Ohtahara syndrome[1] | |
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Other names | Early infantile epileptic encephalopathy with burst-suppression; Early Infantile Developmental & Epileptic Encephalopathy |
Specialty | Neurology |
Ohtahara syndrome (OS), also known as Early Infantile Developmental & Epileptic Encephalopathy (EIDEE)[2] is a progressive epileptic encephalopathy. The syndrome is outwardly characterized by tonic spasms and partial seizures within the first few months of life,[3] and receives its more elaborate name from the pattern of burst activity on an electroencephalogram (EEG). It is an extremely debilitating progressive neurological disorder, involving intractable seizures and severe intellectual disabilities. No single cause has been identified, although in many cases structural brain damage is present.[4]