ALS (Amyotrophic Lateral Sclerosis)

You may have heard of Lou Gehrig’s disease, which is really ALS. It’s often called this because the famous baseball player of the 1920s and 30s had it. His openness about having ALS made people more aware of it.

So what does it mean to have ALS? It affects your motor neurons. These are nerve cells that control your voluntary muscle movements (ones you choose to make), like chewing, swallowing, talking, walking and reaching. Upper motor neurons in your brain and spinal cord send signals to the lower motor neurons in your brain stem (lower part of your brain) and spinal cord. The lower ones pass these signals along to your muscles and tell them to move.

When you have ALS, these motor neurons start to break down, affecting the signals for voluntary movements. This can happen over time or quickly. No one experiences ALS in the same way, but it usually affects both kinds of neurons, causing weakness. Damage to your upper motor neurons will cause your muscles to stiffen. Problems with your lower motor neurons can cause you to lose muscle tone.

Eventually your muscles will start to waste away (atrophy), making it hard to do even simple, everyday things. As ALS progresses (worsens), it can also affect the muscles that help you breathe, and it can eventually cause your lungs to stop working (respiratory failure).

Types of motor neuron diseases (MND)

There are different types of motor neuron diseases. Each affects your motor neurons in different ways. If you’re diagnosed with classical ALS, it means both your upper and lower motor neurons are declining. It’s the most common form of motor neuron disease.

If you have progressive bulbar palsy (PBP), it usually starts with a breakdown of the upper and lower motor neurons that affect your mouth and throat (bulbar). You’ll first start to notice problems with chewing, speaking and swallowing. Sometimes, it will then move to the rest of your body. This is called bulbar-onset ALS.

The other two types of MND each affect only one kind of motor neuron. Progressive muscular atrophy (PMA) causes your lower motor neurons to deteriorate, while primary lateral sclerosis (PLS, the rarest form of ALS) causes a decline in your upper motor neurons.

We also divide ALS into two categories. Sporadic ALS — the type we see most often —happens randomly with no apparent cause, risk factor or family history of the disease. The other category, familial (genetic) ALS, means you’ve inherited the disease through a genetic change (mutation) from one or both of your parents.

We can’t say exactly why you may have amyotrophic lateral sclerosis. The causes are still unknown. And this — combined with the fact the disease’s symptoms overlap with other neurological conditions — can sometimes make it difficult to diagnose.

That’s why it’s important to have healthcare providers who have firsthand experience with ALS every day. Our dedicated ALS team recognizes how it’s different from other movement disorders and diagnoses hundreds of people with ALS each year.

What to expect at your first visit

We’ll start by sitting down and talking with you about what’s been going on. We’ll ask about your medical history and if anyone in your family has had amyotrophic lateral sclerosis or another MND.

We’ll want to hear about your symptoms — how long you’ve had them, what makes them get better or worse and if they’ve worsened steadily since you first noticed them.

Then, we’ll do a physical exam to check your general health and see how these symptoms are affecting you. We’ll also do a neurological exam so we can see how your brain, nerves and spinal cord work.

We might do some bloodwork and have you give us a urine (pee) sample to check your cells, hormone levels and other things that could signal problems in your body.

You can expect us to do some other tests, too, so we can see if you have any injuries or rule out other conditions with the same or very similar symptoms as ALS. These tests might include:

• Electromyogram (EMG): This test helps us measure the electrical activity in your nerves and muscles. A nerve conduction study tests your nerves’ ability to send signals from your brain.
• MRI: This imaging test helps us get a closer look at your brain or spine to see if there are any damaged areas.
• Lumbar puncture (spinal tap): A lumbar puncture lets us get a sample of the fluid that surrounds your brain and spinal cord to check it for signs of problems.
• Biopsy: We might take a tissue sample of your muscles or nerves (biopsy) in affected areas to see what’s going on with them. A pathologist will study these samples under a microscope.

Second opinions for ALS

When you find out you have ALS, it’s important you feel confident about the healthcare providers you work with. You want to be sure you trust them completely. That’s why we encourage second opinions. We want you to have peace of mind knowing you’re in the most capable, compassionate hands.

Our expert ALS team will take the time to help you better understand what’s going on and explain your personalized treatment options. We make sure you’re the center of all that we do — and because we know that motor neuron diseases don’t wait — we’ll try to get your appointment with us scheduled as soon as possible.

Your ALS care team

The providers you’ll work with at Cleveland Clinic treat ALS every day. Your care team will include different skilled providers from different specialties. Each are experts in the different types of ALS and its many symptoms. Your team could include:

• Neurologists.
• Radiologists.
• Pulmonologists.
• Speech-language therapists.
• Physical therapists.
• Occupational therapists.
• Respiratory therapists.
• Psychologists.
• Social workers.
• Home care and hospice providers.

ALS Treatment at Cleveland Clinic

ALS is a chronic (lifelong) condition, so we can’t completely get rid of your weakness and muscle damage. But personalized treatment can help slow down the disease’s progression and keep you feeling comfortable, mobile and independent for as long as possible.

Medications

We have extensive experience with the drugs approved to treat ALS. Riluzole (Rilutek^®) may help reduce damage to your neurons and extend survival by a few months. Edaravone (Radicava^®) and sodium phenylbutyrate/taurursodiol (Relyvrio^®) can slow progressive decline in how you handle daily tasks. We may also prescribe medications to help you manage muscle cramps, stiffness, extra saliva (spit), sleep issues, pain, depression and constipation.

Therapy

Our physical therapists will teach you exercises to help you stay strong and flexible and to ease muscle stiffness and spasms. If you’re up to it, walking and swimming are great ways to do this. So are stretching and range of motion exercises.

Occupational therapists can help you find ways to do your daily activities and stay safe. We might recommend you learn how to use grab bars, reach devices, wheelchairs, splints and braces to help you stay as independent as possible.

If you have trouble speaking, our speech therapists can show you ways to speak loudly and clearly and keep communicating, including nonverbal ways and with devices, like a computer speech synthesizer. They also can help you with swallowing issues.

Nutrition

You might find you lose weight quickly if you have ALS. Our team can help you and your caregiver plan a healthy, balanced diet so you can get the nutrition you need. We’ll also help you choose foods that are easier to swallow. When needed, we can teach you how to use suction devices to remove excess fluids to prevent choking. If you find you can no longer swallow to eat, we offer tube feeding to make sure you get enough nutrition and avoid complications.

Breathing support

Over time, amyotrophic lateral sclerosis can start to affect how your lungs work. We offer noninvasive ventilation (NIV) options, like an oxygen mask, to wear over your nose and mouth to help with shortness of breath. We may also recommend devices to help you cough. At some point, you may need help breathing. If this happens, you’ll have mechanical ventilation (respirator). We’ll work with you and your family to help you make the best decisions.

Supportive care

When you have ALS, it’s important to have healthcare providers who offer more than medical care. You and your family will need support, education and guidance as you navigate each step and cope with the effects of ALS on you and your family. We’re here for you. We make sure you get the help you need. We offer:

• Counseling to help you control the anxiety and depression that can come with managing ALS.
• Genetic counseling to help us find out whether you and your family members should get tested for the genetic mutation that causes ALS.
• Support for your family and others who may help with your care.
• Support groups to help you cope with an ALS diagnosis and connect with other people living with ALS.
• Financial planning so you can plan for the costs of care and make sure your family has enough resources now and in the future.
• End-of-life planning to help you make decisions about treatment and document those decisions for your care team and family.

Taking the Next Step

Finding out you have amyotrophic lateral sclerosis can leave you wondering about a lot of things. But one thing is certain — you’ll want to have a strong, caring team of experienced healthcare providers on your side. Ones who know what it takes to manage this disease. Ones to cheer you on and help you find the best ways to stay mentally and physically strong and independent for as long as you can. Our dedicated, compassionate ALS providers want to be on your team and help you throughout this entire journey.