Amyotrophic lateral sclerosis (ALS)
Find a neuro specialistLou Gehrig’s disease is named after a baseball player known for his strength and endurance who was diagnosed with it after a long career. Amyotrophic lateral sclerosis (ALS) is a chronic neuromuscular disease that causes weakness in voluntary muscles because it damages the motor neurons.
Voluntary muscles are the muscles responsible for body movements that you have conscious control over. Motor neurons are parts of the central nervous system (CNS) that originate and send messages from the brain and spinal cord to the voluntary muscles. As the motor neurons become more damaged, the muscles they affect start to waste away (atrophy).
Your senses (sight, touch, hearing, taste and smell) aren’t affected by ALS because they’re controlled by sensory neurons, not motor neurons. For some reason, movements of the eyes aren’t usually affected by ALS, so people with ALS retain the ability to look around. Other muscles not usually affected by ALS are the muscles of the bladder.
We don’t know what causes amyotrophic lateral sclerosis. Although there have been breakthroughs in ALS research that have made new therapies possible, researchers continue to search for a cause, a cure and more effective treatments.
ALS symptoms
ALS symptoms may affect all parts of the body. For some people, symptoms of ALS may start with the face, the legs or the arms. Common ALS symptoms that happen early in the disease are:
- Difficulty chewing or swallowing
- Dropping things
- Muscle cramps
- Muscle twitches in the arm, leg, shoulder, or tongue
- Muscle weakness
- Slurred or nasal speech
- Tight or stiff muscles (spasticity)
- Tripping
- Unusual fatigue
Amyotrophic lateral sclerosis is a progressive disease, which means symptoms get worse over time. When ALS symptoms worsen, you may have problems with:
- Breathing (dyspnea)
- Chewing food and swallowing (dysphagia)
- Constipation
- Drooling (sialorrhea)
- Maintaining weight and getting enough nutrients
- Speaking or forming words (dysarthria)
- Unintended crying, laughing, or other emotional displays (pseudobulbar symptoms)
People living with ALS disease eventually lose the ability to walk, write, speak, swallow and breathe. They also lose the ability to perform activities of daily living (ADLs) related to personal care such as:
- Dressing
- Eating
- Personal hygiene or grooming
- Toileting
- Transferring (getting in or out of a chair or bed)
People living with ALS sometimes have problems with using language or decision-making, although this is uncommon.
The average survival of people with ALS disease is 2-5 years after diagnosis. However, about 10% of people live for 10 years or more.
Diagnosis of Lou Gehrig’s disease
There’s no test to verify a diagnosis of amyotrophic lateral sclerosis. Because of this, part of the diagnosis involves ruling out other diseases that have similar symptoms.
Your doctor will perform a physical and talk to you about your potential ALS symptoms and your medical history. They’ll test your muscle strength, reflexes and other responses. Some of the tests they’ll order include:
- Blood and urine tests.
- Electromyography (EMG): Evaluates how well nerves and muscles are functioning. This test can include:
- 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.
- Needle exam: Recording technique that detects electrical activity in muscle fibers using a needle electrode.
- Magnetic resonance imaging (MRI): Uses a magnetic field and radio waves to produce detailed images of the brain and spinal cord.
- Muscle or nerve biopsy to rule out other diseases.
- Spinal tap (lumbar puncture) to gather cerebrospinal fluid (CSF) for testing.
It helps to get a confirmed diagnosis as soon as possible because ALS treatments are more effective in the early stages of the disease.
ALS treatments
Treatments for amyotrophic lateral sclerosis can’t reverse damage already done to your muscle neurons. ALS treatments focus on slowing damage, managing symptoms, extending survival and maintaining your quality of life.
Medications & amyotrophic lateral sclerosis
ALS medications that reduce or stop damage to motor neurons operate in different ways, such as:
- Blocking stress signals in cells to help prevent nerve cell death
- Decreasing levels of glutamate, which carries nerve messages
- Slowing functional decline
Symptoms like muscle cramps, pain, excessive saliva, sleep disturbances and depression may also be managed with ALS medications.
Rehabilitation therapy & ALS
Neurological rehabilitation and physical therapy are important parts of ALS treatment. Maintaining muscle strength, range of motion and the ability to balance helps maintain your health and outlook. Low impact exercises work best for people with ALS.
Occupational therapists provide strategies and suggest assistive devices to help with activities of daily living and maintaining independence.
Speech therapists work with you to help you retain clear speech. They’ll provide advice about how to choose breathing aids and speech generating apps or devices when they’re needed.
Assistive devices & ALS
We’ll help you find everything from devices to help with putting on socks to motorized wheelchairs. It’s important to make choices about some devices before you’ll need them because it can take time to set up things like wheelchair ramps. Making choices early also makes it easier for you to decide what will work best for you and your caregivers.
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