What is ALS?

Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease, is a progressive neurological disorder affecting motor neurons, which are nerve cells in the spinal cord that control voluntary muscle movements. ALS disrupts communication between these motor neurons and muscles, causing damage and making it increasingly harder for muscles to function. Over time, this leads to loss of movement and muscle control.

ALS is considered rare, affecting about 9 in every 100,000 people in the US. Each year, approximately 5,000 new cases are diagnosed, according to the Centers for Disease Control and Prevention (CDC). While ALS can develop at any age, it most often begins between 50 and 75 and is more common in men than women.

There is no cure for ALS yet, but treatments can help manage symptoms, slow its progression and improve quality of life.

ALS symptoms

ALS symptoms can vary from person to person, depending on which nerve cells are affected. Early signs of ALS may include:

  • Muscle twitches in the arms, legs, shoulders or tongue
  • Muscle cramps
  • Stiff or tight muscles (spasticity)
  • Weakness in an arm, leg or the neck
  • Slurred speech or a nasal-sounding voice
  • Trouble chewing or swallowing

As the condition progresses, muscle weakness spreads and can make daily activities more difficult. People with ALS may experience:

  • Trouble chewing and swallowing food (dysphagia)
  • Excess saliva or drooling (sialorrhea)
  • Difficulty speaking clearly (dysarthria)
  • Shortness of breath (dyspnea)
  • Sudden, uncontrollable laughing or crying
  • Constipation
  • Trouble maintaining weight and getting enough nutrients

Over time, ALS affects movement more severely, making it impossible to walk, use the hands and arms or breathe without assistance. Most people maintain clear thinking and memory, but some may experience mild to moderate difficulties with language, decision-making or managing emotions, which can add emotional strain for people and their families.

When to see a doctor

See your primary care provider if you develop ALS-like symptoms, especially if a parent or someone else in your family has the condition. Your provider will refer you to a neurologist if they suspect ALS. A neurologist can confirm the diagnosis and coordinate with a team of other providers to deliver well-rounded care.

What causes ALS?

The exact cause of ALS remains largely unknown. The most common form, sporadic ALS, usually develops without a clear reason, and scientists are still working to understand the underlying causes. A smaller percentage of cases—about 5% to 10%—are classified as familial ALS, which is caused by inherited genetic mutations. In some instances, genetic changes may also contribute to sporadic ALS, even in the absence of a family history of the condition.

In addition to genetics, environmental factors may also play a role in the development of ALS.

Most cases of ALS appear to result from a complex interaction between genetic predispositions and environmental influences. Researchers continue to study these potential triggers in hopes of finding ways to prevent or slow the progression of ALS.

ALS risk factors

While the exact cause of ALS isn’t fully understood, certain factors can increase the likelihood of developing the condition. These include:

  • Age: ALS is very rare in people under 40, but the risk increases as you get older, especially between 50 and 75.
  • Sex: Men are more likely to develop ALS than women, though this difference tends to even out after age 70.
  • Genetics: In some cases, ALS runs in families due to inherited gene changes.
  • Lifestyle factors: Smoking is a known risk factor and can increase the chances of developing ALS.
  • Work-related factors: Certain jobs, like being in the military or working as a welder, may increase the risk due to injuries or exposure to harmful substances.
  • Environmental factors: Exposure to chemicals or heavy metals like lead and pesticides may also raise the risk of ALS.

Knowing these risk factors can help you understand your own chances of developing ALS, but it’s important to remember that having one or more of these factors doesn’t mean you will get the disease. Many people with risk factors never get ALS, and some people without any known risk factors do develop it.

If you're concerned about your risk—especially if you have symptoms or a family history of ALS—talking to your doctor is the best way to get personalized guidance and support. Your healthcare provider can help you better understand your individual risk and any steps you can take to protect your health.

Complications

As ALS progresses, muscle weakness and loss can lead to various complications that impact your life—including your physical and mental health, independence and mobility. While these changes can be challenging, working closely with your medical team, caregivers and loved ones can help you navigate complications and get the support you need.

Here are some potential complications of ALS:

  • Aspiration: Difficulty swallowing can cause food or liquid to enter your airway and reach your lungs.
  • Depression and anxiety: The emotional toll of ALS may lead to feelings of sadness, stress or being overwhelmed.
  • Loss of independence: As daily tasks like eating, dressing and bathing become harder, you may need assistance from a caregiver.
  • Pneumonia: If food or liquid enters your lungs, it can increase the risk of developing pneumonia.
  • Pressure sores: Prolonged sitting or lying down may irritate your skin, leading to sores.
  • Respiratory failure: Weakening respiratory muscles can make breathing difficult, and eventually, you may need assistance.
  • Weight loss: Chewing and swallowing difficulties, along with a reduced appetite, can make it hard to maintain weight.

While these complications can be overwhelming, planning ahead and seeking medical and emotional support can help you manage symptoms and maintain the best quality of life possible.

How is ALS diagnosed?

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Diagnosing ALS can be challenging because no single test can confirm the condition. Instead, healthcare providers rely on a thorough clinical examination and a series of diagnostic tests to rule out other conditions that mimic ALS before making a diagnosis. To get a clear picture of what’s happening, your doctor may recommend one or more of the following tests.

Medical history and exam

Your doctor will ask about your medical history, including any health conditions in your family. If a relative has had ALS, be sure to mention it. You’ll also discuss your symptoms—when they started, which parts of your body they affect and whether they’ve worsened over time.

During a physical and neurological exam, your doctor will check for signs of nervous system problems. This may include testing your reflexes, muscle strength, coordination, walking ability and speech.

Electrodiagnostic tests

Electrodiagnostic tests help doctors evaluate how well your nerves and muscles are working. These tests can detect nerve or muscle damage, which may point to ALS or another condition.

  • Electromyogram (EMG): A small needle is inserted into different muscles to record their electrical activity at rest and during movement. This helps determine whether the issue is with the muscles themselves or the nerves controlling them.
  • Nerve conduction study: This test measures how well your nerves send signals to your muscles. It helps identify nerve damage and is almost always done alongside an EMG.

Lab tests

Lab tests help doctors rule out other conditions that might be causing your symptoms. While there isn’t a single blood or urine test to confirm ALS, these tests can still provide important clues.

Your doctor may check for serum neurofilament light (NfL) levels, a marker of nerve damage that is usually higher in people with ALS. Testing for NfL can help doctors diagnose ALS earlier and distinguish it from similar conditions.

Although lab tests alone can’t confirm ALS, they are an important piece of the puzzle in understanding your symptoms and guiding the diagnosis.

Imaging tests

Imaging tests help doctors take a closer look at your brain and spinal cord to rule out other conditions that may be causing your symptoms. One of the most common imaging tests used is a magnetic resonance imaging (MRI) scan.

An MRI scan can help identify conditions like spinal tumors, herniated discs in the neck or other issues that may be contributing to your symptoms. In some cases, MRI scans with high-resolution cameras can even show early signs of ALS changes. While an MRI alone can’t confirm ALS, it plays an important role in helping doctors rule out other potential causes.

Biopsies

In some cases, doctors may need to run additional tests if your symptoms don’t follow the typical pattern of ALS. Biopsies and spinal fluid tests can help rule out other conditions that might be causing your symptoms.

  • Muscle or nerve biopsy: Your doctor removes a small sample of muscle or nerve tissue to check for signs of disease under a microscope. This can help identify other nerve or muscle conditions that may look like ALS.
  • Spinal tap (lumbar puncture): A thin needle is placed into the lower back to collect a small amount of fluid that surrounds the brain and spinal cord. This fluid is tested for infections or other issues that could be affecting your nervous system.

These tests aren’t always needed, but they can provide helpful answers when symptoms are unclear.

Amyotrophic lateral sclerosis treatment

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While there’s no cure for ALS, treatment can help slow the condition, manage symptoms and improve your quality of life.

Caring for ALS takes a team. Your neurologist, primary care doctor and specialists—such as physical therapists, speech therapists and dietitians—will work together to support you. They may recommend medications to slow the disease, therapies to help you stay active and supportive care for breathing, eating and daily tasks.

Although ALS is a challenging condition, the right care and support can help you maintain independence and live as fully as possible.

Medication

Medications can help with ALS in two ways: some treat symptoms like muscle cramps, pain and depression, while others may slow the condition. These include:

  • Edaravone: Taken by mouth or injection, edaravone slows nerve damage, which can help you maintain your ability to do everyday tasks.
  • Riluzole: This oral medication blocks glutamate, a chemical messenger that, at high levels, may damage nerve cells.
  • Tofersen: Given as a spinal injection, this medication is for people with a genetic form of ALS (caused by an SOD1 gene mutation) and may help slow nerve damage.

Rehabilitation therapy

Rehabilitation therapies can help you stay active and independent for as long as possible. These include:

  • Occupational therapy: You’ll learn ways to make daily tasks—like dressing, bathing and eating—easier. Your therapist may also recommend assistive tools, like special utensils or grab bars.
  • Physical therapy: Physical therapists can teach you low-impact exercises that can help you strengthen your muscles and maintain mobility and independence.
  • Speech therapy: If ALS affects your speech or swallowing, a speech therapist can teach you strategies to improve communication and make eating safer.

Supportive care

As ALS progresses, you may need extra support with breathing, eating and feeling comfortable.

  • Breathing support: If breathing becomes difficult, a noninvasive ventilator (a machine that delivers air through a mask) can help. Later, some people may need a mechanical ventilator to assist with breathing.
  • Nutritional support: Chewing and swallowing can become harder over time. A dietitian can help adjust your diet to make eating easier and ensure you’re getting enough nutrition. In some cases, a feeding tube may be recommended.
  • Palliative care: This type of care focuses on comfort and quality of life, helping with pain management, emotional well-being and support for both you and your loved ones.

Find specialized ALS care near you

If you or a loved one has been diagnosed with ALS, finding the right care and support is essential. Baylor Scott & White offers ALS care at various locations throughout North and Central Texas, providing access to specialized treatments, rehabilitation therapies and support services.

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Frequently asked questions

  • How common is amyotrophic lateral sclerosis?

    Amyotrophic lateral sclerosis (ALS) is a rare condition that affects about 9 out of every 100,000 people in the US. Each year, around 5,000 new cases are diagnosed. ALS usually develops between ages 50 and 75 and is more common in men than in women.

  • Is there a cure for amyotrophic lateral sclerosis?

    There is no cure for ALS, and no treatment can reverse nerve damage. However, some treatments can slow the disease’s progression, improve quality of life and extend survival by managing symptoms and providing supportive care.

  • Is ALS genetic?

    About 5 to 10% of ALS cases run in families due to genetic changes in certain genes. The rest of the time, ALS develops without a family history, but genetics may still play a role. Genetic testing can help you understand your risk if ALS runs in your family.

  • How fast does ALS progress?

    The speed at which ALS progresses can vary from person to person. On average, people live 3 to 5 years after diagnosis, though some live longer. Factors like age, the type of ALS, genetics and overall health can influence how quickly symptoms develop and change. Everyone’s experience is different.

  • Is ALS Lou Gehrig's Disease?

    Yes, ALS (Amyotrophic Lateral Sclerosis) is also known as Lou Gehrig's disease, named after the famous baseball player who was diagnosed with it.

  • Does ALS affect the brain?

    ALS primarily affects motor neurons controlling muscle movements. Some people experience mild to moderate cognitive changes in language, decision-making or emotional regulation.

  • Is ALS an autoimmune disease?

    No, ALS is not an autoimmune disease. It involves progressive damage to motor neurons rather than the immune system attacking the body.

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