Signs and Symptoms of Amyotrophic Lateral Sclerosis (ALS)
Medically reviewed by Smita Patel, DO
Formerly known as Lou Gehrig’s disease, amyotrophic lateral sclerosis is now commonly referred to as “ALS.” It is a neurological disease that affects motor neurons, the nerve cells in the brain and spinal cord that control breathing and voluntary muscle movement. As these motor neurons start to die, it causes muscles to twitch, waste away, and weaken. Symptoms from ALS start mild, like minor and limited muscle weakness, but they worsen with time.
Although some treatments may slow the worsening of symptoms, scientists haven’t yet found ways to halt the disease’s progression. Eventually, you lose the ability to control voluntary movements like walking, talking, chewing, and even breathing.
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Early ALS Symptoms
ALS symptoms start gradually. Muscle weakness in the hand or foot is a common early ALS symptom, usually on one side of the body but not the other. The weakness is more long-lasting and doesn’t tend to come and go.
Importantly, this weakness doesn’t initially come with pain, unlike many other neurological conditions that can cause muscle weakness. People with ALS also usually don't have numbness along with their weakness. The affected person might initially notice they are having a hard time gripping things, or they are dropping objects more often than usual. Or someone with ankle weakness might be tripping and falling much more often.
Less commonly, the initial problem is something like dysarthria (slurred speech), due to impairment of the tongue and related muscles. Dysphagia (difficulty swallowing) can also be an initial symptom.
Undesired muscle twitches in the arm, leg, shoulder, or tongue can occur early on. In medical terminology, these are sometimes called "fasciculations." Less commonly, a person might notice painful muscle cramps as part of their initial symptoms.
A little bit later, someone might start to notice a lot of tightness in a muscle, called spasticity. That can make it difficult to move, and it also increases the likelihood of falling.
Later ALS Symptoms
Symptoms will worsen over time. Eventually more and more muscles become affected, and the weakness can become more severe. In addition to all the early symptoms of ALS discussed, progressive symptoms often found in later disease may occur.
Progressive weakness: A person who initially just had weakness in their hand might start to have weakness in their arm or shoulder. The muscles may start to get shorter and smaller, called a muscle contracture.
Difficulty speaking: A person with initial slurring of speech might eventually have trouble forming words at all. Mild difficulty with swallowing might become more severe. Partly because of this, people with more advanced ALS are also at risk of poor nutrition and substantial weight loss.
Sialorrhea (excess drooling): Sialorrhea is another common and bothersome problem, caused by difficulty manage the muscles around the tongue and mouth in the same way.
Constipation: This ocurs because of the way the disease affects the muscles around the anus. Urinary issues with ALS usually aren’t severe, but some people have trouble feeling like they can’t completely empty their bladder, or they might have a little urine leakage (incontinence).
Shortness of breath: This key symptom that typically emerges in the later stages of the disease, as the muscles involved in breathing become affected. Even just the exertion of speaking might bring on the symptom.
Insomnia and fatigue: Insomnia and fatigue are also common, and may be related shortness of breath, pain, other disease features, or depression.
ALS usually results in death within three to five years after symptoms begin, although around 10% of people live a decade or more.
Pain Complications in Later Disease
Although it doesn't directly cause pain, pain is a relatively common complication for people whose ALS has progressed. For example, if you can’t stabilize your shoulder due to muscle weakness, it can lead to stress on the bones and joints, causing pain.
It is possible to develop a pressure injury from not being able to move around well enough in bed. Muscle tightness and shortening can lead to pain as well. Some people also start to experience a pins-and-needles type of pain in some parts of their body.
Brain-Related Problems in Later Disease
Given the challenging and disabling nature of the disease, it’s not surprising that many people with ALS experience symptoms of anxiety and depression.
However, for many people with ALS, the picture is more complex than a typical depression. Although scientists used to primarily consider the symptoms from muscle weakness, it’s now clear that at least half of people with ALS eventually develop additional cognitive problems which can affect their mood and their ability to think.
For example, around 20-50% of people with ALS eventually develop a syndrome called “pseudobulbar affect.” This can cause excess laughing or crying, or it may cause the person to involuntarily express their emotions.
Some people also experience gradual signs of cognitive impairment, although this varies a lot from person to person. Some signs might be:
Changes in personality
Social inappropriateness
Problems with finding words
Apathy, or a lack of interest
Deficits in attention
Poor judgment
Poor self-care, independent of physical capacities
In the more severe cases, 5% or more of people who have ALS may develop a type of impairment called “frontotemporal dementia,” named for the brain areas that are primarily affected (the frontal and temporal lobes of the brain).
When to Contact a Healthcare Provider
People with ALS need frequent contact with a healthcare provider, ideally with a multidisciplinary care team who can meet multiple needs as their symptoms change. People with later stage disease typically require additional nursing support as well.
If you are having new symptoms, get in touch with your healthcare team. In some cases, medications or other therapies might be able to help. You should particularly get in touch for more serious symptoms, like worsening shortness of breath.
For very severe or for sudden symptoms, like severe shortness of breath, get emergency help. You’ll also need to get emergency attention for an issue such as problem with your ventilator.
Questions to Ask Your Provider
There’s so much to learn about ALS that it can be quite overwhelming, especially at first. Your questions will likely change as your diagnosis becomes clear and your symptoms evolve. Here are questions you might ask your healthcare provider at different stages.
Could my hand or foot weakness be a sign of a different medical condition?
What kinds of treatments are available to slow the disease’s progression?
If someone with ALS has difficulty swallowing, when might they need a feeding tube?
What would be signs that someone with ALS needed a ventilator to help them breathe?
What are my options in terms of palliative care?
A Quick Review
ALS is a neurological disorder that causes progressively worsening muscle weakness. The disease can cause worsening slurred speech and difficulty with swallowing. Muscle spasms, muscle twitching, and muscle tightness can also occur. It can lead to symptoms like drooling, constipation, and fatigue.
Some people with ALS also experience cognitive issues from the disease, which can range a lot in severity. Eventually, it causes difficulty breathing, which may require support with a ventilator.
Frequently Asked Questions
At what age do ALS symptoms usually begin?
It’s most common for ALS symptoms to begin between the ages of 55 and 75, but it sometimes starts before that, even in a person’s 20s.
How long can you have ALS without knowing it?
A person with ALS might have mild initial symptoms for a couple of months before seeking treatment. After you see a healthcare provider, it often takes a year or so to get the correct diagnosis, because other more common medical conditions cause similar symptoms. As symptoms worsen, the diagnosis becomes more apparent.
If you’ve had mild, occasional symptoms for several years (e.g., occasional muscle cramps), it’s extremely unlikely that ALS is the problem.
What do ALS twitches feel like?
Muscle twitches from ALS aren’t usually painful, but they are annoying. A strand of muscle in one part of your body starts contracting off and on, and you may see a line moving under your skin. However, muscle twitches are also a part of some other less serious medical conditions, so don’t jump to conclusions.
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