RESUMEN
Ohtahara syndrome (OS) is a rare epileptic encephalopathy that is characterized by an abnormal electroencephalogram (EEG) and intractable seizures in the neonatal and early infantile period. The patient of this reported case was delivered normally at 39 weeks of gestation without any complication. One week after birth, seizures that were refractory to anticonvulsants started with repetitive clustered tonic spasms. The child had no abnormal findings on the initial laboratory investigations. But he was diagnosed with OS according to the frequent tonic spasms, an abnormal EEG pattern of suppression-burst and magnetic resonance imaging of cortical dysplasia. He was planned to undergo an operation for brain lesion. This report describes our experience with the general anesthetic management when we performed craniotomy and right hemispherotomy for a patient with OS.
Asunto(s)
Niño , Humanos , Embarazo , Anticonvulsivantes , Encéfalo , Craneotomía , Electroencefalografía , Imagen por Resonancia Magnética , Malformaciones del Desarrollo Cortical , Parto , Convulsiones , EspasmoRESUMEN
Early myoclonic encephalopathy (EME) is a rare malignant epileptic syndrome. The erratic myoclonus with or without focal motor seizures, onset before 3 months of age, and persistent suppression-burst pattern in electroencephalograph (EEG) are accepted as the diagnostic criteria for EME. We report an 11 month-old infant with EME which was secondary to non-ketotic hyperglycinemia.
Asunto(s)
Anticonvulsivantes/uso terapéutico , Electroencefalografía/métodos , Epilepsias Mioclónicas/diagnóstico , Epilepsias Mioclónicas/tratamiento farmacológico , Epilepsias Mioclónicas/etiología , Humanos , Hiperglicinemia no Cetósica/complicaciones , Hiperglicinemia no Cetósica/diagnóstico , Lactante , MasculinoRESUMEN
No abstract available.