[The Ohtahara's syndrome: a special form of age-dependent epilepsy].
The aim of this report is to review the clinical and the outcome data in four patients with Ohtahara syndrome at Sant Joan de Déu Hospital, Barcelona, following the same clinical and electroencephalografic criteria reported by the author. The etiology of this syndrome is unknown and plurifactorial. Investigation studies were negative except for the EEG and neuroimaging. MRI was performed in two children, and pachigyria was observed in one and mycropoligyria in the other. There were no response to cofactors, phenobarbital, vigabatrine and valproate. Therefore two patients underwent treatment with ACTH with no response in one and good response in the other. Two patients died at 2 and 6 months; other one was transferred to another center at 6 months-old and was lost at follow-up. The fourth patient, fourteen-month-old, is free of seizures with improvement of EEG register with important development retardation. We conclude that Ohtahara syndrome is a severe neonatal epilepsy with poor neurologic outcome; MRI detects often brain malformations as cortical dysplasia and a therapeutic trial of ACTH is indicated because the possibility of control of the seizures.
Campistol
J
J
Servicio de Neurología, Hospital Universitari Sant Joan de Déu, Barcelona, España.
García-García
J J
JJ
Lobera
E
E
Sanmartí
F X
FX
Conill
J
J
Fernández-Alvarez
E
E
D000324:Adrenocorticotropic Hormone / Q000627:therapeutic use; D017668:Age of Onset; D000641:Ammonia / Q000097:blood; D001921:Brain / Q000002:abnormalities; D004569:Electroencephalography; D004827:Epilepsy / Q000175:diagnosis* / Q000188:drug therapy; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D019344:Lactic Acid / Q000097:blood; D008279:Magnetic Resonance Imaging; D008297:Male; D019289:Pyruvic Acid / Q000097:blood; D012189:Retrospective Studies; D013577:Syndrome
D019344:Lactic Acid; D000641:Ammonia; D019289:Pyruvic Acid; D000324:Adrenocorticotropic Hormone
Síndrome de Ohtahara: una forma de epilepsia edad-dependiente.