Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease, is a fatal disease due to progressive degeneration of nerve cells in the spinal cord and brain. Currently, treatment options for the condition are extremely limited. A new study has found that a high calorie, high carbohydrate diet may slow the progression of the disease. The findings were published on February 28 in The Lancet.
As the disease progresses, ALS patients with ALS develop muscle weakness and atrophy, which eventually leading to difficulty swallowing or chewing, paralysis, and usually death from respiratory failure. Many ALS patients lose the ability to eat on their own; therefore, they frequently require a feeding tube and lose a significant amount of weight. As the weight loss progresses, the patients start burning their own muscle. The study authors note that mild obesity is associated with greater survival in these patients; in addition, high calorie diets were reported to increase survival in a mouse model. Therefore, the goal of the study was to evaluate the safety and tolerability of two hypercaloric diets in ALS patients.
The study group comprised 24 adults who were enrolled from December 14, 2009 through November 2, 2012. Eligible participants were aged 18 years or older with no history of diabetes or liver or cardiovascular disease, and who were already receiving percutaneous enteral nutrition (feeding tube from an opening in the skin to the stomach). The participants were randomly assigned to one of three dietary interventions: replacement calories using an isocaloric (normal amount of calories) tube-fed diet (control), a high-carbohydrate hypercaloric tube-fed diet (HC/HC), or a high-fat hypercaloric tube-fed diet (HF/HC). Participants received the diets for four months and were followed-up for five months. The primary outcome measurements were safety and tolerability.
Among the 24 enrollees, 20 began their study diet (6 in the control group, 8 in the HC/HC group, and 6 in the HF/HC group). One patient in the control group, 1 in the HC/HC group, and 2 in the HF/HC group withdrew their consent before receiving the dietary intervention. The patients who received the HC/HC diet had a smaller total number of adverse events than did those in the other groups (23 adverse events in the HC/HC group vs. 42 in the control group vs. 48 in the HF/HC group) and significantly fewer serious adverse events than did those on the control diet (none vs. nine; p=0•0005). Fewer patients in the HC/HC group discontinued their study diet due to adverse events (0 of 8 in the HC/HC group vs. 3 of 6 in the control group). During the five month follow-up, no deaths occurred in the 9 patients assigned to the HC/HC diet compared with 3 deaths in the 7patients assigned to the control diet. Adverse events, tolerability, deaths, and disease progression did not differ significantly between the HF/HC group and the control group.
The researchers concluded that their findings provide preliminary evidence that hypercaloric enteral nutrition is safe and tolerable in patients with ALS, and support the study of nutritional interventions in larger randomized controlled trials at earlier stages of the disease.
The authors are affiliated with Harvard Medical School (Boston, MA), Massachusetts General Hospital (Boston, MA), Emory University School of Medicine (Atlanta, GA), University of Vermont (Burlington, VT), Methodist Neurological Institute (Houston, TX), Carolinas Medical Center Neuromuscular/ALS-MDA Center (Charlotte, NC), Saint Mary's Health Care (Grand Rapids, MI), Columbia Presbyterian Medical Center (New York, NY), University of California at Irvine (Irvine, CA), Sarasota Memorial Hospital (Sarasota, FL), Barrow Neurological Institute/St Joseph's Hospital and Medical Center (Phoenix, AZ), Drexel University (Philadelphia, PA), University of California at San Francisco (San Francisco, CA), and Oregon Health and Science University (Portland, OR).