ALS

About

ALS is a fatal neurodegenerative disorder of unknown etiology for which there is no effective treatment. Progressive denervation of neuromuscular synapses in the peripheral nervous system (PNS) and degeneration of upper and lower motor neurons in the central nervous system (CNS) result in muscle weakness, atrophy, paralysis and ultimately death within 2-3 years from the onset of symptoms. Initial presentation of ALS varies between affected individuals, and typically presents as spinal-onset disease (muscle weakness of the limbs), or bulbar-onset disease (difficulty with speech and swallowing). Sporadic ALS (sALS) accounts for 90% of cases and has no clear aetiology, while familial ALS (fALS) accounts for 10% of cases and contains an underlying genetic component. However, while these two forms differ in causation, they appear pathologically and clinically indistinguishable. There is no known cure for ALS. There are two approved medications to treat ALS, riluzole (a glutamate blocker) and edaravone (a free radical scavenger), but with limited efficacy. Riluzole, approved in 1995, is administered orally twice daily and delays time to tracheostomy or death in patients with ALS (Riluzole package insert 2016), prolonging survival by 2-3 months (Miller et al. 2012). Edaravone, approved in the US in 2017, is administered in courses intravenously and shows efficacy in only a small subset of patients with ALS.

Head of research

Ole-Bjørn Tysnes

Last updated 27.07.20

Trials

Principal Investigators: Ole-Bjørn Tysnes, Charalampos Tzoulis

To test the potential of NR as a neuroprotective therapy for ALS, we will perform NO-ALS, a multi-centre, phase II randomized double-blinded clinical trial, comparing combined oral NR and pterostilbene to placebo in early ALS. Based on power estimations, a total of 180 patients will be recruited nation-wide.

This project is entirely novel and has the potential to discover a therapy modulating disease activity and progression in ALS, thus vastly improving patient care and prognosis.

We started including patients in October 2020.

participating centres

  • Haukeland University Hospital, Bergen
  • Akershus University Hospital, Lørenskog
  • University Hospital of North Norway

funding

  • The Regional Health Authority of Western Norway
  • Norwegian research council, Neuro- SysMed
  • Haukeland University Hospital
  • University of Bergen
  • Participating hospitals

Last updated October 30th, 2020 at 12:48 pm

Collaborators

Professor Trygve Holmøy, Akershus University Hospital.
Professor Hilde Nilsen, University of Oslo.
Associate Professor Evandro Fei Fang, University of Oslo.

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