Amyotrophic lateral sclerosis (ALS) is a serious rapidly progressive disease of the nervous system. The mean survival from the time of diagnosis is 2.5 years. Apart from Riluzole, there is no effective treatment. Care of advanced ALS will have a cost of 4-8 million NOK (Norwegian kroner) per year. Research i.a. from the investigators department has shown that increased activity in histone deacetylation enzymes (sirtuins) together with increased access to Nicotinamide Adenine Dinucleotide (NAD) can delay disease progression. Nicotinamide riboside (NR) can increase cells' access to NAD and Pterostilbene will stimulate sirtuins. The investigators want to study whether combination therapy with NR and Pterostilbene can inhibit neurodegeneration in ALS and thereby delay disease development, increase survival and improve quality of life in ALS. In the NO-ALS extension study the investigators will follow the patients who completed the original NO-ALS study. Objectives are to evaluate adverse events and give patients possibility of compassionate use, and secondarily to see if the combination of NR and pterostilbene (EH301) will decrease progression of motor symptoms and loss of vital capacity, and increase survival time in patients with ALS.
Inclusion Criteria:
- Patients who fulfilled the criteria for the NO-ALS study and have completed the
study will be proposed inclusion in the NO-ALS extension study protocol. Patients
from both arm 1 and arm 2 in the NO-ALS study will be allowed inclusion in the
prolongation study
Exclusion Criteria:
Individuals will be excluded if any of the following exclusion criteria apply:
- Dementia, fronto temporal dementia (FTD) or other neurodegenerative disorder
interfering with compliance.
- Metabolic, neoplastic, or other physically or mentally debilitating disorder.
- Patients who become tracheostomized as part of the treatment of ALS.
- Patients with short expected survival at the discretion of the investigator. Such
cases cannot be expected to follow protocol procedures.
- Use of Vit B3 or blue berry extracts outside the study