Ohtahara Syndrome
Ohtahara Syndrome Causes
The specific cause of Ohtahara syndrome has not been ascertained. However, the disease is closely associated with metabolic disorders or structural damage in the brain. Most cases of Ohtahara syndrome show severe atrophy in the two hemispheres of the brain. Less commonly, Ohtahara syndrome may be caused by an underlying syndrome. To date, no genetic link has been identified.
Ohtahara Syndrome Definition
Ohtahara syndrome pertains to a neurological disorder characterized by epileptic seizures which affect newborn infants, usually within the first 3 months of life and more commonly within the first 10 days.
Ohtahara Syndrome Diagnosis
An electroencephalogram (EEG) is the primary diagnostic method used to determine Ohtahara syndrome in affected newborns. EEGs of affected infants reveal a pattern known as ?burst suppression?, which is characterized by a high voltage spike followed by little activity in the brain.
Ohtahara Syndrome Symptoms and Signs
Primary, affected infants suffer from tonic seizures, but some cases may show partial seizures, and rarely, myoclonic seizures as well. Tonic seizures are generalized seizures characterized by a sudden stiffening of the limbs. Aside from seizures, Ohtahara syndrome also presents with profound physical and mental retardation.
Ohtahara Syndrome Treatment
Treatment options for Ohtahara syndrome are limited. Frequently, anti-epileptic drugs and steroids are used to manage the seizures, but with little success or limited efficacy. In cases where a focal brain lesion has been identified, surgical intervention may be necessitated. Other treatment options for Ohtahara syndrome are mostly symptomatic and supportive.