The symptoms of amyotrophic lateral sclerosis (ALS) often come on subtly or slowly, so much so that many times, the person experiencing them may not even notice a change — at least at first.
Symptoms usually start in the hands, feet, arms, or legs. When the arms or legs are affected first, it’s referred to as limb onset ALS. (1,2)
Over time, other parts of the body become affected as more nerve cells deteriorate — the underlying cause of ALS symptoms — and more muscles weaken.
How quickly symptoms occur and progress varies for each person. However, eventually ALS causes the inability to stand, walk, use your hands and arms, swallow, speak, and ultimately, breathe.
Most people with ALS live for three to five years following their diagnosis. About 10 percent live for at least 10 years. (3)
Early Signs of ALS
Some people first notice something is wrong when they can’t use their hands to do everyday activities like button a shirt or tie their shoes. Others notice that they are tripping more often.
Early signs and symptoms of ALS may include the following:
- Tripping and falling or having a hard time walking
- Feeling clumsy
- Weakness in your hands, legs, feet, or ankles
- Muscle cramps and tightness
- Twitching in your arms, shoulders, and tongue
- Inability to keep your head up or maintain a good posture
- Slurred speech and having a hard time swallowing
- Breathing difficulty
Bowel and bladder control are usually not affected by ALS. Additionally, most people’s sense of hearing, sight, smell, taste, and touch stay intact.
However, while it has long been thought that ALS does not affect a person’s cognitive abilities to reason, remember, and solve problems, some newer research suggests that a fairly large percentage of people with ALS have cognitive or behavioral impairments, according to the ALS Association. (4)
Cognitive problems associated with ALS may show up as problems with these tasks:
- Regulating emotions
Certain behaviors may go along with such cognitive problems:
- Repeating actions or phrases
- Expressing anxiety
- Emotional reactivity
Additional abnormal behaviors associated with cognitive problems may include these reactions:
- Social withdrawal
- Lack of empathy or understanding
Depression and ALS
Receiving a diagnosis of any serious disease can lead to depression in some people, and ALS is no exception. An analysis of Sweden’s national health and population registers published in 2016 found that people with ALS are at higher than normal risk of depression immediately before and after a diagnosis of ALS. (5)
The authors of the study suggest several possible explanations for an increased risk of depression before an ALS diagnosis:
- Depression may be an early sign of ALS
- Depression may be an early symptom of frontal lobe degeneration (cell damage to the brain’s frontal area)
- What appears to be depression may actually be cognitive impairment
- Depression may be a psychological response to other early signs and symptoms of as-yet-undiagnosed ALS
As for the high risk of depression following diagnosis, the authors suggest that it may be a psychological reaction to the diagnosis, similar to that seen in people receiving a diagnosis of other life-threatening illnesses.
Symptoms of depression in a person with ALS may include sadness, guilt, sleep problems, changes in appetite, and irritability. However, as noted above, some symptoms of depression overlap with symptoms of cognitive impairment, so diagnosing depression in a person with ALS can be complicated.
Complications of ALS
As ALS worsens, the following complications may develop. (1)
ALS eventually paralyzes the muscles needed to breathe. When this happens, there are devices available to help with breathing that are similar to equipment used for sleep apnea. For instance, your doctor may recommend using a continuous positive airway pressure machine or a bilevel positive airway pressure machine to help you breathe at night.
In severe cases of ALS, you may opt for a tracheostomy, which is a surgically created hole in the front of your neck that leads to your windpipe, or trachea. A tracheostomy allows you to use a respirator to inflate and deflate your lungs around the clock.
Respiratory failure is the most common cause of death for people with ALS, occurring on average about three to five years after symptoms start. (1)
At some point, speaking clearly becomes an issue for those with ALS. At first, you might periodically slur your words, but eventually, talking becomes harder, and it becomes more difficult for people to understand what you are saying. Technology can allow other ways to communicate.
Because the muscles that control swallowing are affected, eating and drinking become difficult. As a result, people with ALS can develop malnutrition and dehydration and are at a greater risk of aspiration, or getting food, liquids, or saliva into the lungs, resulting in pneumonia. A feeding tube can ensure that you are getting enough food and hydration, and help avoid pneumonia.
ALS can cause memory and decision-making complications. In some cases, people may develop a form of dementia called frontotemporal dementia. According to the ALS Association, many studies of cognitive and behavioral impairment in people with ALS report that up to 20 percent demonstrate dementia. (4)
If you notice these symptoms over time, whether in yourself or a loved one, it is imperative to seek evaluation with a neurologist specifically trained in ALS and neuromuscular medicine. (Ask your primary care doctor for help in finding the specialist if needed.) Quickly establishing a diagnosis and then implementing the multidisciplinary care and services needed for ALS patients are important to long-term outcomes.
Caregiving and ALS
As symptoms of ALS progress and a person is able to do less independently, assistance from others becomes necessary for such tasks as personal care and hygiene, preparing and eating meals, housework, transportation, and much more.
In the United States, the majority of this care is provided by family members, many of whom provide it willingly but at a physical and emotional cost.
Caregiving for a family member with any incapacitating disease can be isolating and emotionally, physically, and financially draining. Caregivers often put their own personal and health needs second, sometimes resulting in poor health and often depression.
If you are the caregiver for someone with ALS, it’s important to reach out for help from such organizations as the ALS Association and the National Family Caregivers Association, both of which can offer tips and information on caregiving and also connect you to resources that can offer respite care, help with financial planning, group support, and other services. (6)