Symptoms of ALS
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Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder in which worsening muscle weakness leads to movement problems and other symptoms such as trouble swallowing, speaking, and breathing. Patients also can experience digestive issues, mental health problems, and changes in behavior.
Also known as Lou Gehrig’s disease, after the famous baseball player who was forced to retire following his diagnosis, ALS is a progressive disorder, meaning that symptoms start as mild in the early stages, and then become more and more severe as patients progress to later disease stages over time.
The disease is marked by damage to motor neurons, the specialized nerve cells that control muscle movement. Symptoms can occur at any age but usually begin around ages 55 to 75.
Muscular symptoms
Muscle weakness is the hallmark symptom of ALS. Usually this weakness starts off mild and only affects one body part, but with the progression of ALS it becomes more severe and starts affecting other body parts.
There can be a lot of variability from person to person in how ALS-related muscle weakness manifests, but patients are commonly divided into two groups based on initial symptoms. These are:
- limb-onset disease, which is observed in around two-thirds of ALS patients and is characterized by symptoms of muscle weakness starting in the arms and/or legs. Usually, the first muscles affected are distal muscles — those further from the torso, in the hands, feet, calves, and forearms. When this affects the upper limbs, it can cause difficulty with tasks like writing, while weakness in the lower limbs often manifests as walking difficulties.
- bulbar-onset disease, seen in about one-third of patients, which first affects the muscles around the face and throat. These muscles in the head and neck are called the bulbar muscles. Difficulty eating and/or speaking are the most common early symptoms in bulbar-onset ALS.
As ALS progresses into later disease stages, muscle weakness may develop into paralysis. Most people with ALS will have limited ability to move voluntarily in the later stages of the disease.
In addition to muscle weakness, other muscle-related manifestations of ALS may include:
- muscle twitches (fasciculations)
- muscle wasting (atrophy)
- muscle fatigue
- muscle cramps and stiffness (spasticity)
- shaking or tremor-like symptoms, which are the result of muscle failure when muscles are pushed to their limit
- muscles becoming hardened, tightened, and shortened (contractures).
Breathing difficulties
Muscles in the chest and abdomen work to pull and push air in and out of the lungs when a person breathes. ALS often causes weakness in these muscles, leading to breathing difficulties. Some people with bulbar-onset ALS may have trouble breathing as an early symptom, though it’s more common for breathing problems to develop in later stages of the disease.
In addition to feelings of overt breathlessness, weakness in the respiratory system can manifest with symptoms such as:
- gasping or labored breathing, sometimes called air hunger
- persistent fatigue and low energy
- frequent yawning or sighing
- waking up frequently during the night
- awaking in the morning with a headache or feeling fuzzy-headed or confused
- trouble lying flat.
Weakness of lung muscles often causes a weak cough, which can make it difficult to remove secretions from the lungs. When these secretions build up, it can set the stage for infections and pneumonia, which are common complications for ALS patients.
By the later stages of the disease, ALS patients usually will require a ventilator to help them breathe. One frequent problem in ALS is respiratory failure, when the lungs are not able to adequately supply the body with enough oxygen to function. Indeed, respiratory failure is the most common cause of death among people with ALS, typically occurring within five years of symptoms first appearing.
Speech problems
Weakness in the muscles around the mouth and jaw can cause speech problems, referred to as dysarthria. This symptom affects more than 80% of people with ALS at some point in the disease course.
Speech problems in patients with limb-onset disease usually don’t develop until the middle or late stages of ALS. In those with bulbar-onset ALS, however, dysarthria may be the first symptom or among the earliest signs of the disorder. Speech problems are eight times more common than swallowing problems as the first symptom of bulbar-onset ALS.
Specific manifestations of dysarthria in ALS can include:
- difficulty articulating and imprecise pronouncing of consonants
- slow and slurred speech
- nasal, breathy, or hoarse voice
- trouble moving the tongue
- a weak or overactive gag reflex.
In addition, patients often feel tired after speaking, and may find it difficult to project their voices due to weakening of the lung muscles. As such, there is a tendency for people with ALS to talk less and use shorter sentences as their disease progresses.
In late stages of the disease, many people with ALS rely on augmented alternative communication or speech-generating devices — strategies for communication that don’t rely on speech, such as text-to-voice apps or communication boards. These devices and strategies can help ALS patients more easily convey their thoughts and desires.
Difficulty swallowing
Around 80% of people with ALS experience difficulty swallowing, referred to as dysphagia. These problems usually occur in the later stages of the disease, though they may occur early in some patients.
Swallowing requires the combined action of many different muscles of the lips, tongue, and throat. Any of these muscles can be affected in ALS, which in turn can impair the swallowing process.
Difficulty swallowing may not be obvious, especially in early stages when the effects are relatively mild. Signs of dysphagia may include:
- frequent coughing or choking on food while swallowing
- food or liquid spilling through the mouth or nose
- food sticking in the throat after swallowing
- pooling of saliva in the mouth that may look like drooling
- unintentional weight loss due to a lack of proper nutrition.
Difficulty swallowing can increase the risk of food or liquid going into the windpipe, which is called aspiration. It may cause choking and can set the stage for infections and complications like pneumonia.
As ALS progresses into its later stages, a feeding tube usually becomes necessary to ensure patients receive needed nutrition.
Digestive issues
Many people with ALS experience gastrointestinal problems. These are thought to arise due to dysfunction of the nerves and muscles that regulate how food is passed through the digestive tract. These mild to severe ALS symptoms also can result from a dysfunction in the autonomic nervous system, which regulates unconscious processes such as heart rate and digestion.
Specific digestive issues that are common in ALS include:
- constipation and cramping
- acid reflux
- nausea and vomiting
- delayed gastric emptying (when food does not clear the stomach as quickly as it should)
- abnormalities in liver and/or pancreas function
- alterations in the composition of bacteria and other microorganisms that live in the digestive tract, called the gut microbiome.
Anxiety and depression
As with any serious illness, ALS has a huge impact on the day-to-day lives of patients, as well their loved ones and caregivers. Depression and anxiety are common among people with ALS, especially soon after getting a diagnosis.
As the condition progresses, patients usually retain their mental reasoning, thinking, and sensory abilities. Thus, they are aware of their progressive loss of motor function and increasing limitations. This makes them prone to anxiety and depression, leading to changes in appetite and sleep, or even suicidal tendencies.
Manifestations of these mental health problems are varied and can include:
- persistent feelings of sadness, worthlessness, and/or hopelessness
- excessive worry, guilt, and/or fear
- trouble sleeping or sleeping too much
- eating too much or too little
- irritability and a tendency to get very angry over small issues
- persistent low energy and fatigue.
While mental health problems can be challenging, a number of strategies and medications can make it easier for ALS patients to cope with anxiety and depression.
For those struggling with depression, effective strategies may include counseling and psychotherapy. During sessions, a therapist can help patients develop strategies to respond to challenging situations more clearly and have a better control of their emotions.
Another useful behavioral approach for some individuals with ALS anxiety is counseling with a specialist who helps individuals change how they think and behave in certain situations. Patients also may benefit from mindfulness programs that teach them how to live in the present without judgment. Other potential therapies include relaxation techniques, such as yoga and meditation, which can help calm the mind and body.
Cognitive issues
While ALS previously was not thought to affect the mind, more recent research has shown that up to half of ALS patients will experience cognitive or behavioral changes at some point during the course of their disease.
These symptoms may range from minor impairments to overt dementia, but the most common changes are abnormalities in executive function — people’s ability to plan their actions and regulate their own behavior.
Other common cognitive and behavioral changes in ALS include:
- difficulty concentrating or shifting attention from one thing to another
- embarrassing, inappropriate, or uncharacteristic behavior
- becoming fixated on certain ideas or repeating things excessively
- aggression, agitation, and strong emotional reactions that are hard to control
- trouble with empathy and lack of judgment
- forgetting the meaning or spelling of words.
Up to 20% of people with ALS will develop dementia, which broadly refers to changes in thinking and behavior that represent a dramatic departure from the person’s normal habits. Dementia can substantially impair an individual’s ability to function in day-to-day life and can place a burden on patients, their family, and their caregivers.
A particular form of dementia called frontotemporal dementia (FTD), which is marked by damage to the brain’s frontal and temporal lobes, is especially common in ALS.
Pseudobulbar affect
About 25%-50% of ALS patients experience pseudobulbar affect, characterized by sudden bursts of emotional displays that are unrelated to a person’s emotional state — laughing though nothing is funny, or bursting into tears when nothing is sad. These displays can occur several times per day, and they often seem inappropriate and can be embarrassing.
Pseudobulbar affect is thought to result from disruptions in the brain’s neural circuits, which control the expression of emotions, though the exact cause is still not clear.
Other symptoms
In addition to the disease’s cognitive and muscular symptoms, patients can experience:
- skin problems, due to poor nutrition and not being able to change positions or clean the skin properly
- urinary urgency, or the need to urinate suddenly, due to spasms in the muscles controlling the bladder
- pain in the muscles and joints, due to muscle spasms and cramps and poor stretching.
In the latter stages of the disease, people with ALS also may be more likely to develop serious complications like pulmonary embolism — when a clot blocks blood flow in the lungs — and abnormal heartbeats known as cardiac arrhythmias.
ALS News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.
FAQs about symptoms of ALS
Amyotrophic lateral sclerosis (ALS) usually progresses quite quickly, though progression is not linear and rates of progression can vary widely from person to person. Most people with ALS do not survive more than five years after the initial onset of symptoms.
The most common initial symptom of amyotrophic lateral sclerosis (ALS) is subtle muscle weakness that affects the hands and/or feet. This is usually first noticed as unusual difficulty with activities like writing or walking. Less commonly, the first symptoms of ALS can affect the muscles around the mouth and throat, most commonly manifesting as problems with speech.
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by muscle weakness that gets progressively worse over time. Usually, the ALS first symptoms are very mild, and then get continually more severe as the disease progresses. It is common for early symptoms of ALS to be so subtle that they are easily ignored; some early symptoms may only be noticed in retrospect.
Amyotrophic lateral sclerosis (ALS) is a progressive disorder, meaning symptoms continually get worse as time goes on. This progression isn’t usually a straight line — symptoms may rapidly worsen for a time, then stay mostly stable for a while before deteriorating again. Patients may experience some day-to-day variability in their experiences of symptoms, and there may even be times when some lost functionality is recovered, but such periods are almost always temporary and patients will eventually experience continued symptom worsening.
There is no treatment that can reverse the nerve damage that causes amyotrophic lateral sclerosis (ALS), though some available treatments may slow disease progression. There also are a number of medications and interventions, like physical and occupational therapy, that can ease specific symptoms of the disease and help patients to maximize their functionality and life quality.
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