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Related Experiment Video

Updated: May 15, 2025

Elevated Plus Maze Test Combined with Video Tracking Software to Investigate the Anxiolytic Effect of Exogenous Ketogenic Supplements
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The Ketogenic Diet: An Underrecognized Therapy for Rett Syndrome.

Babitha Haridas1, Stacey Bessone2, Zahava Turner1

  • 1Department of Neurology, Johns Hopkins University, Baltimore, MD, USA.

Journal of Child Neurology
|April 10, 2025
PubMed
Summary
This summary is machine-generated.

The ketogenic diet shows promise for managing seizures in Rett syndrome, with two-thirds of patients experiencing significant seizure reduction. This diet may also improve cognition and alertness in children with this neurodevelopmental disorder.

Keywords:
Rett syndromechildrendietary therapyepilepsyketogenic diet

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Area of Science:

  • Neuroscience
  • Pediatrics
  • Metabolic Disorders

Background:

  • Rett syndrome is a severe X-linked neurodevelopmental disorder.
  • Epilepsy affects 50-90% of Rett syndrome patients, often refractory.
  • Limited data exists on ketogenic diet efficacy for Rett syndrome seizures.

Purpose of the Study:

  • To evaluate the ketogenic diet's effectiveness and tolerability for seizures in Rett syndrome.
  • To assess impact on cognition and alertness.
  • To explore the role of gastrostomy tubes in diet initiation.

Main Methods:

  • Retrospective case series of 9 children with Rett syndrome on ketogenic diet.
  • Data collected on seizure frequency, cognition, and alertness.
  • Diet initiated via ketogenic formula or food-based approach, often with gastrostomy tube support.

Main Results:

  • 67% (6/9) achieved ≥50% seizure reduction at 6 months; 44% (4/9) at 12 months.
  • Patients with ≥50% seizure reduction showed improved cognition and alertness.
  • Ketogenic diet was well-tolerated, with gastrostomy tubes aiding administration.

Conclusions:

  • The ketogenic diet is effective and well-tolerated for managing seizures in Rett syndrome.
  • Significant seizure reduction correlates with cognitive and alertness improvements.
  • Gastrostomy tubes facilitate ketogenic diet initiation and management in this population.