Amyotrophic Lateral Sclerosis (ALS) or Lou Gehrig’s disease is a progressive, degenerative condition which affects nerve cells called Motor Neurons responsible for control of voluntary muscles. The disease is associated with stiff muscles, muscle atrophy, muscle twitching, steadily declining strength, and worsening disability in most cases leading to death. Symptoms may start in one limb, with problems speaking or swallowing, or more rarely, with problems breathing.
Approximately 5 to 10% of ALS cases are directly inherited from the parents with the remaining 90 to 95% considered sporadic. In the sporadic cases, there appears to be a complex interaction between environment and genes that contributes to the development of ALS, severity of symptoms at initial diagnosis, and speed of decline.
The median survival of with ALS is 32 months from onset of symptoms and 19 months from diagnosis. The 5-year survival after diagnosis is 7%. Although the U.S. Food and Drug Administration recently approved edavarone to treat patients with amyotrophic lateral sclerosis the history of clinical trials to identify effective drugs for the treatment of the condition has a poor track record with more than 50 randomized controlled trials that failed to show positive results. This is only the second drug in 22 years to be approved in the United States, the first being riluzole. The efficacy of edavarone, a drug which must be administered intravenously, is based on its ability to slow the decline in the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) over a period of 24 weeks. The study did not, on the other hand, report on survival. Riluzole, by contrast, approved in the U.S. in 1995, was found to be modestly effective in prolonging survival for patients with ALS, but only by about two months. There was no data on quality of life, but patients treated with riluzole remained in a more moderately affected health state significantly longer than placebo-treated patients.
The idea that environmental or lifestyle factors appear to impact the risk of ALS or its prognosis is not new. Environmental risk factors include smoking, previous exposure to heavy metals (mercury and lead), pesticides and other organophosphates, organic solvents, a history of electrical shock, traumatic brain injury, and physical injury. Evidence for fungal infection in cerebrospinal fluid and brain tissue has been reported. One study found that imbalances in intestinal microbiome had a strong association with ALS. These findings are consistent with factors related to diet that influence the risk of developing ALS and the speed of the decline. Diets higher in vegetable and fruit intake and in omega-3 fatty rich foods lower the risk of developing ALS and slow the speed of the decline. Other factors associated with longer survival include better physical health and the presence of a marital partner.
In addition, autoimmune processes have been identified as a potential factor in the development of ALS. There is a case report of an individual who was initially believed to have a motor neuron disease, possibly ALS, whose symptoms, physical findings, and laboratory findings regressed over a 23-month period while following a gluten-free diet. Another study found that a higher percent of individuals with ALS (59.1%) than controls (28.6%) had HLA-specific alleles that increase the risk of developing Celiac disease. All controls had no evidence for IgA antibodies to transglutaminase and/or gliadin (antibodies associated with Celiac disease or gluten sensitivity), while 15.3% of the ALS patients did have elevated auto-antibodies, suggesting that gluten sensitivity may be a factor for some individuals with ALS. Other autoimmune diagnoses and processes have been noted in the presence of ALS. Autoimmune diagnoses that have preceded the diagnosis of ALS include asthma, celiac disease, myasthenia gravis, polymyositis, Sjögren’s syndrome, ulcerative colitis, and systemic lupus erythematosus.
Individuals with ALS are frequently malnourished and may have reduced energy and nutrient intakes. An elevated energy expenditure which is seen in about half of patients with ALS may also contribute to negative energy balance and weight loss. Individuals who have a lower weight prior to diagnosis or experience significant weight loss and reduction in lean mass may have a poorer prognosis. Thus, maintaining adequate nutrition to preserve body weight and muscle mass may be important treatment considerations in ALS.
A functional medicine approach to ALS, based on these observations, makes a lot of sense. Furthermore, targeted correction of environmental imbalances may have additional impact on the natural history of this near universally fatal illness. The clinical framework that combines diet and lifestyle factors with correction of environmental factors to impact chronic disease is known as functional medicine. To accomplish this goal the Functional Medicine operating system consists of the Functional Medicine Timeline, The Functional Medicine Matrix, and the Therapeutic Lifestyle Factors (Sleep & Relaxation, Movement & Exercise, Nutrition, Stress, and Relationships). Functional Medicine Matrix, based on a scientific framework known as “systems biology,” allows the practitioner to evaluate imbalances at the cellular level. This helps sort out why the disease has occurred in the first place. By understanding each of these imbalances the patient is empowered to make changes to correct them. Each part of the Matrix is called a “node,” and there are 7 nodes on the Functional Medicine Matrix. You can read about them here: Assimilation, Defense & Repair, Energy, Biotransformation & Elimination, Structural Integrity, Transport, and Communication.