Safety and Tolerability of the Ketogenic Diet in Amyotrophic Lateral Sclerosis (ALS)

Study Overview
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Brief Summary
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This research is being done to see if the ketogenic diet (which is high in fat and low in carbohydrates) is safe and tolerable in amyotrophic lateral sclerosis (ALS) patients who are fed through a gastrostomy tube. This is not a study to see if ketogenic diets are effective in the treatment of ALS.

Official Title
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Safety and Tolerability of the Ketogenic Diet in ALS

Conditions
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Amyotrophic Lateral Sclerosis

Intervention / Treatment
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* Dietary Supplement: KetoCal


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Familial or sporadic ALS diagnosed as probable, laboratory-supported probable or definite according to the World Federation of Neurology El Escorial criteria Age 18 or older Capable of providing informed consent and complying with trial procedures Gastrostomy tube in place for the prior month Appel ALS score less than 100 Able to stand on a scale with assistance For patients with Appel ALS scores greater than 80, availability of caregiver who is willing and able to: Prepare, administer and log tube feeds Check and log gastric residuals Assist with weighing subject at home if necessary Willing to chart food intake during the six-month study Patients either not taking Riluzole (Rilutek) or Minocycline or on a stable dose of these for 30 days Not taking Coenzyme Q10 or on a stable dose and brand for 30 days Absence of exclusion criteria Exclusion Criteria: Forced vital capacity <50% of predicted Dependence on mechanical ventilation for more than 12 hours per day Exposure to any experimental agent within 30 days of onset of this protocol Women who are pregnant or planning to become pregnant Women of childbearing potential not practicing contraception Enrollment in another research study within 30 days of or during this trial Mini-Mental State Exam (MMSE) score <20 Patients with symptomatic cardiac disease or hypercholesterolemia Patients with myocardial infarction within 6 months of this trial Renal dysfunction defined as BUN and creatinine >2XULN Known mitochondrial disease BMI<18.5 Prior use of a 4:1 ketogenic diet or Atkins diet within 1 month of this trial Impaired liver function, defined as AST or ALT of 3 X ULN Patients who have a pacemaker or other internal electronic medical device

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
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: KetoCal<br>KetoCal tube feeding formula | Dietary Supplement: KetoCal<br>* Ketogenic Diet food via gastrostomy tube - 80% fat, 17% protein, 3% carbohydrates<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Study will determine if humans with ALS fed a strictly controlled diet designed to generate large amounts of ketones is safe and well-tolerated without evidence of weight loss or other adverse effect |  | 28 weeks | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Evaluate changes in motor function, strength, fatigue, body fat and cognitive function |  | 28 weeks | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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ALS, Ketogenic Diet