Motor neuron disease (MND) refers to a group of progressive neurological disorders that cause damage to your motor neurons over time. Motor neurons are a type of nerve cell that control muscle movement.
When your motor neurons become damaged, they can’t transmit signals from your brain and spinal cord that produce muscle movement. As damage to your motor neurons worsens, you may experience trouble walking, talking, swallowing, or breathing. Eventually, this leads to disability and death.
Although there is no cure for MND, UPMC neurology experts can help you manage symptoms with medication, rehabilitation, supportive care, and mental health counseling.
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What Is Motor Neuron Disease (MND)?
Motor neuron disease (MND) is a group of progressive neurological disorders that cause damage to your motor neurons over time. Motor neurons are a type of nerve cell that control muscle movement.
When your motor neurons become damaged, they can’t transmit signals from your brain and spinal cord that cause your muscles to move. As damage to your motor neurons worsens, you may experience trouble walking, talking, swallowing, or breathing — eventually leading to disability and death.
What are the types of MND?
There are several types of MND, including:
- Amyotrophic lateral sclerosis (ALS) — Also known as Lou Gehrig’s disease, ALS is the most common MND. It affects motor neurons in your brain and spinal cord.
- Kennedy’s disease — Primarily affects males and causes weakness in the arms and legs, as well as pain and numbness in the hands and feet.
- Post-polio syndrome (PPS) — Occurs in polio survivors up to 40 years after recovery. It causes muscle weakness, fatigue, pain, muscle twitches, and cold intolerance.
- Primary lateral sclerosis (PLS) — Affects only the motor neurons in your brain. It causes symptoms that first appear in your legs, followed by your arms, hands, and torso, and finally in the muscles that control speaking, swallowing, and chewing.
- Progressive bulbar palsy (PBP) — Also known as progressive bulbar atrophy, PBP affects motor neurons in your spinal cord that connect to your brainstem. It causes problems with speaking, chewing, and swallowing.
- Progressive muscular atrophy (PMA) — Affects only the motor neurons in your spinal cord and occurs mainly in males. Symptoms include weakness in the hands that spreads to the lower body and torso.
- Spinal muscular atrophy (SMA) — An inherited genetic disorder that affects motor neurons in your spinal cord. It causes muscle wasting and weakness that leads to death in young children.
How common is MND?
MND is an uncommon condition. It affects about 5 to 9 people out of 100,000 in the U.S.
What causes MND?
MND occurs when there is damage to your motor neurons that gets worse over time. However, researchers are uncertain what causes the damage.
Some cases of MND are caused by genetic changes passed from biological parent to child. Other cases are caused by sporadic genetic changes or could be linked to environmental factors, viruses, or toxins.
MND risk factors
Risk factors for MND include:
- Being age 50 or older.
- Having a family history of MND or frontotemporal dementia.
If you have a family history of MND or frontotemporal dementia, your doctor may refer you to a genetic counselor to learn more about your risk for MND.
Complications of MND
Complications of MND may include:
- Breathing problems.
- Difficulty swallowing.
- Disability.
- Death.
What Are the Signs and Symptoms of MND?
MND symptoms develop gradually and typically occur after age 50. However, symptoms can start at any age and may include:
- Breathing difficulties, especially when lying down.
- Cognitive problems that affect thinking, concentration, and memory.
- Difficulty swallowing.
- Fatigue.
- Frequent chest infections.
- Lower body muscle weakness that causes difficulty walking and frequent falls.
- Morning headaches.
- Muscle cramps and twitching.
- Sleep difficulties.
- Slurred speech.
- Uncontrollable expressions of emotion, such as laughing or crying at inappropriate times.
- Weakness in the hands that causes you to have trouble holding things.
- Weight loss due to muscle loss (atrophy).
What are the first signs of MND?
Mild muscle weakness, tripping, dropping things, and slurred speech are often among the first signs of MND.
When should I see a doctor about my MND symptoms?
If you have symptoms of MND, you should schedule an appointment with your medical provider right away.
There is no cure for MND, but an accurate diagnosis can help you and your care team manage your condition and improve your quality of life.
How Do You Diagnose MND?
To diagnose MND, your provider will review your symptoms and medical history. They'll perform physical and neurological exams and may also order tests to help make a diagnosis or rule out other problems.
What to expect during your visit
During your visit, your provider will:
- Ask you about your symptoms.
- Review your medical history.
- Perform a physical and neurological exam.
- Order tests.
Tests to diagnose MND
There's no conclusive test to diagnose MND, and it can take time to receive an official MND diagnosis. However, as the condition worsens, it is possible to diagnose MND based on symptoms alone.
If your MND is in its early stages, your doctor may order tests to provide information or rule out other problems. These include:
- Blood and urine tests — Checks for abnormalities and rules out other conditions.
- Cerebral spinal fluid analysis (spinal tap or lumbar puncture) — Involves inserting a needle into your lower back to collect a sample of cerebrospinal fluid (CSF) for analysis in a lab.
- Electromyography (EMG) — Sometimes called an electromyogram, an EMG allows a doctor to record and study the electrical activity of your muscles.
- Genetic tests — Blood tests to look for signs of genetic changes related to MND.
- Magnetic resonance imaging (MRI) — Creates images of your brain and spine using contrast dye to look for damaged or abnormal areas.
- Nerve conduction study (NCS) — Measures the speed of electrical activity passing through your nerves.
- Neurological examination — A noninvasive test to check your reflexes, balance, movement, memory, and ability to think.
- Neuropsychological examination — Checks your ability to think, reason, and remember.
MND prognosis
MND is progressive, meaning that it gets worse over time. There is no cure for MND.
Your prognosis depends on how quickly your disease progresses. That may vary depending on the type of MND you have, your overall health, and other factors.
What is the life expectancy for MND?
MND will significantly shorten your life expectancy. Some people live only a few years after diagnosis, while others live for a decade or more. Your life expectancy may vary depending on the type of MND you have, your overall health, and other factors.
How Do You Treat MND?
There is no cure for MND. The goals of MND treatment are to manage symptoms, reduce your risk of complications, and improve quality of life. Treatment options may include:
Medication
Your doctor may recommend medications to manage symptoms, including:
- Antidepressants — Treat depression and anxiety.
- Baclofen — Treats muscle stiffness and spasms.
- Benzodiazepines — Treat pain.
- Glycopyrrolate — Treats drooling.
- Phenytoin or quinine — Treat muscle cramps.
If you are diagnosed with ALS, you may be a candidate for medication that can slow the progression of the disease.
Supportive care
Your doctor may prescribe treatments to manage symptoms and complications, including:
- Feeding and hydration support — This can help if MND affects the muscles that help you chew and swallow. IV fluids will prevent dehydration, and a nasogastric feeding tube will prevent malnutrition. Eventually, you may need to receive nutrition through a gastrostomy tube that is surgically placed in your abdomen.
- Mental health counseling — Counseling can help you and your loved ones cope with the challenges of an MND diagnosis. It can also help you manage mental health conditions, such as anxiety and depression.
- Respiratory therapy — If MND affects the muscles that control your breathing, your provider may recommend medications or mechanical ventilation to help you breathe.
Rehabilitation
You may receive different types of rehabilitation therapy to help you maintain strength, mobility, and independence. Rehabilitation treatment may include:
- Physical therapy — Maintains strength, balance, and mobility using exercises, stretching, massage, electrical stimulation, and other techniques.
- Occupational therapy — Teaches techniques and strategies to help you perform everyday activities, such as bathing, dressing, cooking, and grooming. sometimes with the assistance of a cane, brace, walker, or wheelchair.
- Speech and swallowing therapy — Helps you maintain the ability to swallow, chew, and speak.
Clinical trials
You may be eligible to take part in a clinical trial studying new treatments for MND. Your provider will let you know if you qualify for a clinical trial.
How effective is treatment?
MND is progressive, so your symptoms will get worse over time. However, treatment can help you manage your symptoms and improve your quality of life.
Why Choose UPMC for MND Care?
When you choose UPMC for MND care, you will receive:
- Access to advanced clinical trials — Our team participates in national, multicenter clinical trials evaluating new treatment options. This allows you to contribute to research advancing care for MND.
- Multidisciplinary care — Our team of neurologists will work with you and your loved ones to develop a customized treatment plan that optimizes your quality of life and reduces your risk of complications.
- Seamless, ongoing treatment — If you are diagnosed with MND, our team will develop a plan for your ongoing care that reflects your goals, preferences, and wishes.
