ALS Disease – Motor Neuron Disease

Amyotrophic lateral sclerosis or ALS disease is a motor neuron disease type attributable to the deterioration of neurons that are situated in the spinal cord’s ventral horn. ALS is a progressive disorder which can often degenerate one’s health and usually leads to death as it attacks both the nerves and the muscles of a person.

This is also known as Lou Gehrig’s disease, which came from the name of a New York Yankee slugger who died in 1941 because of this condition. This is an incurable disease characterized by weakness of the muscles, which could then result to paralysis.

This not only affects the body of a person but also speech, breathing and swallowing. Since ALS mainly attacks the motor neurons which are responsible for the voluntary muscles, the senses and the brain of the patients are not impaired. About 14 cases of this disease are diagnosed in the United States everyday.

Amyotrophic Lateral Sclerosis ALS Explained

Amyotrophic is from the Greek language where “A” means negative or no. “Myo” pertains to muscle and “trophic” denotes nourishment in the said language. Hence, “no muscle nourishment” is what ALS is about. When the muscles are not nourished, they waste away or atrophy. Lateral defines the areas in the spinal cord where parts of the nerve cells that take care of the nourishment of the muscles can be found. If the said area degenerates, scarring in that region will occur. Sclerosis is typically described as scarring.

The ALS disease is a motor neuron disorder which means that it easily affects the nerve cells which control the voluntary muscles in the body. The motor neurons are nerve cells that are located in the spinal cord and in the brain. When ALS develops, the muscles gradually deteriorate, causing them to weaken and become emaciated.

The patients of this disease will eventually be disabled as well as have difficulty in swallowing and in speaking. Often, they may succumb to infections specifically pneumonia. Even though the victims are paralyzed, they can still think clearly and can communicate but without speech. Mostly, they maintain control over their bladder and bowel movement. In some cases, the remaining abilities can be helpful to cope with the condition.

The most common form is sporadic ALS which affects anyone at any time. Other terms that are often used to categorize the classical variants of the ALS disease include progressive bulbar palsy, primary lateral sclerosis and spinal muscular atrophy.

Symptoms of ALS Disease

Initial signs that one has ALS disease may vary but they include stiffness of the muscles and increasing weakness especially the feet and the hands. The earliest symptoms of this disease are typically muscle atrophy and obvious weakness. Others include fasciculation or twitching of the muscles, cramps and/or stiffness of the affected parts, muscle weakness on a leg or an arm and/or nasal or slurred speech.

Depending on where the motor neurons are affected in the body, the early symptoms can be spotted there where they will be damaged first. Around 75% of the patients go through limb onset which is a form of ALS in which the first symptoms appear in the arms (upped limb) or legs (lower limb). Patients that experience leg onset will find it awkward to walk around or notice that they often stumble. They frequently have a dropped foot which pertains to the other foot that drags along the ground.

Meanwhile, arm onset includes difficulty with tasks that require manual handiness such as writing, buttoning a shirt and such. Bulbar onset is rare but not uncommon. Patients will first discover that they have difficulty in speaking clearly and/or swallowing food.

Who Can Have ALS Disease?

95% of the time, there are patients that do not have family history of the ALS disease. As a matter of fact, there is no known cause for the condition but there is a hereditary factor particularly in FALS or familial ALS. This happens when chromosome 21 (superoxide dismutase) is defective. This type of mutation is considered as autosomal dominant. A4V, the most common SOD1 mutation that causes the ALS disease in North America, can rapidly bring the patient from the onset stage to death.

The children whose parents have been diagnosed with familial ALS are exposed the development of the disease. However, those whose family members have been diagnosed with sporadic ALS disease are not often at risk with the condition. This suggests that there are environmental or non genetic factors that cause ALS disease.

Prolonged exposure to BMAA, a dietary neurotoxin may put a person to risk with the condition. BMAA is produced by cyanobacteria, which is one of the possible compounds that can be found in a tropical plant commonly found in Guam. This was used as food supply during 1950s up to early 1960s.

There were also a large number of soccer players in Italy who acquired the disease and this raised the concern that there is a link between the pesticides used in the field and the disease. In 2004, there was a study conducted that tried to connect the high incidence of the disorder with the use of performance enhancing drugs. But it was later found out that cyclists in the same region did not contract ALS disease even though they were also using such drugs. It was then concluded that it was possible that soccer players experience head trauma, collisions and falls compared to the cyclists who rarely fell and wore head protection.

Treatments for ALS Disease

The FDA has supported only one treatment for ALS disease and it is Rilutek or Riluzole. This is believed to have the ability to reduce damage to our motor neurons. What this drug treatment does is that it decreases the release of glutamate by means of activating the glutamate transporters.

Riluzole also offers other effects that can protect the neurons using calcium and sodium channel blockades, NMDA receptor antagonism promotion and protein kinase C inhibition. According to clinical tests, the drug can help the patient survive for several months and may help fight the bulbar ALS disease. However, it is important to note that the drug will not be able to reverse the motor neuron damage that has already been done to the body. Also, patients who take the drug should be monitored for the liver malfunctioning which appears about 10% in the victims. There are also side effects but Riluzole is the first step towards progression in fighting ALS disease.

There are also other treatments that can relieve the patient from the symptoms and can also improve the life of the patients while they are battling with the ALS disease. Physicians can prescribe drugs to reduce fatigue, muscle cramps, spasm and reduce phlegm and excessive salivation. These medications can also help patients fight depression, pain and sleep disturbances.

Medical Breakthroughs in Amyotrophic ALS Disease

Clinical trials are in progress around the world. KNS-760704 is being investigated and tested in ALS patients in hopes of finding neuroprotective effects. The said medication is an enantiomer of pramipexole that is used in treating Parkinson’s disease as well as with restless legs syndrome. Olesoxime or TRO19622 is being investigated also. This is a molecule that has structure comparable to cholesterol and shows that it has strong neuroprotective elements. This continuing clinical study intends to check the efficacy, tolerability, safety and the plasma levels of the ALS disease patients.

The response of the patients are monitored everyday to see if a single dose of two capsules can improve their life and reduce pain or other symptoms. RNAi is a new discovery which has showed early signs that it can help treat ALS disease. This has been injected in lab rats to quickly shut off certain genes that can lead to the condition.

The recent findings demonstrate that even though ALS disease is fatal, there are possible ways that can help the patients diminish the symptoms and hopefully survive this ordeal.