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Tideglusib for the Treatment of Amyotrophic Lateral Sclerosis

Phase 2
Not yet recruiting
Conditions
Amyotrophic Lateral Sclerosis
Interventions
Registration Number
NCT05105958
Lead Sponsor
University Hospital, Geneva
Brief Summary

Amyotrophic lateral sclerosis (ALS) is a severe neurodegenerative condition, mainly characterized by progressive weakness and wasting of the limbs, the respiratory and bulbar muscles. Respiratory insufficiency leads to a fatal outcome after a mean diseases duration of only three to five years. The disease is characterized by pathological accumulations of a protein called TDP-43, which can be found large cortical and sub-cortical areas of post-mortem ALS brains.

No causal treatment for this condition is known to date, and there is a large unmet need to develop new strategies in order to halt or slow down its progression.

The aim of this study is to test the safety and tolerability of Tideglusib, a treatment that is already in clinical trials for other neuromuscular conditions, in patients with ALS. It is assumed that this drug may have a significant therapeutic benefit in this population due to his mode of action: In the ALS mouse model, Tideglusib decreases significantly the amount of accumulated TDP-43 proteins within the cells.

Detailed Description

Not available

Recruitment & Eligibility

Status
NOT_YET_RECRUITING
Sex
All
Target Recruitment
98
Inclusion Criteria
  • Possible, probable (clinically or laboratory supported) or definite ALS according to the revised version of the El Escorial criteria
  • Disease duration < 18 months
  • Vital capacity of more than 60% of normal (defined as slow vital capacity, best of three measurements)
  • Age more than 18 years
  • On a stable dose of riluzole for at least four weeks or not taking riluzole
  • On a stable dose of edaravone for at least four weeks or not taking edaravone
  • Capable of thoroughly understanding all information given and giving full informed consent according to GCP
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Exclusion Criteria
  • Previous participation in another clinical study within the preceding 12 weeks
  • Proven SOD1- or FUS - mutation
  • Tracheostomy or assisted ventilation of any type during the preceding three months
  • Pregnancy or breast-feeding females
  • Any medical condition known to have an association with motor neuron dysfunction which might confound or obscure the diagnosis of ALS
  • Presence of any concomitant life-threatening disease or impairment likely to interfere with functional assessment
  • Evidence of a major psychiatric disorder or clinically evident dementia precluding evaluation of symptoms
  • Alcoholism
  • Cardiovascular disorder/arrhythmia
  • Impaired kidney function, defined as creatinine levels of 2.5 x upper limit of normal (ULN)
  • Impaired liver function, defined as aspartate aminotransferase (AST) or alanine aminotransferase (ALT) of 3 x ULN
  • Liable to be not cooperative or comply with trial requirements as assessed by the investigator, or unable to be reached in the case of emergency
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Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
TideglusibTideglusibPatients receive 1000 mg Tideglusib once daily per os
PlaceboTideglusibPatients receive placebo matching Tideglusib 100 mg once daily per os
Primary Outcome Measures
NameTimeMethod
Increase in Alanine Aminotransferase14 weeks

Increase in Alanine Aminotransferase \< 3x of Upper Limit of Normal

Secondary Outcome Measures
NameTimeMethod
Most common side effect14 weeks

Occurence of diarrhea in less then 18 % of patients

Trial Locations

Locations (5)

University Hospital Lausanne

🇨🇭

Lausanne, Switzerland

University Hospital Zurich

🇨🇭

Zürich, Switzerland

University Hospital Bern

🇨🇭

Bern, Switzerland

Kantonsspital St. Gallen

🇨🇭

Saint-Gall, Switzerland

University Hospital Geneva

🇨🇭

Genève, Switzerland

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