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).

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

Data will be available beginning 3 months and ending 5 years following article publication. Data will be shard with researchers who provide a methodologically sound proposal. Data will be shared for analyses to achieve the aims in the approved proposal. Proposals should be directed to johannes.dorst@uni-ulm.de; to gain access, data requestors will need to sign a data acess agreement. The study protocol will be available for 5 years at htpps://www.uniklinik-ulm.de/neurologie.html.

3 month after publication until 5 years after publication

Data will be shared with researchers who provide a methodologically sound proposal. Data will be shared for analyses to achieve the aims in the approved proposal. Proposals should be directed to johannes.dorst@uni-ulm.de; to gain access, data requestors will need to sign a data acess agreement.