Amyotrophic lateral sclerosis (ALS): What you should know

A type of motor neuron disease is amyotrophic lateral sclerosis. It refers to a group of progressive, neurological diseases that cause nerve damage that regulate movement of the muscles.

This leads to fatigue in the muscle and changes in how the body functions. Amyotrophic lateral sclerosis ( ALS) affects the nerves that regulate breathing in the later stages and this can be fatal.

The most prevalent form of motor neuron disease (MND) is ALS. After the famous baseball player who had the illness, it is also called Lou Gehrig ‘s disease. The Ice Bucket Challenge 2014 aimed to raise awareness and research support.

The Centers for Disease Control and Prevention ( CDC) reports that in 2016 in the United States (U.S.) between 14,500 and 15,000 individuals had ALS, with about 5,000 people getting a diagnosis per year. Around the world, between 2 and 5 people in every 100,000 are thought to be affected.

Most people with ALS will live after first having symptoms for 3 to 5 years, but about 10 percent of patients will live for another 10 years or more.

There is no cure, but treatment can alleviate symptoms and enhance quality of life.

Important facts about ALS

Here are some key points about ALS. More detail is in the main article.

  • ALS affects nerve cells in the brain and spinal cord, leading to muscle weakness, a loss of motor function, paralysis, breathing problems, and, eventually, death.
  • Most people with ALS will live between 3 and 5 years after symptoms appear.
  • The exact cause is unknown, but environmental and genetic factors may be involved.
  • There is currently no cure, and treatment aims to relieve symptoms, provide social and emotional support, and possibly slow disease progression.

What is ALS?

Adaptive equipment can help people with ALS to stay mobile.
Adaptive equipment can help people with ALS to stay mobile.

ALS is a kind of MND. It attacks the nerve cells , known as motor neurons, which are used in voluntary muscle actions. There are acts we can manage, like those in the arms, the face and the legs.

There are motor neurons in the brain and the spinal cord. As ALS goes on, those cells degenerate and die. They stop sending out muscle messages. The brain can’t control voluntary movement anymore and the muscles are weakening and wasting away.

As ALS progresses, all voluntary muscles are affected by this. The person can’t control his head, face and legs any more. In time, the unsupported inability to breathe will lead to respiratory failure.

Half of all people with ALS will live after diagnosis for 3 years or more but some will live longer. After diagnosis, about 20 per cent of people will live 5 years or more, 10 per cent will live 10 years or more, and 5 per cent will live 20 years.

Steven Hawking, the well-known physicist, was diagnosed with ALS, aged 21. Now well over 70 years old, he remains a leader in the field of science.

Causes and types of ALS

It’s not clear what exactly causes ALS. There are different types according to their signs and symptoms and whether a clear genetic association exists or not.

ALS can be sporadic or familial.

Sporadic ALS happens at random, comprising 90 to 95 percent of cases. There is no clear factor or cause for the risk.

Familial ALS is inherited. Approximately 5 to 10 percent of cases are relatives. A person’s child with ALS would have a 50 per cent chance to inherit the disorder. Rarely, a person in their teens can be affected. Researchers are looking into which genes are involved.

Other possible causes of ALS include:

  • 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.

Possible environmental factors

Environmental factors may also play a role.

One study found that military personnel deployed in the Gulf region during the war of 1991 were more likely than military personnel deployed elsewhere to grow ALS.

Some possible links have been found between ALS and exposure to:

  • mechanical or electrical trauma
  • military service
  • high levels of exercise
  • high levels of agricultural chemicals
  • high levels of a variety of heavy metals

However, there is no conclusive evidence that specific lifestyle changes can decrease the risk.

Signs and symptoms

ALS symptoms typically occur when a person is in their late fifties or early sixties, but it may happen at other ages.

Progression varies according to individuals. Signs and signs may be barely apparent in the early stages but over time the deficiency becomes more evident.

Common symptoms include:

  • difficulty carrying out daily activities, including walking
  • increased clumsiness
  • weakness in the feet, hands, legs, and ankles
  • cramping and twitching in the arms, shoulders, or tongue
  • difficulty maintaining good posture and holding the head up
  • uncontrolled outbursts of laughing or crying, known as emotional lability
  • cognitive changes
  • slurring of speech and difficulty with voice projection
  • pain
  • fatigue
  • problems with saliva, and mucus
  • difficulty breathing and swallowing, in the later stages

In all cases of ALS, progressive muscle weakening happens, but this may not be the first symptom of the disease.

Early signs can include clumsiness, excessive weakness in the arms, cramps and twitches in the muscles and slurred speech. Symptoms spread to every part of the body as ALS progresses.

Some people may have difficulties with memory and decision making, eventually progressing to a type of dementia called frontotemporal dementia.

Emotional lability can cause mood fluctuations and emotional response.

Treatment and prevention

There is no cure for ALS, but treatment aims at alleviating symptoms, avoiding needless complications and delaying progression of disease.

ALS can cause a variety of physical , mental and social changes, so patients will also be assisted by a team of clinicians to manage their symptoms and treatment, improve their quality of life, and extend survival.

The Food and Drug Administration ( FDA ) approved riluzole (Rilutek) for ALS care in 1995, and the progression of the disease seems to be slowing. It can function by decreasing glutamate levels in the body, an excitotoxin that has been associated with neuronal harm.

Radicava (Edaravone) had been approved for treatment of ALS in May 2017. It can delay one-third of the decline in physical function.

A variety of research studies are looking at ways to address various aspects of ALS using new and existing medications. Doctors can prescribe medicines to treat the various symptoms, too.

Therapy

Physical therapy can help people with ALS cope with pain and deal with mobility problems.

A physical therapist can provide help and information with:

  • low-impact exercises to enhance cardiovascular fitness and overall well-being
  • mobility aids, such as walkers and wheelchairs
  • devices to make life easier, such as ramps

Occupational therapy can help a patient maintain their independence for longer by:

  • helping patients choose adaptive equipment and assistive technologies to help them keep up their daily routines
  • train them in ways to compensate for hand and arm weaknesses

In time, respiratory therapy will be required, as the breathing muscles become weaker.

Breathing devices can help the patient breathe better at night. Mechanical ventilation may be needed in some patients. One end of a tube is attached to a respirator, while the other end is inserted through a surgically-created hole in the neck, or tracheostomy, into the windpipe.

Speech therapy is helpful when the ALS becomes more difficult to talk. Teaching functional strategies will benefit speech therapists. Other correspondence techniques include computer based networking devices and writing.

Nutritional support is significant, because it can be difficult to swallow to get enough nutrients. Nutritionists should advise on the preparation of more easily swallowing healthy meals. Suction equipment and feeding tubes may help.

Diagnosis

No single test can diagnose ALS, so the diagnosis is based on the symptoms and test results to rule out other disorders with similar symptoms.

Tests that may help diagnose ALS are:

  • electromyography (EMG), which detects electrical energy in muscles
  • nerve conduction study (NCS), which tests how well the nerves send signals

These tests may help rule out peripheral neuropathy or peripheral damage to the nerves, and myopathy or muscle disease.

An MRI scan can diagnose other symptom-causing conditions, such as a tumor in the spinal cord or a herniated neck disk.

Other tests can include blood and urine tests and a muscle biopsy to rule out any conditions.

HIV, Lyme disease, multiple sclerosis (MS), polio virus, and West Nile virus are medical conditions that may cause similar symptoms to ALS.

If both the upper and lower motor neurons exhibit signs, ALS may be present.

Symptoms of the upper motor neuron include stiffness and muscle movement resistance, and rapid reflexes. Lower motor neuron signs include fatigue, twitching and muscle atrophy.

Tips for living with ALS

A variety of tips can help individuals with ALS and their loved ones adapt to their changing situation.

Keep in touch: It ‘s important to have social interaction. Keep in touch with friends and keep up with as many events as possible beforehand. Local or online support groups can exist which can answer questions and provide guidance through shared experiences.

Be practical: Have a bag ready to go out with tissues, hand wipes and easy-to-hold cutlery. Register to get a car handicap placard. Make modifications at home, such as a toilet seat raising unit.

Prep ahead: When a loved one discovers out they can no longer do what they might do before, it can be hard to predict future limitations that will help you get ready for when they arrive.

Financial analysis help: Care can get costly as the disease progresses. Find out if you are eligible to support with the programs of Social Security Disabilities, Medicare , Medicaid and Veteran Affairs.

Arrange caregivers relief: Arrange for a friend, parent, or caregiver to come and stay for a weekend or take the person out for the day with ALS. Caregivers should ensure they take care of their own health and that of their loved one.

What you can do would rely somewhat on your circumstances, but through guidance or practical assistance, local support groups can help people deal with the financial and emotional difficulties associated with ALS.

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