Ph3 Randomised Double-blind Study of Arimoclomol in Patients with ALS

  • Research type

    Research Study

  • Full title

    A Phase 3, Randomised, Placebo-Controlled Trial of Arimoclomol in Amyotrophic Lateral Sclerosis

  • IRAS ID

    246934

  • Contact name

    Michael Hanna

  • Contact email

    m.hanna@ucl.ac.uk

  • Sponsor organisation

    Orphazyme A/S

  • Eudract number

    2018-000137-13

  • Duration of Study in the UK

    2 years, 2 months, 1 days

  • Research summary

    Amyotrophic lateral sclerosis (ALS), also called Lou Gehrig's disease, is a progressive, fatal neurodegenerative disorder and the symptoms include loss of muscle control in hands, arms and legs, tripping and falling, difficulty speaking, swallowing and/or breathing, persistent fatigue, and twitching and cramping of the muscles. In addition, cognitive and behavioural changes are often seen. To date only 2 drugs are approved for the treatment of ALS., therefore the purpose of this trial to evaluate the efficacy and safety of a drug called Arimoclomol over a 76 weeks’ treatment period.
    Orphazyme A/S is developing arimoclomol for the treatment of ALS. Arimoclomol works by increasing the heat shock response under conditions of cellular stress. The heat shock response promotes natural folding of nascent proteins and refolding of damaged or mutated proteins via enhanced heat shock protein expression, a mechanism of action that is thought to be highly relevant to an essential and early pathophysiological event that leads to neurodegeneration in ALS.
    The trial will be conducted at approximately 26 sites in approximately 11 countries in North America and Europe over a 76 weeks’ treatment period. This study will include 22 visits (11 of these visits will be done remotely by phone calls, and the rest will be done at the clinic by the study doctor or site staff). The study will consist of Screening, Baseline, Treatment Phase, End of Trial and End of Treatment visits. Patients who are eligible will be randomised in a 2:1 ratio to one of the following treatments:
    • Arimoclomol 1200 mg/day (400 mg TID)
    • Placebo (matching)
    Study-related tests and procedures include: neurological and physical examination, lumbar puncture for CSF, completion of questionnaires, blood & urine analyses and ECG.

  • REC name

    London - Brighton & Sussex Research Ethics Committee

  • REC reference

    18/LO/1313

  • Date of REC Opinion

    9 Nov 2018

  • REC opinion

    Further Information Favourable Opinion