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ALS is a devastating, progressive neuromuscular condition also known as Lou Gehrig’s disease or amyotrophic lateral sclerosis in the United States and Canada. It's usually called motor neuron disease or MND in other English-speaking countries

ALS - devastating, progressive neuromuscular condition

ALS is a devastating, progressive neuromuscular condition also known as Lou Gehrig’s disease or amyotrophic lateral sclerosis in the United States and Canada. It's usually called motor neuron disease or MND in other English-speaking countries. The term amyotrophic lateral sclerosis is a combination of Greek words:

  • A, meaning without,
  • Myo-, referring to muscle,
  • Trophic, referring to nourishment,
  • Lateral, referring to nerves branching out of the spinal cord, and
  • Sclerosis, referring to scar formation or hardening.


Amyotrophic lateral sclerosis occurs when the nerves branching out of the spinal cord are insufficiently nourished and they grow hard, encased in scar tissue.

The symptoms of ALS are gradual, almost unnoticeable at first. Typically, the disease first affects just one hand or one foot. Then it "moves" up to the upper arm and shoulder or the knee and thigh. As the condition becomes more generalized, there will be falling, tripping, and difficulty holding or picking up objects. At this point, a range of symptoms sets in:

  • Muscle weakness
  • Muscle cramps
  • Muscle stiffness
  • Twitching (called fasciculations)
  • Changes in the timbre and pitch of the voice
  • Hoarseness
  • Slow speech, slurred speech
  • Swelling in the feet, ankles, and legs
  • Difficulty swallowing
  • Difficulty holding urine
  • Difficulty breathing


ALS begins in the muscles that are under voluntary control, such as the hands and feet. It progresses to the muscles that are under involuntary control, such as the muscles that control breathing. ALS is a particularly cruel disease in that the harder people who have ALS try to use their muscles, the more muscle tissue breaks down.

When this process only occurs in the neurons in the lower body, it is known as progressive muscle atrophy, or PMA. Usually PMA progresses to ALS, but these patients may have many more years, even 20 to 30 years, and much more mobility. ALS is usually the result of a gene inherited from at least one parent, but PMA is usually the result of a genetic mutation. Certain families in Guam have a form of ALS that imitates Parkinson's disease, and certain families in the United Kingdom have a form of ALS called familial British dementia.
 

What causes the symptoms of ALS?

ALS has long been known to be associated with an excess of a neurotransmitter called glutamate. This chemical essentially clogs the gaps between nerve cells so that they cannot transmit messages to each other. As glutamate builds up, the nerves eventually die.

For many years, this observation led researchers to try to explain the disease in terms of defects in antioxidant defense. Something in the nerve cells affected by ALS caused them to be unable to defend their DNA against free radical damage, or at least so researchers believed.

This observation actually is of practical value in treating ALS. There are indications that avoiding foods and food additives that are extremely high in glutamate, such as MSG and aspartame, may actually slow down the progress of symptoms. The most recent research, however, has found that ALS is due to a defect in the ability of DNA to transcribe its instructions onto the RNA that codes specific proteins in the nerve cell.

Nerve cells lose their ability to make the enzymes that regulate the energy-producing mitochondria, growth factors fail to be formed, and "suicide genes" are activated, along with the accumulation of toxic levels of glutamate. Most scientists now believe, however, that the defective gene precedes the accumulation of glutamate and free radical damage, rather than glutamate and free radical damage causing the disease.
 

Natural treatment for Amyotrophic Lateral Sclerosis?

No nutritional supplement or combination of nutritional supplements is clearly helpful in treating ALS. There was  some hope that taking the branched-chain amino acids (L-leucine, L-isoleucine, and L-valine) might be helpful, but later studies suggest they could actually do harm.

In the first study, physician scientists at the Mount Sinai School of Medicine in New York gave  early-stage ALS patients a cocktail of L-isoleucine, L-leucine, and L-valine. Taking the amino acid supplements seemed to be very helpful in maintaining strength in the feet and hands and in helping patients continue to be able to walk. A follow-up study at the University of Vermont College of Medicine, however, found that the same combination (and also supplementation with the amino acid L-threonine) actually accelerated loss of the ability to breathe, bringing death nearly 2-1/2 times faster. Until this contradiction can be resolved, it is not a good idea to try to treat ALS with nutritional supplements. Unfortunately, there is also no evidence proving the value of treating ALS patients with creatine, coenzyme Q10, genistein, vitamin B12, or guanidine.
 

Pharmaceutical treatment for ALS

The only medication that is known to extend life in ALS is riluzole, a drug that combats the accumulation of glutamate. If it is begun earlier in the course of the disease, it slows the progress of symptoms enough probably to extend life 2 or 3 months. It is usually not practical by the time ALS has caused loss of mental functions, and it is not equally effective in all racial groups. Three studies in North America found benefit in taking this medication, but a study in Japan did not.

Other medical treatments make symptoms more bearable. Since people who have ALS cannot swallow saliva, they may be given Botox to stop salivation. Many people with ALS can be given breathing assistance that does not require a tracheotomy, a cut in the throat in which to insert an oxygen tube. And various kinds of feeding assistance can prevent malnutrition in many cases.
 

Read More: Diagnosed with ALS: Frequently Asked Questions

Is there hope for people with ALS?

Despite the usually gloomy prognosis, there actually are people—notably the famed British physicist Richard Hawking—who live 20, 30, 40, and even 50 years with the disease. Some people with sporadic ALS have symptoms that get worse and get better, remitting and relapsing, for many years. And a few people continue to have some basic muscle functions many years after the onset of the disease.

 

  • ALS Association, Living with ALS Manuals.
  • Armon C. An evidence-based medicine approach to the evaluation of the role of exogenous risk factors in sporadic amyotrophic lateral sclerosis. Neuroepidemiology. Jul-Aug 2003.22(4):217-28.
  • Hasegawa M, Arai T, Akiyama H, et al. TDP-43 is deposited in the Guam parkinsonism-dementia complex brains. Brain. May 2007.130:1386-94
  • Neumann M, Sampathu DM, Kwong LK, et al. Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science. Oct 6 2006.314(5796):130-3.

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