Lately, there’s been a lot online about ALS after the passing of actor Eric Dane. Curious to understand how ALS leads to death, I did some research so I could better explain it to others. Join me as I share what I’ve learned about ALS—I hope you find it helpful!
What is ALS?
ALS (Amyotrophic Lateral Sclerosis), which is also known as Lou Gehrig’s disease, is the most common motor neuron disease. ALS affects multiple parts of the nervous system, including the brain, spinal cord and peripheral nerves. It is a progressive disease, which means that the symptoms get worse over time, ranging from months to years. ALS is also a degenerative disease, which means that it destroys the nervous system, bit by bit, slowly changing the way a person moves, or carries out normal bodily functions, like breathing. It continues to break down the body in various ways until the body can no longer function. This is when people pass away, as basic body functions can’t occur, and the body cannot sustain life.
No one really understands how ALS starts or how it is caused, but many different theories have been proposed, which suggest that aspects of the body’s biochemistry can lead to the disease. ALS is generally quite rare, with 0.001% cases globally in 2020. This means for every 100,000 people in the population, roughly 1-2.6 people were diagnosed with ALS. There are different types of ALS, which are classified according to the types of symptoms, the parts of the nervous system that are affected and how often these symptoms are seen. Some of the types include Primary Lateral Sclerosis, Progressive Muscular Atrophy and Progressive Bulbar Palsy.
Signs and Symptoms
As ALS is a motor neuron disease, the majority of the signs and symptoms are linked to movement and muscular difficulty. However, there are some symptoms that may seem unrelated to the motor neurons that are important to identify when thinking about ALS. Some of the general symptoms include:
- Arm and hand weakness – this means it becomes harder to move your arms and hands, which can make daily activities like brushing your hair or teeth difficult.
- Stiffness in walking – this is linked to leg and foot weakness, similar to arm weakness, but these symptoms cause difficulty when walking.
- Unsteady, unbalanced walking – again, linked to leg weakness
- Muscle spasms – can be linked to dysfunction of the peripheral nerves.
- Difficulty standing from sitting, or climbing up the stairs – again, this is linked to generalised body weakness.
- Foot drop – this is what happens when you cannot hold your foot up while walking, and so it ends up looking like your foot is dragging or dropping.
- Head drop – this is linked to neck muscle weakness.
- Stooping and slouching body posture – again, linked to generalised body muscle weakness
Symptoms that are linked to ALS but don’t seem related are:
- Difficulty breathing – this is because the chest muscles and diaphragm get tired quickly, making it hard to carry out the function of breathing.
- Coughing or choking on liquids – difficulty swallowing. This can be linked to weakness and a lack of coordination between the tongue and throat muscles. This, unfortunately, can lead to complications like chest infections.
- Slowed, strained speech – this is a result of weakness in the vocal cords and throat.
Having these symptoms doesn’t automatically mean that you have ALS. It’s just a good thing to be aware of these symptoms as they are linked to many different neurological diseases, which can go unnoticed for some time.
What tests can be done to find out if you have ALS?
There are lots of different tests to determine whether you have ALS, some of which are carried out to find out if you have true motor neuron disease signs and others to rule out other diseases as the cause of your symptoms (2). Some of these tests include:
- Nerve conduction studies – this is where electrical signals are sent down specific nerve pathways to see if there is a problem with the transfer of signals down specific nerves (2).
- MRI scan of the brain and spinal cord – this helps to identify if there are any structural growths or inflammation causing the symptoms seen.
- Lumbar puncture – this is a test which allows doctors to obtain and analyse a sample of the fluid which is around the spinal cord (2). This sample is obtained by inserting a needle into the bottom of the spine, on the back. This test will be explained in detail if carried out, as it is an anaesthetic procedure. It is a test to rule out other causes when considering ALS. This test should generally be normal.
How is ALS treated?
Unfortunately, ALS does not have a cure. If you are diagnosed with ALS, the disease keeps worsening over time. However, to help with living with the disease, medical treatment is offered to help manage the symptoms. Many different healthcare specialists are involved in the care of people who have been diagnosed with ALS, as they need support for the widespread symptoms (1). Individuals and professionals often involved in the care of patients with ALS include respiratory physicians, physiotherapists, occupational therapists, dieticians, speech and language therapists and other advanced nurse practitioners.
Medical Treatments of ALS
- Riluzole – this medication is given to all people diagnosed with ALS at the time of diagnosis. No one knows exactly how it works in the body (3), but it is a very effective management of the symptoms of ALS (2). Many studies have shown that it helps to prolong survival in patients and slows the progression of the symptoms (1). It is offered immediately after diagnosis, as Riluzole is most effective in people who have had symptoms for less than 5 years.
- Unfortunately, Riluzole does come with its own problems. For example, it can cause problems with your liver, and therefore it is important that you stay up to date with your monitoring tests when using Riluzole (1, 2).
- Edaravone – this is also a medication that can be offered to help with symptom management. It targets one of the theorised ways in which ALS occurs and reduces this effect. However, this treatment is not approved in Europe, only in the US and some other countries (2).
Other general treatment approaches to ALS
- Non-invasive ventilation – these are methods that are used to help people who are struggling with their breathing to better regulate their breathing.
- Speech and language therapy – this is to help train the muscles to swallow safely and to prevent choking risks. With this, they may also be taught to develop ways to help their speech.
- Diet modification or feeding tubes – this can be offered if the swallowing risks are too great for patients with more advanced forms of ALS.
- Physiotherapy – therapy to build and train muscles to still function with their new limitations.
How can ALS lead to death?
As mentioned above, ALS has a huge effect on the basic functions of the human body; breathing, walking, control of our heart and blood vessels, etc. Over time, the effects of ALS become so severe that these basic functions just can’t continue anymore, which means they can’t sustain life, and therefore, the person, unfortunately, dies. Breathing difficulty is often the most common cause of death in people with ALS (4).
I know this is a very short recap of ALS, but hopefully, this gave you a good summary of what the condition is generally.
References:
- Brotman RG, Moreno-Escobar MC, Joseph J, Munakomi S, Pawar G. Amyotrophic lateral sclerosis [Internet]. StatPearls – NCBI Bookshelf. 2024. Available from: https://www.ncbi.nlm.nih.gov/books/NBK556151/
- Amyotrophic lateral sclerosis – Symptoms, diagnosis and treatment | BMJ Best Practice US [Internet]. Available from: https://bestpractice.bmj.com/topics/en-gb/330
- Riluzole [Internet]. Drug Bank. 2026 [cited 2026 Mar 19]. Available from: https://go.drugbank.com/drugs/DB00740
- Wolf J, Safer A, Wöhrle JC, Palm F, Nix WA, Maschke M, et al. Todesursachen bei amyotropher Lateralsklerose. Der Nervenarzt [Internet]. 2017 Feb 9;88(8):911–8. Available from: https://pubmed.ncbi.nlm.nih.gov/28184974/
