Using a ketone ester to improve energy levels in ALS patients

Efficacy and Tolerability of Beta Hydroxybutyrate Ester in Patients With Amyotrophic Lateral Sclerosis (ALS)

Not applicable Interventional University of Ulm · NCT04820478

This study is testing if a special energy supplement can help people with ALS feel more energetic and maintain their weight while they continue their usual treatment.

Quick facts

PhaseNot applicable
Study typeInterventional
Enrollment76 (estimated)
Ages18 Years and up
SexAll
SponsorUniversity of Ulm Academic / other
Locations1 site (Ulm, Baden-Wurttemberg)
Trial IDNCT04820478 on ClinicalTrials.gov

What this trial studies

This clinical trial investigates the efficacy and tolerability of beta hydroxybutyrate ester as a nutritional supplement for patients with amyotrophic lateral sclerosis (ALS). The study aims to address weight loss, a negative prognostic factor in ALS, by providing a high-energy substrate that may help mitigate energy deficits in affected cells. Participants will receive either the ketone ester or a placebo while being continuously treated with riluzole. The trial will assess changes in functional rating scales to evaluate the intervention's impact.

Who should consider this trial

Good fit: Ideal candidates include adults aged 18 and older with a confirmed diagnosis of ALS who have experienced a decline in their functional rating scores.

Not a fit: Patients with metabolic disorders that prevent the utilization of ketone bodies or those with severe gastro-esophageal reflux may not benefit from this study.

Why it matters

Potential benefit: If successful, this intervention could improve energy levels and overall health outcomes for ALS patients.

How similar studies have performed: While various interventional studies have explored nutritional approaches in ALS, the specific use of beta hydroxybutyrate ester is relatively novel and has not been extensively tested.

Eligibility criteria

Show full inclusion / exclusion criteria
Inclusion Criteria:

* Probable (clinically or laboratory) or definite ALS according to the revised version of the El Escorial World Federation of Neurology criteria
* loss of Amyotrophic Lateral Sclerosis Functional Rating Scale Revised (ALSFRS-R) of ≥ 0.33 points per month since onset (first paresis), based on the formula: (48 - score at screening visit) / (months between onset and screening visit)
* age ≥ 18 years
* continuously treated with 100 mg riluzole per day for at least 4 weeks
* capable of thoroughly understanding all information given and giving full informed consent according to good clinical practice (GCP)

Exclusion Criteria:

* hyperinsulinism
* pyruvate decarboxylase deficit
* disturbance of fatty acid oxidation
* disturbance of gluconeogenesis
* acute porphyria
* metabolism disorders which prevent utilization or degradation of ketone bodies
* severe gastro-esophageal reflux
* renal insufficiency (medical history and/or elevated serum creatinine levels and/or glomerular filtration rate (GFR) \<90 ml/min
* previous participation in another interventional study within the preceding 4 weeks
* tracheostomy
* pregnancy or breast-feeding females
* evidence of a major psychiatric disorder or clinically evident dementia
* intake of diuretics
* severe dysphagia
* nutrition via percutaneous endoscopic gastrostomy (PEG)
* electrolyte or acid-base imbalance
* heart failure New York Heart Association (NYHA) II or above

Where this trial is running

Ulm, Baden-Wurttemberg

Study contacts

How to participate

  1. Review the eligibility criteria above with your treating physician.
  2. Visit the official trial page on ClinicalTrials.gov for the most current contact information and recruitment status.
  3. Contact the listed study coordinator or principal investigator to request pre-screening. Pre-screening is free and never obligates you to enroll.
Conditions Amyotrophic Lateral Sclerosisnutritionbeta hydroxybutyrate esterhigh-caloricketone esterketone monoester
Last reviewed 2026-06-13 by the Find a Trial editorial team. Information on this page is for educational purposes and is not medical advice. Always consult qualified healthcare professionals about clinical trial participation.