Amyotrophic lateral sclerosis

Non-pharmacologic aspects of ALS: correlation of higher BMI with greater survival, beneficial use of cannabidiol, respiratory evaluation, exercise and anesthetic considerations 

Virginia Thornley, M.D., Neurologist, Epileptologist

March 4, 2018

ALS is a progressive neurodegenerative illness which affects the lower motor neurons causing progressive weakness and eventually respiratory failure. There are currently only 2 prescription agents available for slowing the progression of the disease. Management is largely symptomatic treatment of debilitating symptoms.  Much consideration is being directed towards alternative treatments such as dietary considerations and use of cannabidiol.

ALS and supplements and BMI

Based on one large review of studies, there was not enough evidence to support recommendations of Vitamin A, and C or Coenzyme Q10. For Vitamin B complex, Homocysteine, Vitamin D, there is limited data which is not enough to support or refute recommendation, more clinical trials are needed. Vitamin E may be beneficial in preclinical patients with a familial tendency. Omega-3 was found to accelerate disease progression with increased vacuolization of anterior horn cells and are deleterious in presymptomatic patients. L-Carnitine may increase survival time with a slower ALSFRS or ALS functional revised score and greater FVC volumes but more trials are needed (1). Body mass index of less than 18.5kg/m is found with less survival time, while those with a higher BMI have greater survival time. One small study suggested a high calorie, hypercaloric enteral diet was tolerable in patients but unclear if associated with better outcome in terms of survival time.

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Cannabidiol as a novel therapeutic agent

Cannabidiol is known in animal models to be anti-inflammatory and anti-oxidant. In the G93A SOD-1 mouse model, it was found to slow progression of the disease and increase survival time. In a study of 13 patients, it reduced drooling, loss of appetite, pain, and spasticity.

In one study, cannabidiol was used in human gingiva-derived mesenchymal stromal cells. The transcriptomic sequence in the next generation shows a change in gene expression in ALS related genes. There was a change in the genes connected to ALS regarding oxidative stress, mitochondrial dysfunction and excitotoxicity in the human gingiva-derived mesenchymal stromal cells when treated with cannabidiol. This suggests that cannabidiol may serve as a modulatory role in the early pathogenesis of ALS (2).

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Other aspects of ALS

Exercise 

Studies regarding exercise showed flaws in methodology or were in small numbers. Therefore, based on available studies it is unclear if exercise benefits a patient’s long-term outcome.

 

Sialorrhea

Sialorrhea should be addressed. Aside from conventional medications, other options include botulinum toxin and small doses of radiation therapy delivered to the salivary glands.

Anesthesia 

Anesthetic agents such as neuromuscular blockers are contraindicated in patients with ALS due to the risk of increased potassium release. In one study of 51 patients with ALS, general anesthesia was safely administered.

Pulmonary function 

Pulmonary function tests are monitored every few months depending on the rapidity of the progression of the disease. Non-invasive positive pressure ventilation is used in patients with ALS with an oral or nasal mask. As force vital capacity (FVC) declines, it may be administered through a bi-level positive airway ventilation machine continuously at night(1).

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References

1. Karam, et al, “Palliative care issues in amyotrophic lateral sclerosis: an evidence-based review,” American Journal of Palliative Care, 2016, Feb., 33(1):84-92.

2. Rajan. et al, “Gingival stromal cells as an in vitro model: cannabidiol modulates genes linked with amyotrophic lateral sclerosis,” Journal of Cellular Biochemistry, 2017, Apr., 118(4):819-828.

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