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Primer on ALS Terminology

ALS (Amyotrophic Lateral Sclerosis)
This is the most common form of adult inherited motor neuron disease and is synonymous with Lou Gehrig’s disease.

MND (Motor Neuron Disease)
The family of diseases that affect the nerves that control skeletal muscles, which are the muscles under voluntary control.

Upper Motor Neuron and Lower Motor Neuron
These nerves make up the motor system.  Every muscle cell has both an upper motor neuron and a lower motor neuron.  When the upper motor neuron is damaged, symptoms include slowness, stiffness and overly jumpy reflexes.  When the lower motor neuron is damaged, symptoms include weakness, atrophy, twitching (fasciculations) and absence of reflexes.

PLS (Primary Lateral Sclerosis)
An isolated upper motor neuron disease that often starts in the legs and is usually slowly progressive.

PMA (Progressive Muscular Atrophy)
An isolated lower motor neuron disease.

PBP (Progressive Bulbar Palsy)
A motor neuron disease that only affects the bulbar segment (area supplied by the cranial nerves, essentially above the neck).

ALS and Exercise

ALS related weakness and muscular fatigue are caused by the progressive loss of motor neurons that provide nerve supply to muscles.  There is no current information to suggest that exercise accelerates or retards this process. More research is needed to definitively answer this question. Meanwhile, we usually counsel patients to tread lightly with exercise. In general exercise will not make muscles weakened by ALS stronger. In fact these muscles are more susceptible to fatigue induced by exercise. While this will recover after a period of rest over minutes or hours it can result in a temporary increase in fatigue that may make arm or leg weakness transiently worse and result in temporary loss of function. This can make performing activities of daily living with the arms and hands temporarily more difficult. It can also make leg weakness transiently worse increasing the risk of falls.  Overall, common sense should prevail and the idea of “use it or lose it” should not be considered a valid concept in ALS.

Make an appointment at the Penn Comprehensive ALS Center

To make an expedited appointment with a Penn ALS neurologist, call 215-829-3053

FAQs

Are there exercises I can do to strengthen my breathing muscles (breathing exercises for ALS)?

Just as with other muscles in the body affected by ALS, exercise does not make the breathing muscles stronger.  But there are many things that can be done to support the respiratory muscles when they become weak.  The diaphragm is the main muscle of breathing.  When the diaphragm contracts, the lungs expand and fill with air.  Muscles are not needed to exhale the air – the lungs exhale by “elastic recoil,” like a rubber band that is stretched and then released.  We need muscles to exhale forcefully, for example with coughing.  The intercostal muscles (muscles between the ribs) and the abdominal muscles assist in exhaling forcefully when we cough.

Symptoms of a weak diaphragm often occur during sleep or when lying flat.  We all breathe less deeply and less frequently when we sleep.  If the diaphragm is weak, a person will take in less air during sleep, which then causes low oxygen levels and elevated carbon dioxide levels.  This can result in poor quality sleep with daytime headaches, fogginess, and sleepiness.  Breathing during sleep can be assisted with non-invasive ventilation, also called BiPAP or Trilogy, which are the names of machines that help with breathing. A mask is worn over the nose and the machine gently pushes air into the lungs, giving bigger breaths, which improves carbon dioxide and oxygen levels.

Another support machine is called a cough assist or inexsufflator.  It helps with the muscles needed for coughing and can be used if a person with ALS develops difficulty coughing up mucous when he or she has a cold or bronchitis.  Since getting a respiratory infection can be tough on a person with ALS, we recommend yearly flu shots as well as one time vaccine with Pneumovax (a vaccine that helps with prevention of pneumonia).  We also recommend avoiding close contact with friends or family members who have a cold.

Is there a connection between ALS and dental fillings?

It is not necessary or advisable to have fillings removed in the setting of ALS. There is no evidence to indicate that ALS is caused or influenced by the mercury contained in mercury amalgam fillings. We have had hundreds of patients pursue this painful and costly course of action and we have not once seen a benefit to the patient. The progression of ALS is not influenced by this procedure.

Is there a connection between Lyme disease and ALS?

ALS is not caused by infection with Borrelia burgdorferi, the tick born spirochete bacterium responsible for Lyme disease. Lyme is actually quite easy to diagnose by a simple blood test and the CDC has well laid out guidelines for the diagnosis of Lyme disease.  Symptoms of neurologic Lyme disease include muscle weakness that can mimic ALS, but also almost always include severe pain and sensory symptoms.  For Lyme to cause neurologic issues it must be present in the nervous system, which means that it can be seen either by evidence on MRI scan or in the cerebrospinal fluid from results of a spinal tap.  If infection with the Lyme agent cannot be confirmed by blood or CSF studies as detailed by the CDC, it is not advisable to undergo prolonged treatment with an oral or intravenous antibiotic regimen for Lyme disease. This can cause considerable side effects including the risk for infectious diarrhea as well as gallstones requiring gall bladder removal.

What kind of genetic testing should I have for ALS?

About 10% of ALS is caused by one of a group of genetic mutations. Known as Familial ALS (FALS), most FALS patients have a family history of ALS, Parkinson’s disease or Parkinsonism, young onset or atypical dementia or atypical psychiatric disease. Since the discovery of the C9ORF72 gene in 2011 it has become clear that a small percentage of ALS patients without a family history are also positive for this gene. Currently, knowing an individual patient’s genetic status does not influence any aspect of their clinical care but of course this information may affect related individuals including children, parents, siblings, and others who will be faced with a decision of whether or not to seek personal genetic testing.  Recent ALS clinical trials using molecular techniques that may be able to silence mutant ALS genes may influence the decision to be genetically tested since this knowledge may allow participation in these trials. The Penn ALS Center is one of only a few in the United States that have on site genetic counseling available for our patients and families.

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