Status:

RECRUITING

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

Lead Sponsor:

University of Ulm

Conditions:

Amyotrophic Lateral Sclerosis

Eligibility:

All Genders

18+ years

Phase:

NA

Brief Summary

Weight loss is a known negative prognostic factor in amyotrophic lateral sclerosis (ALS). One potential mechanism of weight loss in ALS is a disturbance of the mitochondrial complex I which causes an ...

Eligibility Criteria

Inclusion

  • 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

  • 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

Key Trial Info

Start Date :

April 1 2022

Trial Type :

INTERVENTIONAL

Allocation :

ESTIMATED

End Date :

October 1 2025

Estimated Enrollment :

76 Patients enrolled

Trial Details

Trial ID

NCT04820478

Start Date

April 1 2022

End Date

October 1 2025

Last Update

May 8 2024

Active Locations (1)

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1

University of Ulm

Ulm, Baden-Wurttemberg, Germany, 89081