Safety and Tolerability Ultra-high-caloric Food Supplements in Amyotrophic Lateral Sclerosis (ALS)

Study Overview
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Brief Summary
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The LIPCAL-ALS study (NCT02306590) has provided preliminary evidence that a high-caloric nutrition might prolong survival in fast-progressing ALS patients. Since increasing the amount of calories of the intervention might possibly increase the beneficial effect, the investigators seek to investigate whether an ultra-high caloric diet (UHCD), featuring the double amount of calories compared to LIPCAL-ALS, will be well tolerated by ALS patients and may serve as an intervention for a potential LIPCALII study. For this purpose, the investigators will compare two different UHCDs (one fat-rich and one carbohydrate-rich) with regard to safety and tolerability over a time frame of 4 weeks. A third group will receive the original diet from LIPCAL, and a fourth group will receive no intervention (control group).

Official Title
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Safety and Tolerability of Fat-rich vs. Carbohydrate-rich High-caloric Food Supplements in Patients With Amyotrophic Lateral Sclerosis (ALS)

Conditions
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Amyotrophic Lateral Sclerosis

Intervention / Treatment
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* Dietary Supplement: high-caloric fatty diet
* Dietary Supplement: ultra-high-caloric fatty diet
* Dietary Supplement: ultra-high-caloric carbohydrate-rich diet


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Diagnosis of Amyotrophic Lateral Sclerosis (ALS) according to the revised version of the El Escorial criteria (Ludolph et al. 2015) Slope of ALS Functional Rating Scale Revised (ALSFRS-R) of >0.25 points per month at baseline visit based on the formula (48 - score at baseline visit) / (time between date of first symptom and baseline visit) stable on standard therapy riluzole (100 mg/day) for at least 4 weeks capable of thoroughly understanding all information given and giving full informed consent according to good clinical practice (GCP) Exclusion Criteria: already taking any dietary supplements participation in another clinical trial within the preceding 8 weeks tracheostomy or assisted ventilation of any type which exceeds 23 hours per day pregnancy or breast-feeding females

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Treatment
Allocation: Randomized
Intervention Model: Parallel Assignment
Masking: Single


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Active Comparator: high-caloric fatty diet<br>intake of 405 kcal (45g fat) per day in addition to normal food intake | Dietary Supplement: high-caloric fatty diet<br>* see arm/group description<br>|
| Experimental: ultra-high-caloric fatty diet<br>intake of 810 kcal (90g fat) per day in addition to normal food intake | Dietary Supplement: ultra-high-caloric fatty diet<br>* see arm/group description<br>|
| Experimental: ultra-high-caloric carbohydrate-rich diet<br>intake of 900 kcal (111.4g carbohydrate, 34.9g fat, 36.0g protein) in addition to normal food intake | Dietary Supplement: ultra-high-caloric carbohydrate-rich diet<br>* see arm/group description<br>|
| No Intervention: control<br>normal food intake (no intervention) | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Adverse Events and Serious Adverse Events | Incidence of Adverse Events and Serious Adverse Events | 4 weeks | 
| Laboratory values | Incidence of abnormalities in clinical laboratory assessments, vital signs and physical examinations | 4 weeks | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Appetite | Change of Appetite-Score (Council of Nutrition appetite questionnaire, CNAQ) compared to baseline; values 8-40; higher values mean better appetite | 2 weeks; 4 weeks | 
| Eating Habits | Change in eating habits as evaluated by a standardized questionnaire (Ulmer eating habits questionnaire); descriptive information and evaluation; no overall score | 2 weeks; 4 weeks | 
| Taste of Intervention | taste of intervention (visual analogous scale); values 1-9, higher values mean better taste | 2 weeks; 4 weeks | 
| Body Weight | change of body weight compared to baseline | 2 weeks; 4 weeks | 
| Biomarkers | change of neurofilament light chains (NfL) in serum | 4 weeks | 
| Adverse Events and Serious Adverse Events | Incidence of Adverse Events and Serious Adverse Events (evaluation via phone call 2 weeks after intervention was finished) | 6 weeks | 
| ALSFRS-R | change of Amyotrophic Lateral Sclerosis Functional Rating Scale Revised (ALSFRS-R) compared to baseline; values 0-48; higher values mean less impairment | 4 weeks |