A Guide for

Individuals and
Families

Amyotrophic Lateral
Sclerosis
Amyotrophic lateral sclerosis (ALS) is a progressive Each person’s disease course is unique, but eventually most
neuromuscular disease that destroys muscle-controlling individuals with ALS will not be able to stand or walk, get in or
nerve cells called motor neurons. out of bed on their own, use their hands and arms, or breathe
without assistance.
In ALS, motor neurons in the brain and spinal cord are
affected. As these nerve cells deteriorate and are lost, they The involuntary muscles, such as those that control the
stop sending signals to muscles. In the absence of signals, heartbeat, gastrointestinal tract and bowel, bladder and sexual
the muscles they control atrophy, becoming weak and then functions are not directly affected in ALS. Sensations, such as
nonfunctional. vision, hearing and touch, are also unaffected.

ALS is estimated to affect 5 out of every 100,000 people in In many cases, ALS does not affect a person’s thinking ability.
the United States. Between 15,000 and 20,000 Americans are However, as many as 50% of people with ALS develop some
estimated to be living with ALS in the U.S. degree of cognitive (thinking) or behavioral abnormalities.

Onset of ALS symptoms usually occurs in late middle age or Lifespan after an ALS diagnosis typically is 2 to 5 years.
later, although it also can occur in young adults, as well as in
the elderly. Currently, there is no cure for ALS and no effective treatment
to halt or reverse the progression of the disease. However,
ALS affects persons of all races and ethnicities, and men are there are treatments that may modify or slow the disease
slightly more likely to develop ALS than women. course, as well as therapies that can help control symptoms,
prevent unnecessary complications, and make living with
Some studies suggest that military veterans are about 1.5 the disease easier.
to 2 times more likely to develop ALS. ALS is recognized
as a service-connected disease by the U.S. Department of
Veterans Affairs.

Most ALS cases are sporadic, meaning there is no family

history of the disease. About 5 to 10 percent of cases
are familial, meaning the disease runs in the family.
Both familial and sporadic ALS can stem from genetic causes,
and some people who have a diagnosis of
sporadic ALS may carry ALS-causing genetic variations
that can be passed on to their children. A genetic counselor
can help people with ALS understand
inheritance and any associated risks for family members.

The causes of the vast majority of ALS cases are

still unknown.

ALS affects many parts of the body, most notably

the skeletal and respiratory (breathing) muscles.
 What are the signs and symptoms of ALS?

Nervous system Skeleton and muscle

• Impaired motor function • Muscle weakness
• Pseudobulbar affect • Fatigue
• Cramps
Cognition • Fasciculations
• Impaired decision-making • Spasticity
• Contractures
Behavioral • Dysarthria
• Anxiety
• Depression
Respiratory system
Gastrointestinal • Breathing difficulties
• Chest infections
• Dysphagia
• Respiratory failure
• Constipation
• Malnutrition

What should I know about ALS?

1 There is no test that can provide a 4 In the early stages of ALS, muscles may 6 In the late stages of ALS, most
definitive ALS diagnosis. Diagnosis become weak and soft or stiff, tight and voluntary muscles are paralyzed.
is based on a detailed history of spastic. Muscle cramping and twitching The muscles that help move air in
symptoms and signs observed (fasciculations) occurs, as does loss of and out of the lungs are severely
by a physician during physical muscle bulk. Symptoms may be limited compromised. Mobility is extremely
examination, along with a series to a single body region or may affect limited, and help is needed in
of tests meant to rule out other more than one region. The person may caring for most personal needs.
diseases that can mimic ALS. experience fatigue, poor balance, slurred Poor respiration may cause fatigue,
words, a weak grip, tripping when fuzzy thinking, headaches and
walking or other minor symptoms. susceptibility to pneumonia.
2 For many individuals the first sign Speech, or eating and drinking by
of ALS may appear in a hand or mouth may
arm, as they experience difficulty not be possible.
with simple tasks such as buttoning 5 Symptoms become more widespread
a shirt, writing, or turning a key in a in the middle stages of ALS. Some
lock. In other cases, symptoms muscles are paralyzed, while others are 7 In ALS, death typically results from
initially affect one of weakened or unaffected. Fasciculations respiratory failure.
the legs, and people experience may continue. Unused muscles may lead
awkwardness when walking or to contractures, in which the joints
running or they notice that they become rigid, painful and sometimes
are tripping or stumbling a lot. deformed. If a person falls, he or she
may not be able to stand back up without
help. Weakness in swallowing muscles
3 When symptoms begin in the may cause choking and greater difficulty
arms or legs, it is referred to as eating and managing saliva. Weakness in
limb-onset ALS. In bulbar-onset breathing muscles can cause respiratory
ALS individuals first notice speech insufficiency. Some people experience
or swallowing problems. bouts of uncontrollable and
inappropriate laughing or crying
(pseudobulbar affect).
 How is ALS treated?
The U.S. Food and Drug Administration Respiratory devices such as BiPAP
(FDA) has approved the drugs riluzole (bilevel positive airway pressure) can
(brand name Rilutek*), edaravome assist the movement of air in and out
(brand name Radicava*), thickened of the lungs. This support, delivered
riluzole (brand name Tiglutik*) and through a mask over the nose and/
Nuedexta to treat ALS. or mouth, is called noninvasive
• Rilutek is believed to protect motor ventilation (NIV). As ALS progresses and
neurons by decreasing levels of the muscles weaken further, individuals
neurotransmitter glutamate. Clinical may consider forms of mechanical
trials in people with ALS showed ventilation, in which a machine inflates
that Rilutek prolongs survival by a and deflates the lungs. Doctors may
few months, but does not reverse place a breathing tube through the
the damage already done to motor mouth or may surgically create a hole
neurons. at the front of the neck and insert a tube
leading to the windpipe (tracheostomy).
• Tiglutik is an oral suspension liquid
The tube is connected to a respirator.
formulation of riluzole. Exservan is Nutritional support is an important
an oral film formulation of riluzole. part of care.Nutritionists can teach
Developing new formulations of individuals and caregivers how
riluzole allows the drug to be easier to plan and prepare small meals An array of assistive
to take for individuals who have throughout the day that provide technology products can
difficulty swallowing. enough calories, fiber, and fluids, help maintain mobility
and how to avoid foods that are and independence even
• Radicava is thought to work when muscles become very weak.
by relieving the effects of oxidative difficult to swallow. Suction devices
may be used to remove excess Standers, walkers, various kinds
stress, which has been suspected to of powered and manual wheeled
play a role in the death of nerve cells fluids or saliva and prevent choking.
vehicles and wheelchairs can help
in people with ALS. Radicava people with ALS stand or move
has been shown to slow the decline around.
in clinical assessment of daily Muscle relaxants may reduce spasticity.
functioning in persons with ALS. Botulinum toxin may be used to treat
jaw spasms or drooling, and there are
• Nuedexta is approved for treatment medications that can be used to reduce
of pseudobulbar affect (PBA), a excessive saliva. Nuedexta can be
condition experienced by 15-45% of prescribed to help control pseudobulbar
ALS patients. The exact mechanism affect. Antidepressants and anxiolytics
of action of the drug is not known. may be helpful in treating depression
Nuedexta has been shown to reduce and anxiety, and other drugs may help
the rate of daily episodes in people with pain, sleep disturbances and
experiencing PBA. constipation.

Speech therapy can help A machine called the cough assist can
with difficulties speaking help to remove secretions from lungs.
or swallowing. Adaptive technology
can help with writing, art projects, A gastrostomy tube (sometimes
using a computer or cellphone, and called a g-tube or feeding tube) allows
electronically controlling the liquid nutrition to enter the stomach
environment. Computer-based directly,bypassing the mouth, throat
speech synthesizers that and esophagus, when weakness in the
use eye-tracking technology muscles of the throat makes chewing
can help with nonverbal or swallowing difficult and prevents
communication, and voice banking individuals from being able to get
allows patients enough nourishment from eating.
to store their own voice Physical therapy helps to restore and
for future use. maintain muscle strength and
function through exercise, as well as
to maintain range of motion through
stretching. Occupational therapy can
help people with ALS conserve energy
and remain mobile.

*Please talk to your medical provider to obtain

information about ALS treatments.
 MDA Glossary
Aspiration containing molecules) and the ability of
When food or liquid accidentally enters the body to counteract or detoxify their
the windpipe instead of the stomach harmful effects through neutralization
by antioxidants
Atrophy
A decrease in the size and mass Progressive
of muscle tissue Describes a disease in which the
symptoms get worse over time
Contracture
A shortening of muscles or tendons Pseudobulbar affect
around joints that can limit mobility Bouts of uncontrollable and
inappropriate laughing or crying
Dysarthria
Difficulty speaking or forming words Sialorrhea
Excessive saliva
Dysphagia
Difficulty swallowing Spasticity
An unusual tightness or
Dyspnea stiffness of muscles
Difficulty breathing
Variation
Fasciculations A flaw in the DNA code
Muscle twitching

Oxidative stress
An imbalance between the production
of free radicals (highly reactive oxygen-

To learn more about ALS, visit mda.org or contact the MDA

Resource Center at 833-ASK-MDA1 (275-6321).

Designated a Top-Rated Charity by the American Institute

of Philanthropy, MDA is the first nonprofit to receive a
Lifetime Achievement Award from the American Medical
Association for “significant and lasting contributions to the mda.org • 1-833-ASK-MDA1
health and welfare of humanity.” facebook.com/MDAOrg
@MDAorg

66-0024 July 2021