Praevilia Salendu, Deddy Ria Saputra, Irawan Mangunatmadja
OBJECTIVE.Ohtahara syndrome is a rare epileptic encephalopathy that is characterized by an abnormal electroencephlography and intractable seizures in the neonatal and early infantile periode. The disorder affects newborn, usually within the first three months of life. The underlying causes are heterogenous. CASE.As a case demonstration of Ohtahara syndrome.A 1-month-old infant girl was admitted with chief complain of recurrent seizures without accompanying fever. Seizure type was brief tonic spasm generalized and lateralized, mimicking a startle movement which occured frequently. Neurologic examination found positive clonnus, mild increase muscle tone, normal head size and physiologic reflex, without any cranial nerve nor motoric parases. Electroencephalography revealed burst suppression. Patient was diagnosed with Ohtahara syndrome and treated with prednisone with dose of 2 mg/kg/day for two months. Antiepileptic drugs combination were also added because of the intractable seizure. First line drug such as Phenobarbital and phenytoin injection were previously administered followed with valproic acid and clobazam. Overall, the seizures were not significantly controlled. CONCULSION.Ohtahara syndrome is electroclinical syndromes, defined by clinical presentation and specific electroencephalography findings. Survivors invariably manifest psychomotor impairment and may develop into West syndrome and Lennox-Gastaut syndrome. Antiepileptic drugs are used to control seizures, but are unfortunately not usually very effective for disorder. Corticosteroid are occasionally helpful.