Amyotrophic lateral sclerosis (ALS) is a rare neurodegenerative disease which attacks the nerve cells responsible for controlling voluntary muscle movement in the brain and spinal cord.
Voluntary muscles produce movements like swallowing, walking, chewing, and speaking.
The disease is progressive and the symptoms get worse with time. At present, there is no cure for ALS and no effective treatment to halt its progression.
The ALS Association evaluates that around 30,000 Americans are affected by this disease. As ALS progresses, it kills the nerve cells of the body. This results in weakened muscles, paralysis and ultimately, death. There is no complete cure.
What are the symptoms?
The onset of ALS can be so vague that the symptoms are usually ignored. But with time these symptoms develop into more noticeable weakness or atrophy which may cause a physician to suspect ALS. Some of the early signs and symptoms of ALS include;
- Difficulty in walking or doing normal activities of daily life
- Tripping and falling
- Weakness in legs, feet or ankles
- Hand weakness or clumsiness
- Unclear speech or trouble swallowing
- Muscle spasms and twitching in arms, shoulders, and tongue
- Difficulty keeping good posture or holding your head up
Often, ALS starts in the hands, feet or limbs, and then it spreads to other parts of the body. As the disease progresses and your nerve cells are destroyed, muscles progressively weaken.
This ultimately affects swallowing, chewing, talking and breathing. ALS doesn’t generally affect your bowel or bladder control, your senses or thinking ability.
Causes of ALS
The actual cause of ALS is unknown, and experts do not yet know why ALS attacks some people and not others. But, evidence from several scientific studies proposes that both genetics and environmental factors play a critical role in the development of ALS.
It is inherited in 5 to 10 percent of cases, while the rest have no identified cause. Possible causes of ALS are as follows;
- Gene mutation; several genetic mutations can cause inherited ALS. It causes almost the same symptoms as the noninherited form.
- Chemical imbalance; people with ALS usually have higher than normal glutamate level. Glutamate is a chemical messenger in the brain which is present around the nerve cells in their spinal fluid. Its high concentration is known to be lethal to some nerve cells.
- Disorganized immune response; sometimes the immune system of a person begins attacking some of its own normal cells. This may lead to the death of nerve cells.
- Protein mishandling; mishandled proteins present within the nerve cells may lead to a gradual accumulation of abnormal forms of these proteins in the cells. Thus, destroying the nerve cells.
As the disease develops, individuals with ALS experience many complications, which may include;
Most people with this disease will develop trouble talking over time. This generally starts as rare, mild slurring of words, but progresses to become more and more severe.
Speech ultimately becomes much difficult for others to understand. Furthermore, people with ALS often depend on other communication machinery to communicate.
Some people with this condition experience difficulties with memory and making decisions. Moreover, some are even diagnosed with a form of dementia called frontotemporal dementia.
People with ALS can also develop dehydration and malnutrition from damage to the muscles which control swallowing. They are also at greater risk of getting food, liquids or saliva into the lungs.
That can cause pneumonia. A feeding tube can help you in this regard as it can reduce these risks and ensure proper nutrition and hydration.
ALS paralyzes the muscles you use to breathe over time. Therefore, you may require a device to help you breathe at night.
For instance, you may be given constant positive airway pressure (CPAP) or bi-level positive airway pressure (BiPAP) to support your breathing at night.
Some individuals with advanced ALS pick to have a tracheostomy for full-time use of a respirator which inflates and deflates their lungs. Tracheostomy is a surgically created hole at the front of the neck leading to the windpipe.
Moreover, respiratory failure is the most common cause of death for people with ALS. On average, death occurs within 3-5 years after symptoms begin. But around 10% of patients will live for another 10 years or longer.