Disease of Nerve Cells That Cause Movement

Amyotrophic lateral sclerosis is also known as ALS or Lou Gehrig’s disease. It is a disease of the nerve cells in the brain and spinal cord that control muscle movement. In a person with ALS, the cells that control muscles, called motor neurons, waste away and no longer send messages to the muscles. Over time, this leads to the muscles getting weak. The arms, legs, mouth and tongue muscles may all be affected. If the muscles of the chest and diaphragm weaken, it becomes hard or impossible to breathe. The experts at Penn State Health Milton S. Hershey Medical Center ALS Clinic provide a definite diagnose and offer complete care that helps a person with ALS function as normally as possible and have a better quality of life.

Our clinic is recognized by the ALS Association as a Certified Treatment Center of Excellence that evaluates and treats ALS and related conditions. About 80 new patients with ALS are diagnosed at the center every year and more than 230 are followed long-term at any given time.

What is ALS?

In ALS, the cells that control muscles, called motor neurons, waste away and no longer send messages to the muscles. Over time, this leads to the muscles getting weak. The arms, legs, mouth and tongue muscles may all be affected. If the muscles of the chest and diaphragm weaken, it becomes hard or impossible to breathe.

Causes of ALS

About one out of 10 cases of ALS are inherited. Having a close family member with ALS raises the chances of having the disease. Other risks include military service and heavy contact with some pesticides (insect killers). In most cases, the cause isn’t known.

ALS Symptoms and Diagnosis

Amyotrophic lateral sclerosis is also known as ALS or Lou Gehrig’s disease. This is a disease of the nerve cells in the brain and spinal cord that control muscle movement. The experts at Penn State Health Milton S. Hershey Medical Center ALS Clinic provide a definite diagnose and offer complete care that helps the person with ALS function as normally as possible and have a better quality of life.

While, in most cases, the cause isn’t known, about one in 10 cases of ALS is inherited. Risk factors include:

  • Having a close family member with ALS
  • Military service
  • Heavy contact with some pesticides

Symptoms

Symptoms of ALS usually don’t begin until after age 50, but they can also occur in younger people. People with ALS lose muscle strength and coordination over time. Weakness can first affect the arms or legs, or breathing and swallowing. This gets worse and eventually makes it difficult or impossible to perform normal tasks, like climbing stairs, getting out of a chair, swallowing or breathing.

ALS doesn’t affect the senses of sight, smell, taste, hearing and touch. Most patients are able to think normally, although a small number have dementia, which causes problems with thinking or changes in behavior.

Signs that a person may have ALS:

  • Trouble breathing
  • Trouble swallowing: choking easily, drooling or gagging
  • Head drop due to weak neck muscles
  • Muscle cramps
  • Muscle stiffness, which is called spasticity
  • Muscle twitching due to small contractions, which is called fasciculations
  • Muscle weakness that starts in one body part, such as the arm or hand, and slowly gets worse until it leads to trouble with lifting, climbing stairs and walking
  • Speech problems, such as a slow or different speech pattern (slurring of words, hoarseness)
  • Weight loss

Causes and Risk Factors

Possible complications of ALS:

  • Breathing food or fluid into the windpipe (aspiration)
  • No longer able to care for self
  • Respiratory (breathing) failure
  • Pneumonia
  • Pressure sores
  • Weight loss

If you suspect you have ALS, you will see a specialist called a neurologist, who is an expert in the diagnosis and treatment of nervous system diseases like ALS.

Diagnosis

The neurologist will perform a complete examination and review your medical history. Using that information, your specialist will order diagnostic tests, which may include:

  • Studies of blood and urine
  • X-rays
  • Computed tomography (CT) scans
  • Magnetic resonance imaging (MRI)

 Most patients will also have electrodiagnostic testing (EMG and nerve conduction tests) in our EMG laboratory. Other tests may include:

  • Lumbar puncture (spinal tap)
  • Muscle biopsy
  • Nerve biopsy

After tests are complete, the neurologist will make an appointment with you, your family and any other caregivers to talk about the diagnosis. If you have ALS, the doctor will give you information about your treatment plan, including proven and trial treatments.

An ALS nurse specialist is often at that visit as well. If not, you’ll schedule a follow-up visit with a nurse specialist. The neurologist and nurse work with you and your family on a plan to treat the disease and manage symptoms.

Patients with ALS are seen about once every three months in our multidisciplinary ALS clinic, which includes a team of specialists skilled in the treatment of this disease. During these appointments, you and your family stay in one examining room for about three to four hours while the different team members meet with you.

You will always have your weight and breathing capacity measured and see the neurologist and nurse. You will also see some or all of these team members.

  • Mental health specialist
  • Occupational therapist
  • Pastoral care counselor
  • Physical therapist
  • Registered dietitian 
  • Social worker
  • Speech therapist

The team members you will see during those visits depend on your specific needs. 

Patients should continue seeing their primary care doctor while using the ALS clinic's specialized services. After each ALS clinic visit, your primary care doctor gets a written update from your neurologist, including a summary of what the ALS team recommends.

Over time, most people with ALS can’t function and care for themselves. Death often occurs within two to five years after diagnosis, but this timeframe can vary. About one in five patients lives for more than five years after being diagnosed. Some patients live much longer, but they may need help breathing from a ventilator or other device.

Goals of the Penn State ALS Clinic

If you have ALS, the multidisciplinary team of experts at Penn State Health provides comprehensive care to address every aspect of your condition and reduce its impact on your quality of life.

Our Penn State ALS Clinic is recognized by the ALS Association as a Certified Treatment Center of Excellence that evaluates and treats ALS and related conditions. At the clinic, we:

  • Focus on special services for patients with ALS and related health problems.
  • Give each patient information about the disease process.
  • Offer therapeutic drug trials and opportunities to become involved in other research.
  • Serve as a center to diagnose, treat and provide follow-up care to patients with ALS.
  • Take a team approach to help patients function to their best ability.
  • Work with primary care doctors to treat the patient.

What to expect

Patients coming to us for the first time see a neurologist (nervous system doctor) who is an expert in ALS. The goal is to be as sure as possible about a diagnosis.

After doing a series of tests, the neurologist will make an appointment with you, your family and other caregivers to talk about the diagnosis. If you have ALS, the doctor will give information and talk about proven and trial treatments.

An ALS nurse specialist is often at that visit as well. If not, you’ll we’ll schedule a follow-up visit with a nurse specialist. The neurologist and nurse work with you and your family on a plan to treat the disease and manage symptoms.

Follow-up visits

Patients with ALS are seen about once every three months in our multidisciplinary clinic, which includes various specialists on our team. During these appointments, you and your family stay in one examining room for about three to four hours while the different team members meet with you.

At these visits, you will always have your weight and breathing capacity measured and see the neurologist and nurse. You will also see some or all of these team members

  • Physical therapist
  • Occupational therapist
  • Speech therapist
  • Registered dietitian 
  • Mental health specialist
  • Social worker
  • Pastoral care counselor

The team members you will see during those visits depends on your specific needs. 

Working with the primary care doctor

Patients should continue seeing their primary care doctor while using the ALS Clinic's specialized services. After each ALS Clinic visit, your primary care doctor gets a written update from your neurologist, including a summary of what the ALS team recommends.

Why Choose Penn State Health for Care

Compassionate, Comprehensive Care

We’ve been recognized by U.S. News and World Report as a high-performing specialty for consecutive years. Our medical team is consistently recognized nationally through Best Doctors in America and America’s Top Doctors awards. Our specialists also participate in worldwide conferences and speaking engagements in countries including India, Korea, Germany, Japan and others.

Telemedicine Program

In March 2015, our center launched a telemedicine program for people with ALS and their families. This program allows us to stay connected with our ALS patients, even when they can’t travel. Telemedicine visits occur through secure video conferencing over the internet between one or more health care providers at Penn State Health Milton S. Hershey Medical Center and a patient and his or her family and caregivers in their home. You can find more information on telemedicine visits for ALS here.

ALS OnDemand Appointment

Experts in Care

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Research and Clinical Trials

We strongly believe that research into what causes ALS and other motor neuron diseases will be the key to more effective treatments and a cure. We also believe that research can lead to a better understanding of the factors that maintain and improve quality of life for patients with ALS and their families, permitting the development of optimal care.

Learn more about ALS research and clinical trials

The Penn State ALS Clinic and Research Center is funded by the ALS Association Greater Philadelphia Chapter and by private donations from many individuals and groups.

We carry out our studies at two levels:

  • Clinical trials of medicines that show promise for treating ALS
  • Clinical research to improve the care and support of patients and their caregivers

Our Generous Supporters

We’re thankful for the support we receive from a variety of sources. Without them, the ALS Clinic couldn’t further its missions.

Convenient Locations

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