Citra Raditha,Pandu Caesaria Lestari,Anna Tjandrajani
Pediatric Neurology Working Group Harapan Kita Women and Children Hospital Jakarta
Background : Ohtahara syndrome constitute the earliest presenting of the age-dependent epileptic encephalopathy syndrome. The disorder affects newborn, usually within the first three months of life. It is an electroclinical syndrome defined by its clinical features and electroencephalographic findings. The etiology varies but the majority of cases have been associated with structural brain injury.
Case Presentation Summary : A 1-month-old female infant was admitted with recurrent seizures without accompanying fever. Seizure type was brief tonic spasm, generalized and eyes glanced to right side, mimicking a startle movement which occured frequently. There was a history of hypoxic ischemic injury and epidural hematoma on left parietotemporal region on head CT scan. Neurologic examination found clonus, mild increase in muscle tone, normal head circumference and physiologic reflex, without any cranial nerve nor motoric paresis. Electroencephalography revealed burst suppressions. Patient was diagnosed with Ohtahara syndrome and treated with 2 mg/kg/day prednisone for two months. Antiepileptic drug combination were also added because of the intractable seizure. First-line drugs such as phenobarbital and phenytoin were previously administered intravenously followed by valproic acid and oxcarbamazepine. Last drugs used were levetiracetam and clonazepam. Overall, the seizures were not significantly controlled.
Learning Points/Discussion : Ohtahara syndrome generally has a poor prognosis, especially if caused by structural damaged in the brain as in this case. Survivors invariably manifest psychomotor impairment and may develop into West syndrome and Lennox-Gastaut syndrome. Management is challenging with only anecdotal evidence to support the use of specific antiepileptic drugs in this condition. Steroid and adrenecorticotropic hormone therapy also exerts limited efficacy.