What Is ALS?
Amyotrophic Lateral Sclerosis (ALS), formerly known as Lou Gehrig's disease, is a neurological disorder that affects motor neurons, the nerve cells in the brain and spinal cord that control voluntary muscle movement and breathing. As motor neurons degenerate and die, they stop sending messages to the muscles, which causes the muscles to weaken, start to twitch (fasciculations), and waste away (atrophy). Eventually, in people with ALS, the brain loses its ability to initiate and control voluntary movements such as walking, talking, chewing and other functions, as well as breathing. ALS is progressive, meaning the symptoms get worse over time.
The U.S. Food and Drug Administration has approved several drugs for ALS that may prolong survival, reduce the rate of decline, or help manage symptoms. However, there is currently no known treatment that stops or reverses the progression of ALS.
ALS Symptoms
Early symptoms include:
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Muscle twitches in the arm, leg, shoulder, or tongue
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Muscle cramps
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Tight and stiff muscles (spasticity)
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Muscle weakness affecting an arm, a leg, or the neck
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Slurred and nasal speech
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Difficulty chewing or swallowing
As the disease progresses, muscle weakness and atrophy spread to other parts of your body. People with ALS may develop problems with:
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Chewing food and swallowing (dysphagia)
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Drooling (sialorrhea)
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Speaking or forming words (dysarthria)
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Breathing (dyspnea)
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Unintended crying, laughing, or other emotional displays (pseudobulbar symptoms)
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Constipation
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Maintaining weight and getting enough nutrients
Eventually, people with ALS will not be able to stand or walk, get in or out of bed on their own, use their hands and arms, or breathe on their own. Because they usually remain able to reason, remember, and understand, they are aware of their progressive loss of function. This can cause anxiety and depression in the person with ALS and their loved ones. Although not as common,
people with ALS also may experience problems with language or decision-making.
Most people with ALS die from being unable to breathe on their own (known as respiratory failure,) usually within three to five years from when the symptoms first appear. However, about 10% survive for a decade or more.
How Is ALS Diagnosed?
It is important to get an accurate ALS diagnosis as soon as possible. ALS treatments may be most effective early in the course of the disease. A neurologist familiar with ALS can help a person get diagnosed early after symptom onset.
There is no single test that can definitely diagnose ALS. A healthcare provider will conduct a physical exam and review the person’s full medical history. A neurologic examination will test reflexes, muscle strength, and other responses. These tests should be performed at regular intervals to assess whether symptoms are getting worse over time.
A healthcare provider may conduct muscle and imaging tests to rule out other diseases. This can help support an ALS diagnosis. These tests include:
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Electromyography (EMG)—evaluates how well nerves and muscles are functioning. This test can include:
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A Nerve Conduction Study (NCS) — measures the electrical activity of nerves and muscles by assessing the nerve's ability to send a signal along the nerve or to the muscle.
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A Needle Exam — a recording technique that detects electrical activity in muscle fibers using a needle electrode.
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Magnetic Resonance Imaging (MRI) — uses a magnetic field and radio waves to produce detailed images of the brain and spinal cord.
Blood and urine tests may be performed based on the person’s symptoms, test results, and findings from a neurological exam. In some cases, a spinal tap (lumbar puncture) may be performed to obtain the fluid that surrounds the brain and spinal cord called cerebrospinal fluid (CSF) for additional testing. A physician may order these tests to eliminate the possibility of other diseases. A muscle biopsy may be performed to help determine whether the person may have a muscle disease other than ALS.
Care Giver
As the person with ALS progresses in their disease, they will need more and more help with daily activities. Being a caregiver for a person with ALS, while rewarding, can be challenging for the person’s loved ones and caregivers. It is important for caregivers take care of themselves and to seek support when needed. Free and paid resources are available to provide home health care services and support.
Visit the organizations listed on our site to find support in your area.