Ohtahara syndrome: management

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Ohtahara syndrome: management. This shows a suppression burst pattern. Fully referenced neurology checklist.

Electroencephalogram (EEG)

  • This shows a suppression burst pattern
  • There are high amplitude spikes and polyspikes
  • These alternate with periods of electric suppression
  • The abnormalities persist in sleep
  • There is hypsarrhythmia when West syndrome sets in

Anti-epileptic drugs (AEDs)

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Other treatments

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References

  1. Beal JC, Cherian K, Moshe SL. Early-onset epileptic encephalopathies: Ohtahara syndrome and early myoclonic encephalopathy. Pediatr Neurol 2012; 47:317-323.
  2. Fullston T, Brueton L, Willis T, et al. Ohtahara syndrome in a family with an ARX protein truncation mutation (c.81C>G/p.Y27X). Eur J Hum Genet 2010; 18:157-162.
  3. Kural Z, Ozer AF. Epileptic encephalopathies in adults and childhood. Epilepsy Res Treat 2012; 2012:205131.
  4. Wilmshurst JM, Gaillard WD, Vinayan KP, et al. Summary of recommendations for the management of infantile seizures: Task Force Report for the ILAE Commission of Pediatrics. Epilepsia 2015; 56:1185-1197.

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