what is als

Everything You Need to Know About ALS

What is ALS?

The disease goes by many names, but in America, ALS is commonly known as Lou Gehrig’s disease (after the hall-of-fame baseball player who contracted it). So, what is ALS? It is a seriously debilitating, progressive condition that destroys the lines of communication between nerves and muscles.

Since the neuromuscular system includes such a wide range of nerve cells, the damage of ALS is likewise widespread, where little daily acts, both conscious and subconscious, become increasingly difficult. Not only will the body struggle with more complex motor activities, like lifting objects, but even the simple act of breathing can be interrupted.

ALS Explained

This condition is frightening and tough to comprehend, which can make a diagnosis even more difficult to handle. Understanding the disease begins with breaking down the complicated title.

  • A (amyotrophic). This translates to “no muscle nourishment.” As the muscles lose nourishment, they become weaker, and muscle strength in every region of the body begins to suffer as the muscles waste away.
  • L (lateral). This refers to the sides of the spinal cord, where the disease attacks the nerves that feed and control the muscles around the body.
  • S (sclerosis). Scar tissue develops in the place of healthy tissue. In this case, the damaged nerves at the sides of the spinal cord give way to hardened tissue, which stops the nerve signals from traveling to the muscles.

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In the end, the nerve damage, scar tissue and muscle wasting lead to paralysis of the body, by severing the neuron pathways between the brain, spinal cord and the muscles. But even though neurons in the brain do suffer, the physical senses and mental comprehension are not necessarily affected.

The damage occurs in the upper and lower motor neurons, which are nerve cells in the brain and spinal cord that command muscle movement but are not in charge of other sensations, like hot and cold, pain, pressure, or emotional response. One of the most tragic elements of ALS is the mind often continues to function well but the body just will not respond, and so expression and communication becomes frustratingly difficult.

ALS Risk Factors

It is a fairly rare disease — it only affects about 3 in every 100,000 people each year. Given that it is relatively uncommon, it mimics other diseases and is difficult to predict, so there is also a good deal of mystery involved.

However, experts have uncovered some facts that can help to determine ALS risk and help spot the first symptoms.

1. Adult Onset

Theoretically, anyone can develop this condition, but it typically manifests in adults between the ages of 40 and 70. Some of these adults will develop symptoms quickly, while others begin to show signs that worsen gradually.

2. Gender

ALS affects men more often than women and Caucasian and non-Hispanic men are at a slightly higher risk. However, ALS does not discriminate: anyone can be at risk of developing the disease.

3. Hereditary

Experts estimate that between 5% to 10% of ALS cases are hereditary (classified as familial ALS). When there is a hereditary element in ALS, only one parent needs to carry the gene for children to inherit it. However, the gene mutation does not guarantee a grim prognosis — it may lead to ALS at some point, but it also may not.

Around 90% of ALS cases are sporadic (they cannot be traced to any genetic risk), which can make it difficult to diagnose early. In these cases, family members of the patient are not at any increased risk of developing ALS.

Diagnosis and Prognosis

Research on ALS has escalated in recent years (notably since the ALS Ice Bucket Challenge swept through the online community) and doctors have made gains in diagnosis and treatment. However, there is no cure for ALS; to slow down progression and preserve independence for longer, it is important to find and treat the disease early.

Symptom Pattern

  • Weakness. Like some other neuromuscular diseases, ALS typically begins with weakness in one or more areas of the body, like the hands when they are performing manual tasks. More than half of all ALS patients will experience weakness in their arms and legs as the disease takes hold.
  • Loss of coordination. The other common early symptoms of ALS — stumbling, twitching, dropping things and slurred speech — can lead a medical team to suspect Parkinson’s disease or multiple sclerosis before more severe and distinct symptoms arise.
  • Spasticity. Although lower neuron damage can cause muscles to lose all action, upper motor neuron damage can cause spasticity, or exaggerated reflexes, including an overactive gag reflex that makes swallowing difficult.

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As more voluntary muscles are affected, more drastic symptoms of nerve damage and muscle atrophy will develop: the arms and legs may become paralyzed, then the rest of the body, and eventually the deep muscles in charge of swallowing and breathing will fail. Tragically, most people with ALS die from respiratory failure within five years from the onset of symptoms.

ALS does spare some areas. Intelligence and comprehension are usually not affected, and all the senses continue to operate as usual. Most patients remain in control of their eye movements, bladder function and bowel function. However, there is a possible link between late-stage ALS and dementia.

Imaging Tests

Diagnosing ALS means ruling out other diseases. The tests used (like x-rays, electromyography (EMG) and nerve conduction studies) seek to distinguish the specific nerve damage of ALS from other types of nerve damage.

MRI scans can help pick up underlying problems. While regular MRI scans will not turn up evidence of ALS, they can reveal other conditions that could be causing the ALS-like symptoms, such as a herniated disk, a cyst in the spinal cord or a tumor.

Blood Tests and Biopsies

After imaging tests, your doctors may want to take blood and urine samples to rule out other conditions. If a myopathy (muscle disease) is suspected, a muscle can be a useful test. By extracting a small sample of tissue, your doctor can examine it closely to determine the nature of the muscle tissue.

Other acute and chronic conditions can bring on ALS-like symptoms, so diagnosis can be complicated. It’s important to ask any questions that come to mind and get a second opinion from another neurologist if you feel you need it.

Once you have an ALS diagnosis, certain drug treatments can be started to slow the progression of symptoms (namely, a drug called riluzole), and there are other medications to improve quality of life and relieve discomfort.

In Conclusion

ALS is not easy to live with, and not easy to care for. Symptoms can become increasingly harder to handle, so it is important to reach out for help when you need it.

Doctors, nurses, hospice caregivers, respiratory therapists, speech therapists and physical therapists all have a role to play in ALS management. Don’t try to go through the challenge alone, and be sure to keep up with recent treatment advances.

More research is happening all the time, and new findings are improving ALS understanding and treatment every year.