Ohtahara syndrome
ICD-10 G40.8 · ICD-11 8A62.Y.4

Ohtahara Syndrome: Next-Line Management When Adrenocorticotropic Hormone Therapy Has Not Controlled Seizures

In Ohtahara syndrome, achieving seizure control is the central clinical objective. When a first-line treatment approach does not reach that goal, a specific next-line protocol is indicated. This page outlines that escalation scenario.

Previous treatment — goal not met

When adrenocorticotropic hormone therapy fails

Adrenocorticotropic hormone (ACTH) therapy is used with the aim of controlling seizures in Ohtahara syndrome. Failure to achieve seizure control with this approach is the trigger that warrants escalation to the next treatment line described below.

Next-line approach

This protocol involves a specialised dietary intervention. The complete regimen, including implementation parameters and monitoring guidance, is available in the structured protocol.

Clinical goal

Control of seizures.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1016/j.pediatrneurol.2012.06.002

Some success in controlling seizures has been reported with the ketogenic diet in Ohtahara syndrome, but not in early myoclonic encephalopathy.

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