Factors
D005998:Glycine
Encefalopatía epiléptica infantil precoz y encefalopatía glicinémica.