Ketone Ester Supplementation to Alleviate Symptoms of Angelman Syndrome

Tech ID: 15A038

Advantages

  • Improves motor coordination 
  • Improves learning and memory
  • Decreases seizure frequency 
  • Increases seizure threshold 
  • Helps to normalize weight 
     

Summary

Angelman syndrome (AS) is a devastating neurological disorder for which there is no cure. Research in the AS field primarily focuses on finding an overall treatment for the disorder, without specific examination of the devastating epilepsy phenotype. Epilepsy is a common cause of death in AS and is present in >80% of AS patients, with 77% of these patients remaining refractory. Unfortunately, common anti-epileptic drugs have serious adverse side effects. Dietary therapeutic approaches are promising; however, they can be especially problematic in this patient population. AS patients typically suffer from GI abnormalities, olfactory aversions, low body weight, and reduced muscle tone. Thus, the use of a ketone ester supplement may represent a more accessible and palatable method of inducing ketosis in AS patients.

Our researchers have developed a protocol for the use of a ketone ester supplement to treat the neurological symptoms of Angelman syndrome. The results indicate improvements in seizure, motor, and various cognitive functions in AS. This therapy could help increase life expectancy and may possibly affect overall cognition if treatment is started early. Additionally, this may lead to further clinical evaluation in both AS patients and any individuals suffering from intractable epilepsy.

Modified TCA Cycle

Desired Partnership

  • License

 

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