Tideglusib for the Treatment of Amyotrophic Lateral Sclerosis

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 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