Brain Function and Disorders

What is Amyotrophic Lateral Sclerosis Disease?

Last updated on October 20th, 2022 at 07:41 am

Amyotrophic lateral sclerosis (ALS) is the most common degenerative disease of the motor neuron system. Although ALS is incurable, with a median survival of 3 years, treatment can extend the length and meaningful quality of life for patients.

What is amyotrophic lateral sclerosis disease?

ALS is short for amyotrophic lateral sclerosis. You might also have heard it called Lou Gehrig’s disease, after the baseball player who was diagnosed with it in the 1930s. A French doctor named Jean-Martin Charcot discovered the condition in 1869.

ALS is a progressive disease, which means it gets worse. It affects nerves in your brain and the spinal cord that controls your muscles. As your muscles get weaker, it gets harder for you to walk, talk, eat, and breathe.

What are the main types of ALS?

There are two types of ALS;

  1. Sporadic ALS is the most common form. It affects up to 95% of people with the disease. Sporadic means it happens sometimes without an obvious cause.
  2. Familial ALS (FALS) runs in families. About 5% to 10% of people with ALS have this type. FALS is caused by changes to a gene. Parents pass the faulty gene to their children. If one parent has the gene for ALS, each of their children will have a 50% chance of getting the gene and having the disease.

Signs and symptoms of amyotrophic lateral sclerosis

The symptoms of ALS usually appear when a person is in their late 50s or early 60s, but it can happen at other ages.

Progression varies between individuals. In the early stages, signs and symptoms may be barely noticeable, but the weakness becomes more visible over time.

What are the symptoms of amyotrophic lateral sclerosis?

12 Common ALS symptoms
  1. difficulty carrying out daily activities, including walking
  2. increased clumsiness
  3. weakness in the feet, hands, legs, and ankles
  4. cramping and twitching in the arms, shoulders, or tongue
  5. difficulty maintaining good posture and holding the head up
  6. uncontrolled outbursts of laughing or crying, known as emotional lability
  7. cognitive changes
  8. slurring of speech and difficulty with voice projection,
  9. pain
  10. fatigue
  11. problems with saliva and mucus
  12. difficulty breathing and swallowing, in the later stages

Other symptoms

  • Progressive muscle weakness occurs in all cases of ALS, but this may not show the condition.
  • Early symptoms often include clumsiness, abnormal limb fatigue, muscle cramps and twitches, and slurred speech. Symptoms will spread to all parts of the body as ALS progresses.
  • Some people may have problems with decision-making and memory, eventually leading to a form of dementia called front temporal dementia.
  • Emotional lability can cause fluctuations in mood and emotional response.

What is a motor neuron?

Motor neurons of the spinal cord are part of the central nervous system (CNS) and connect to muscles, glands, and organs throughout the body.

ALS upper and lower motor neurons

ALS is a disease that affects your motor neurons. These nerve cells send messages from your brain to your spinal cord and then to your muscles.

You have two main types of Motor Neuron:

  1. Upper motor neurons: Nerve cells in the brain.
  2. Lower motor neurons: Nerve cells in the spinal cord.

These motor neurons control all your voluntary movements, the muscles in your arms, legs, and face. They tell your muscles to contract so you can walk, run, pick up your smartphone, chew and swallow food, and even breathe.

ALS is one of a few motor neuron diseases. Some others include:

  • primary lateral sclerosis (PLS)
  • progressive bulbar palsy (PBP),
  • pseudobulbar palsy

Where does amyotrophic lateral sclerosis affect the body?

With ALS, motor neurons in your brain and spinal cord break down and die. When this happens, your brain can’t send messages to your muscles anymore. Because the muscles don’t get any signals, they become very weak. This is called atrophy. In time, the muscles no longer work and you lose control over their movement.

At first, your muscles get weak or stiff. You may have more trouble with fine movements, such as trying to button a shirt or turn a key. You may stumble or fall more than usual. After a while, you can’t move your arms, legs, head, or body.

Eventually, people with ALS lose control of their diaphragm, the muscles in the chest that help you breathe. Then they can’t breathe on their own and will need to be on a breathing machine. Losing breathing causes many people with ALS to die within 3 to 5 years after they’re diagnosed. Yet some people can live over 10 years with the disease.

People with ALS can still think and learn. They have all of their senses, sight, smell, hearing, taste, and touch. Yet the disease can affect their memory and decision-making ability.

ALS isn’t curable. Yet scientists now know more about this disease than ever before. They are studying treatments in clinical trials.

What causes amyotrophic malarial parasite ?

Researchers still don’t know exactly what causes motor neurons to die with ALS. Gene changes, or mutations, are behind 5% to 10% of ALS cases. Over 12 different gene changes have been linked to ALS.

One change is to a gene that makes a protein called SOD1. This protein may be toxic to motor neurons. Other gene changes in ALS might also damage motor neurons.

The environment could also play a role in ALS. Scientists are studying whether people who come into contact with certain chemicals or germs are more likely to get the disease. For example, people who served in the military during the 1991 Gulf War have got ALS at higher rates than usual.

Scientists are also looking at these other probable causes;

Glutamate. This chemical signals and from the brain and nerves. It’s a type of neurotransmitter. With ALS, glutamate builds up in the spaces around nerve cells and may damage them. The medications riluzole (Rilutek) works by lowering glutamate levels and can help slow the development of the disease.

Immune system problems. Your immune system protects your body from foreign invaders such as bacteria and viruses. In your brain, microglia are the key type of immune cells. They destroy germs and damaged cells. With ALS, microglia might also destroy healthy motor neurons.

Mitochondria problems. Mitochondria are the parts of your cells where energy is made. A problem with them might lead to ALS or make an existing case worse.

Oxidative stress. Your cells use oxygen to make energy. Some of the oxygen your body uses to make energy may form into toxic substances called free radicals, which can damage cells. The medication edaravone (Radicava) is an antioxidant that can help control these free radicals.

Researchers learn more about ALS every day. What they discover will help them develop medications to treat symptoms and improve the lives of people who have this disease.

Amyotrophic lateral sclerosis diagnosis

ALS Diagnosis

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

Tests that may help diagnose ALS are:

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

These tests can help rule out peripheral neuropathy, or peripheral nerve damage, and myopathy, or muscle disease.

  1. An MRI scan can detect other problems that could cause symptoms, such as a spinal cord tumour or a herniated disk in the neck.
  2. Further tests to rule out other conditions may include blood and urine tests and a muscle biopsy.

Medical problems that can produce similar symptoms to ALS include HIV, Lyme disease, multiple sclerosis (MS), the poliovirus, and West Nile viruses.

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If there are symptoms in both the upper and lower motor neurons, ALS may be present.

Upper motor neuron symptoms include stiffness and resistance to movement in the muscles and brisk reflexes. Lower motor neuron symptoms include weakness, muscle atrophy, and twitching. Originally published in WebMD.

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