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[Effect of the ketogenic diet for West syndrome into which early infantile epileptic encephalopathy with

Y Takusa1, M Ito, A Kobayashi

  • 1Department of Pediatrics, Shimane Medical University, Izumo.

No to Hattatsu = Brain and Development
|September 1, 1995
PubMed

Insights

Early infantile epileptic encephalopathy (EIEE) in an infant responded dramatically to a ketogenic diet after other treatments failed. This suggests early trial of ketogenic diet for EIEE management.

Area of Science:

  • Neurology
  • Pediatric Neurology
  • Epileptology

Background:

  • Early infantile epileptic encephalopathy (EIEE) is a severe form of epilepsy presenting in the first year of life.
  • Suppression-burst pattern on electroencephalogram (EEG) is characteristic of EIEE.
  • Standard treatments for EIEE often have limited efficacy.

Observation:

  • A male infant presented with tonic spasms and suppression-burst EEG at one month of age.
  • Initial treatments with pyridoxal phosphate, thyrotropin-releasing hormone (TRH), and ACTH showed transient or no seizure control.
  • ACTH therapy was associated with transient seizure remission but subsequent reappearance and apparent cerebral shrinkage.

Findings:

  • The patient evolved into West syndrome, a severe epilepsy typically developing later.
  • Introduction of a ketogenic diet led to immediate seizure cessation.
  • EEG findings significantly improved following the ketogenic diet.

Implications:

  • The ketogenic diet may be a highly effective early therapeutic option for EIEE.
  • This case highlights the potential benefits of early ketogenic diet intervention in severe infantile epilepsies.
  • Further research into ketogenic diet efficacy in EIEE is warranted.

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