Amyotrophic lateral sclerosis, a type of motor neuron disease refers to a group of progressive neurological diseases that cause dysfunction in the nerves that control muscle movement.
Commonly known as ALS disease, it leads to muscle weakness and changes the basic operations of the body. ALS disease can also affect the nerves that control breathing and can be fatal.
It is also called Lou Gehrig's disease after the famous baseball player who had the condition. The Centre for Disease Control and Prevention (CDC) estimates that between 14,500 and 15,000 people in the United States had ALS in 2016, with about 5,000 people receiving a diagnosis annually, medicalnewstoday.com said. It is believed to affect between two and five people in every 100,000, worldwide.
While there is no cure found out for ALS disease, treatment can relieve symptoms and improve quality of life. Most people with ALS live for 3 to 5 years after experiencing the symptoms but around 10 percent live for 10 years longer.
ALS affects the nerve cells in the brain and spinal cord leading to muscle weakness, a loss of motor function, paralysis, breathing problems, and eventually death.
As ALS progresses, the muscle cells degenerate and die, they stop sending messages to muscles, the brain can no longer control voluntary movement and the muscles weaken and waste away. It attacks the voluntary muscles as it advances where a person can no longer control their arms, face and legs and eventually lead to respiratory failure.
World-renowned physicist Stephen Hawking was diagnosed with ALS at the age of 21 years that gradually paralysed him over the decades. He used a speech-generating device to communicate after his loss of speech, Hawking died on March 14, 2018 at the age of 76, after living with the disease for more than 50 years.
Causes and Types:
ALS can be sporadic which occurs randomly and accounts for 90-95 percent of the cases. There is no clear risk factor or cause.
Familial ALS is inherited and accounts for 5-10 percent cases. The child of a person suffering from ALS will have a 50 percent chance of developing ALS. Scientists are still doing research on which genes are involved.
Other possible causes of ALS: Disorganized immune response: The immune system may attack some of the body's cells, possibly killing nerve cells. Chemical imbalance: People with ALS often have higher levels of glutamate, a chemical messenger in the brain, near the motor neurons. Glutamate in high quantities is known to be toxic to nerve cells. Mishandling of proteins: If proteins are not processed correctly by nerve cells, abnormal proteins could potentially accumulate and cause the nerve cells to die. Other factors include environmental factors such as mechanical or electrical trauma military service high levels of exercise high levels of agricultural chemicals high levels of a variety of heavy metals Symptoms:
The symptoms for the disease starts showing when a person is in their late 50s or early 60s. However, there have been cases where it has been detected at early ages.
Common symptoms include progressive muscle weakness, clumsiness, abnormal limb fatigue, muscle cramps and twitches, and slurred speech. The symptoms spread to all parts of the body as ALS progresses.
Some people may also have problems with decision-making and memory, eventually leading to a form of dementia called frontotemporal dementia.
Treatment and Prevention:
While there is no cure for ALS, treatment helps to alleviate symptoms, prevent unnecessary complications, and the slow rate of disease progression.
ALS can cause a range of physical, mental, and social changes, so a team of specialists will often help patients manage their symptoms and care, improve their quality of life, and prolong survival.
The US Food and Drug Administration in 1995 approved Riluzole (Rilutek), a drug produced to diagnose ALS disease which appears to slow the progression of the disease. It may work by reducing the body's levels of glutamate, an excitotoxin that has been linked to neuronal damage.In May 2017, Radicava (Edaravone) was approved to treat ALS. It may slow the decline in physical function by one third.