Evaluating the Potential of the Diet as Disease Modifier in Amyotrophic Lateral Sclerosis (JERN_ALS)
Condition: Amyotrophic Lateral Sclerosis; Dietary Habits; Disorder of Fatty Acid Metabolism
MeSH terms: Motor Neuron Disease; Amyotrophic Lateral Sclerosis; Sclerosis
Study type: Observational

Summary: 
      The proposed observational trial will collect substantial data concerning dietary intake
      documented by ALS patients complemented by the analysis of fatty acid distribution in
      erythrocyte lipids. Both data sets are related to disease status and progress.
    

Detailed description: 
      Amyotrophic lateral sclerosis (ALS) is a currently incurable, multifactorial motor neuron
      disease characterized by upper and lower motor neuron degeneration, skeletal muscle atrophy,
      paralysis, and death. Multiple mechanisms caused by genetic and environmental factors
      proposed as responsible for ALS pathogenesis include decreased availability to neurotrophic
      factors, disturbances in calcium metabolism, increased neuroinflammatory status, cytoskeletal
      changes, mitochondrial dysfunction, dysfunction of protein degradation, glutamate
      excitotoxicity, apoptosis and oxidative stress.

      Currently, the only available drug to treat ALS is riluzole which slightly prolongs life.
      Nutritional management has become more important in the treatment of ALS because body mass
      index and nutritional status seems to be independent, prognostic factors for survival and
      disease complications. Malnutrition is common in ALS, so caloric supplementation is
      essential. Additionally, many ALS patients self-medicate with vitamins, herbs, and other
      dietary supplements.

      The objective of the current project is establishing the link between nutritional intake and
      disease status and progress. In detail, we like to assess if the improved outcomes are
      associated with specific nutrients, or simply the provision of excess calories. In this
      context, one of the most promising dietary candidates are polyunsaturated fatty acids (PUFA)
      and in particular the long-chain n-3 PUFA docosahexaenoic acid. This important structural
      component in neuronal membranes plays a role in neurogenesis and neuroprotection as well as
      exerts well-described anti-inflammatory effects in the brain.

      The proposed observational trial will collect substantial data concerning dietary intake
      documented by ALS patients (Food Frequency Protocols, FFPs, periodic over 5 days) combined
      with the analysis of fatty acid distribution in erythrocyte lipids which reflects fatty acid
      distribution of the consumed fatty or oily foods (time period: approximately the last 2-3
      months).

      The fatty aids distribution in erythrocyte lipids as well as the nutrient intake calculated
      by FFPs are related to disease status and progress.

      Thus, the current research activities focus on identification of dietary factors that are
      associated with disease progress or survival to develop beneficial interventions and therapy
      options.
    

Observational model: Case-Only
Time perspective: Prospective
Sampling method: Non-Probability Sample
Study population: 
        ALS patients diagnosed according to El Escorial / Awaji criteria for ALS
      
Biospecimen description: 
      Blood sampling every six months (1x 9mL Sarstedt monovette)
    
Keywords: fatty acids, amyotrophic lateral sclerosis, nutrient status