Persistence of suppression-bursts in a patient with Ohtahara syndrome.
Early infantile epileptic encephalopathy, or Ohtahara syndrome, is characterized by tonic spasms and a suppression-burst pattern on the electroencephalography (EEG). The EEG demonstrates a suppression-burst pattern during waking and sleeping states that often evolves into hypsarrhythmia and followed later by a diffuse slow spike-wave pattern. In other patients, the EEG evolves into focal spike discharges or multiple independent spike foci. We report a 5-year-old girl with Ohtahara syndrome that persistently demonstrated tonic spasms and suppression-burst on multiple EEGs. Over her lifetime, neither hypsarrhythmia nor diffuse slow spike-wave pattern were seen. This suggests that in Ohtahara syndrome, a suppression-burst pattern can persist over a long period of time.
Saneto
Russell P
RP
Division of Pediatric Neurology, Children's Hospital and Regional Medical Center/University of Washington, Seattle, Washington 98105, USA. russ.saneto@seattlechildrens.org
Sotero de Menezes
Marcio
M
D002675:Child, Preschool; D004569:Electroencephalography; D004829:Epilepsy, Generalized / Q000503:physiopathology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases / Q000503:physiopathology*; D012890:Sleep / Q000502:physiology