A Trial of Nicotinamide/Pterostilbene Supplement in ALS: The NO-ALS Extension Study

N/AActive Not RecruitingNCT05095571

Haukeland University Hospital300 enrolled

Overview

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.

Study Type

INTERVENTIONAL

Allocation

Purpose

TREATMENT

Masking

NONE

Enrollment

300

Conditions

Amyotrophic Lateral Sclerosis

Eligibility

Sex: ALLMin age: 35 Years

Medical Language ↔ Plain English

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

Locations (17)

Haukeland University Hospital

Bergen, Norway

Nordlandssykehuset HF

Bodø, Norway

Vestre Viken HF

Drammen, Norway

Helse Førde HF

Førde, Norway

Helse Fonna HF

Haugesund, Norway

Sørlandet sykehus

Kristiansand, Norway

Sykehuset Innlandet HF

Lillehammer, Norway

Akershus University Hospital

Lørenskog, Norway

Helse Møre og Romsdal

Molde, Norway

Helse Nord-Trøndelag HF

Namsos, Norway

...and 7 more locations

Outcomes

Primary Outcomes

Incidence of adverse events

Studies of adverse events including changes in lab parameters induced by high dose oral NR/pterostilbene.

Time frame: Through study completion, 1 year

Secondary Outcomes

Disease progression as assessed by Amyotrophic Lateral Sclerosis Functional Rating Scale Revised (ALSFRS-R)

ALSFRS-R is a validated rating instrument for monitoring the progression of disability in patients with ALS. The minimum score is 0 and the maximum score is 48, the higher score the more function is retained.

Time frame: Determined after 12 months of follow-up.

Change in vital capacity

Vital capacity measured by spirometry.