Evaluating the Potential of the Diet as Disease Modifier in Amyotrophic Lateral Sclerosis (JERN_ALS) (JERN_ALS)

Observational Study for Evaluating the Potential of Diet and Food Components as Disease Modifiers in Amyotrophic Lateral Sclerosis (JERN_ALS)

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.

Study Overview

• Status: Completed
• Conditions: Amyotrophic Lateral Sclerosis; Dietary Habits; Disorder of Fatty Acid Metabolism

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.

• Study Type: Observational
• Enrollment (Actual): 50

Contacts and Locations

Study Locations

• Germany
  • Thuringia
    • Jena, Thuringia, Germany, 07743
      • Jena University Hospital, Hans Berger Department of Neurology

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years to 90 years (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

ALS patients diagnosed according to El Escorial / Awaji criteria for ALS

Description

Inclusion Criteria:

• ALS patients diagnosed according to El Escorial / Awaji criteria for ALS
• patients should have the ability to comprehend the full nature and purpose of the study, to cooperate with the investigator, to understand verbal and written instructions, and to comply with the requirements of the entire study.

Exclusion Criteria:

• patient's request or if patient compliance with the study protocol is doubtful

Study Plan

How is the study designed?

Design Details

• Observational Models: Case-Only
• Time Perspectives: Prospective

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Clinical neurological examination	ALS functional rating scale-revised (ALSFRS-R) - the parameter will be assessed every three months through study completion	through study completion, an average of 1 year

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Nutrient intake by diet	Food Frequency Protocol (FFP) over 5 days before blood sampling (FFP originate from Prodi® 6.4 software; Nutri-Science GmbH, Freiburg, Germany). Data analysis via Prodi®; calculation food nutrient profiles originated from the 'Bundeslebensmittelschlüssel'; calculation of daily energy intake, intake of fat and fatty acids, carbohydrates (individual sugars, dietary fibres), protein and amino acids, vitamins and minerals, sterols, etc.; the parameter will be assessed every three months through study completion	through study completion, an average of 1 year
Fatty acid distribution in erythrocyte lipids	marker for the fatty acid distribution of the consumed fatty or oily foods; reflect the fatty acid distribution of the consumed fatty or oily foods over the last 2-3 months as well as the endogenous metabolism and conversion of fatty acids; the parameter will be assessed every six months through study completion	through study completion, an average of 1 year
Blood lipids	Total cholesterol, HDL-cholesterol, LCL-cholesterol, triacylglycerides - the parameter will be assessed every six months through study completion	through study completion, an average of 1 year
Inflammatory parameter	c-reactive protein - the parameter will be assessed every six months through study completion	through study completion, an average of 1 year
Metabolic serum parameters	glycated hemoglobin HbA1C - the parameter will be assessed every six months through study completion	through study completion, an average of 1 year
Clinical neurological examination, part II	EQ5D-5L (population based QoL questionnaire) - the parameter will be assessed every six months through study completion	through study completion, an average of 1 year

Collaborators and Investigators

• Sponsor: University of Jena
• Collaborators: Jena University Hospital
• Investigators: Principal Investigator: Julian Grosskreutz, PhD, Jena University Hospital, Hans Berger Department of Neurology; Principal Investigator: Christine Dawczynski, PhD, University of Jena, Department of Nutritional Biochemistry and Physiology; Principal Investigator: Stefan Lorkowski, Professor, University of Jena, Department of Nutritional Biochemistry and Physiology

Study record dates

Study Major Dates

• Study Start (Actual): October 1, 2015
• Primary Completion (Actual): October 1, 2018
• Study Completion (Actual): October 1, 2018

Study Registration Dates

• First Submitted: October 6, 2015
• First Submitted That Met QC Criteria: October 7, 2015
• First Posted (Estimate): October 9, 2015

Study Record Updates

• Last Update Posted (Actual): March 7, 2019
• Last Update Submitted That Met QC Criteria: March 6, 2019
• Last Verified: September 1, 2015

More Information

Terms related to this study

• Keywords: fatty acids, amyotrophic lateral sclerosis, nutrient status
• Additional Relevant MeSH Terms: Pathologic Processes; Metabolic Diseases; Central Nervous System Diseases; Nervous System Diseases; Neuromuscular Diseases; Neurodegenerative Diseases; Spinal Cord Diseases; TDP-43 Proteinopathies; Proteostasis Deficiencies; Sclerosis; Motor Neuron Disease; Amyotrophic Lateral Sclerosis
• Other Study ID Numbers: H01_15

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No
• product manufactured in and exported from the U.S.: No