Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease, is a progressive neurodegenerative disease that affects motor neurons, or nerve cells controlling muscle movements.
Symptoms of ALS can occur at any age but usually begin around ages 55 to 75 and progress gradually. Muscles in arms and legs are generally affected first, followed by muscles involved in speech and swallowing, although the sequence of symptoms can vary from person to person.
Depending on the stage of disease progression, ALS symptoms may range from mild to severe. The most common symptoms of ALS include:
One prominent problem for ALS patients is muscle weakness. This affects their ability to perform everyday tasks, such as walking, speaking, and eating. It can also make chewing and swallowing difficult, and result in impaired breathing.
As a result of muscle weakness, patients often begin by experiencing poor balance and awkwardness when walking or running, leading to more common tripping. They also have difficulty grasping objects.
Additional muscle symptoms include twitches, cramps, spasms, pain, and muscle stiffness (spasticity). Some patients may experience tremor-like symptoms, which is the result of muscle failure when muscles are pushed to their limit.
Symptoms of muscle weakness continue to worsen as the disease progresses, leading to extremely limited mobility and paralysis, in later stages.
In some cases, breathing difficulties are among the first symptoms of ALS, as the chest muscles and diaphragm that control breathing become affected. In other patients, breathing difficulties may only appear as the disease progresses.
Breathing symptoms include shortness of breath, shallow breathing at night that may cause sleep interruptions, excessive saliva production, and difficulty coughing. Patients may also feel breathing discomfort while eating, speaking, or doing other activities.
As ALS progresses, the muscles involved in breathing become progressively weaker, ultimately resulting in respiratory failure, which can be life-threatening.
ALS patients may experience digestive problems, such cramping or constipation, that can be so mild that they are often not immediately noticed or attributed to ALS. Patients can also experience delayed gastric emptying, which occurs when stomach doesn’t empty food as fast as it should.
One possible reason for this are changes in the composition of the harmless microorganisms that inhabit the digestive system and assist with food digestion — the so-called gut microbiome. Changes in the gut microbiome have been linked to a number of health conditions, including ALS.
Swallowing requires the combined action of many different muscles of the lips, tongue, and throat. When any of these muscles becomes affected, patients experience difficulty swallowing.
This symptom, medically known as dysphagia, impacts about 85% of ALS patients during the course of their disease, and is more common in the later stages of the disease.
Difficulties in swallowing aren’t always apparent, but there are several signs that indicate a possible problem. These include frequent coughing or choking on food while swallowing, food escaping from the mouth or nose, drooling, and difficulty managing saliva.
Difficulty swallowing also increases the risk of food or liquid going into the windpipe, which is called aspiration. Aspiration can cause infections and other complications.
The exact symptoms experienced by patients will depend on the stage of swallowing that is affected by the disease. Patients can introduce a variety of strategies to improve swallowing efficacy, but a feeding tube usually becomes necessary in later stages.
The patient’s health and nutrient status can be impacted by swallowing difficulties, due to reduced calorie intake. As they also burn calories at a faster rate than most people without ALS, patients tend to lose weight rapidly and can become malnourished.
Speech problems, or dysarthria, are common and among the first symptoms of ALS, occurring in over 80% of the cases.
The speaking process involves many different nerves that control the movement of the lips, jaw, tongue, and vocal cords. The degeneration of motor neurons in ALS causes a reduction in the stimulation of these muscles and their proper use becomes difficult.
This results in difficulty managing the voice’s tone, pitch, and rhythm, which causes the patients’ speech to become slow, slurred, and difficult to understand. Patients also may find difficult to pronounce certain consonants and have a nasal-sounding voice and hoarseness.
Anxiety and Depression
As with any serious illness, ALS a has huge impact on a person’s life and in the lives of their family members and caregivers.
As the condition progresses, patients usually retain their mental reasoning, thinking, and sensory abilities, and are aware of their progressive loss of motor function. This makes them prone to anxiety and depression, leading to changes in appetite, sleep, or even suicidal tendencies.
These symptoms are common in patients with ALS, particularly immediately following a diagnosis.
About half of patients with ALS experience mild to moderate cognitive or behavioral symptoms. These include childish behavior, loss of manners, lack of attention, and aggressiveness. About one in five patients develop dementia.
Some ALS patients may also experience a rare condition called the pseudobulbar affect characterized by sudden and brief bouts of laughter or crying for no reason.
These outbursts, which do not necessarily reflect how the person is actually feeling, are thought to result from the disruption in the brain’s neural circuits that control the expression of emotions, but the exact cause is still unclear.
These episodes can be quite severe and can occur several times a day. Embarrassment or anxiety caused by pseudobulbar affect may lead to isolation, which may impact patients’ employment or relationships.
Other symptoms of ALS include abnormal fatigue of the arms and legs, which can either be experienced initially or in later stages, as well as urinary urgency, or the sudden need to pee.
At later stages of the disease, patients are also more prone to have pneumonia.
Last updated: Aug. 10, 2021
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