This is an open label trial of a supplement called HMB in patients with ALS. The researchers are evaluating its safety and tolerability, as well as its ability to lower insulin-like growth-factor binding protein 7 (IGFBP7) and Neurofilament light chain levels (NFL) and to slow ALS Functional Rating Scale, Revised (ALSFRS-R) progression.
Male or female aged at least 18 years.
Sporadic or familial ALS diagnosed as per Gold Coast Criteria (37).
Patient is able to understand and express informed consent (in the opinion of the site investigator).
Patient is able to read and write English.
Actively or recently (within past 30 days) participating in another intervention trial.
Currently or recently (within 30 days) taking HMB
Prior side effects from HMB deemed to be significant by the investigator
Patient has a medical or psychiatric illness that could in the investigator's opinion interfere with the patient's ability to participate in this study.
Single group
β-hydroxy-β-methylbutyrate (HMB)
DrugOur source of HMB will be Life Extension's "Wellness Code Muscle Strength \& Restore Formula. The dose will be 3g daily, which has previously been shown to be safe and well-tolerated, and to reduce IGFBP7 blood levels in humans.
ALS Functional Rating Scale, Revised (ALSFRS-R)
A quickly administered (five minute) ordinal rating scale (ratings 0-4) used to determine patients' assessments of their capability and independence in 12 functional activities. All 12 activities are relevant to people living with ALS.The ALSFRS-R declines linearly with time over a wide range during the course of ALS and it has been validated for telephone use. The ALSFRS-R Range is 0-48. The more points, the better the person is functioning.
Speech (Listener Effort)
Listener effort (LE) is a perceptual rating of the amount of work necessary for a listener to understand speech, rated by an expert speech-language pathologist on a visual analog scale from 0 (easily understood) - 100 (unintelligible even with full effort) and it has proven to be one of the most robust overall measures of dysarthria.
Neurofilament Light Chain levels (blood)
Neurofilaments are neuron-specific components of the cytoskeleton. They exist in heavy, medium, and light chain forms. Neurofilament light chain levels are elevated in the spinal fluid and the blood of patients with ALS and other neurodegenerative diseases, and higher levels predict more severe disease progression.
Neurofilament Light Chain levels (Cerebrospinal fluid (CSF))
Neurofilaments are neuron-specific components of the cytoskeleton. They exist in heavy, medium, and light chain forms. Neurofilament light chain levels are elevated in the spinal fluid and the blood of patients with ALS and other neurodegenerative diseases, and higher levels predict more severe disease progression.
Insulin-like growth-factor binding protein 7 (IGFBP7) levels (blood)
Non-competitive inhibitor of the IGF-1 receptor
Insulin-like growth-factor binding protein 7 (IGFBP7) levels (Cerebrospinal fluid (CSF))
Non-competitive inhibitor of the IGF-1 receptor
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Duke University
Lead sponsor
Temple University
Collaborator
This trial is not recruiting at the moment. You can still explore other options:
