In this blog post, we’ll explore the significance of the Ice Bucket Challenge, as well as the causes, symptoms, and current treatments for ALS—and why we need to remain committed to raising awareness.
Recently, videos of people dousing themselves with ice water have been steadily appearing on various social media platforms such as Facebook and YouTube. This ice water relay, in which many celebrities and ordinary people are participating, is called the “Ice Bucket Challenge” and has spread worldwide. This campaign, which spreads rapidly as each participant nominates three others, was launched to raise awareness of the suffering of ALS patients and increase public interest in the disease.
In particular, as news that world-renowned physicist Stephen Hawking suffered from ALS became widely known, many people began to take an interest in the disease, and the Ice Bucket Challenge has established itself as a leading public awareness campaign for ALS. Recently, new variations of the Ice Bucket Challenge campaign aimed at raising research funds and supporting patients have continued to gain significant attention. While many people have heard the name “ALS” at least once, few actually understand exactly what the disease entails. In this article, let’s take a detailed look at what ALS is, its symptoms and causes, and how far current treatments have advanced.
ALS is not merely a disease that causes physical paralysis; it imposes a significant psychological and financial burden on both patients and their families. For this reason, many people participate in donations and volunteer activities to help ALS patients, and these efforts not only provide tangible assistance to patients but also play a crucial role in raising awareness of ALS throughout society.
First, the medical term for the disease commonly known as Lou Gehrig’s disease is amyotrophic lateral sclerosis (ALS). In South Korea, the term “muscle atrophy lateral sclerosis” was used for a long time, but “amyotrophic lateral sclerosis” is now the standard medical term. To the general public, the disease is better known as “Lou Gehrig’s disease,” named after the legendary American baseball player Lou Gehrig, who suffered from this condition.
Our bodies contain nerve cells that connect the brain to various organs. Nerve cells can be broadly divided into three categories based on their functions. First, there are sensory neurons, which transmit information received from the senses—such as the eyes, nose, tongue, skin, and ears—to the brain; association neurons, which process this information in the brain and spinal cord and connect with other neurons; and motor neurons, which transmit processed commands to the muscles to initiate actual movement.
Lou Gehrig’s disease is a classic neurodegenerative disorder that selectively damages only the motor neurons among these. As the upper motor neurons—which command movement—and the lower motor neurons—which transmit signals from the spinal cord to the muscles—are gradually destroyed, paralysis slowly progresses not only in the muscles of the limbs but also in the respiratory muscles.
In the early stages, damage to the upper motor neurons makes it increasingly difficult to move the muscles of the face, arms, legs, and trunk, and may cause weakness in the limbs or an unsteady gait. In addition, facial muscle movements may become sluggish, leading to unnatural facial expressions or muscle stiffness.
As the disease progresses, lower motor neurons are also damaged, causing the muscles of the tongue, throat, and respiratory system to weaken. This results in progressively slurred speech and dysphagia, a difficulty in swallowing food. Aspiration—where food or saliva enters the airway—occurs frequently, which can lead to aspiration pneumonia. As respiratory muscle function weakens further, patients find it difficult to breathe adequately on their own; without appropriate respiratory support, respiratory failure becomes the leading cause of death.
The exact cause of ALS has not yet been clearly identified. However, current research suggests that a combination of various factors is involved. Mutations in specific genes have been identified in some patients; notably, genes such as C9orf72, SOD1, TARDBP, and FUS have been found to be associated with ALS. However, these genetic factors are primarily associated with familial ALS, which accounts for only about 10% of all cases, while the majority of patients have sporadic ALS with no specific family history.
In addition, various hypotheses—including the abnormal accumulation of proteins within cells, oxidative stress, mitochondrial dysfunction, inflammatory responses in neurons, and environmental factors—are being studied; however, a single cause capable of explaining all cases has not yet been identified.
Consequently, no cure for ALS has been developed to date. However, treatments are steadily advancing compared to the past. Notably, riluzole is used as a medication that somewhat slows the progression of the disease, and edaravone has also been confirmed to slow the rate of progression in some patients and is being utilized in treatment. Recently, research on gene therapies targeting patients with specific genetic mutations has been actively underway, and some treatments are being applied in actual clinical settings under limited conditions. However, current treatments are aimed at slowing the progression of the disease and maintaining patients’ quality of life rather than curing it.
