21 June: The Global Day for Amyotrophic Lateral Sclerosis
21 June: The Global Day for ALS
ALS is a degenerative neurological disease that leads to progressive
weakness. ALS does not discriminate on the basis of race, religion,
nationality, gender, or economic status. The cause of ALS is still
unknown and there is no cure...however, there is hope.
What is amyotrophic lateral sclerosis (ALS)?
ALS, often referred to as Lou Gehrig's disease or motor
neuron disease (MND)—is a progressive, degenerative
disease affecting motor neurons. Motor neurons are
specialized nerve cells that carry impulses from the brain to
the muscles by way of the brainstem and the spinal cord. The
muscles then move in response to these impulses.
In ALS, motor neurons gradually cease functioning and die.
As this happens, the muscle tissues waste away because no
movement is being stimulated. This results in gradually
worsening muscle weakness, atrophy and often spasticity.
Only the motor neurons are affected. Other nerve cells, such
as sensory neurons that bring information from sense organs
to the brain, remain healthy.
Who gets ALS?
ALS occurs throughout the world regardless of race, ethnicity
or socioeconomic status. Men are affected slightly more
frequently than women. It most commonly occurs between 40
and 70 years of age, although the disease can strike at any age.
How Common is ALS in the United States?
More than 5,600 Americans are diagnosed with ALS each year.
Approximately 35,000 people at any given time are living with
ALS in the United States. The incidence of ALS is close to that
of multiple sclerosis and four times that of muscular dystrophy.
What are the symptoms of ALS?
Some of the early symptoms of ALS might include:
Weakness or poor coordination in one limb
Changes in speaking or swallowing
Unusual muscle twitches, spasms or cramps
Unusual weight loss or loss of muscle bulk
Typically, ALS affects motor neurons in both the brainstem and spinal
cord. Symptoms related to the brainstem neurons (sometimes referred
to as “bulbar symptoms”) can include spasticity or stiffness in the
lower limbs, face or jaw. Feelings of heaviness, fatigue, stiffness and
lack of coordination are common. Reflexes may be very brisk or
Unprovoked outbursts of laughter or crying can occur, a condition
often referred to as “pseudobulbar affect” or “emotional lability.”
Symptoms related to the spinal cord neurons (sometimes referred to
as “somatic symptoms”) can include weakness, muscle wasting or
It is important to remember that ALS strikes people in different ways.
The symptoms and progression of the disease will be different from
person to person.
How is ALS diagnosed?
The diagnosis of ALS is a “clinical diagnosis,” meaning there is
no specific test for it. Often, tests will be administered to rule out
illnesses with similar symptoms. These may include an MRI of
the brain or spinal cord, an electromyography (EMG) study of
nerve and muscle function, and a variety of blood and urine tests.
After reviewing these test results and the patient's medical
history, and performing a complete neurological exam, a
neuromuscular specialist can usually reach a diagnosis. It may
take several months of observation and retesting to reach a
definitive diagnosis and that diagnosis should be confirmed via a
second opinion from another neuromuscular specialist.
What is the prognosis of ALS?
ALS progresses at different rates in each individual. The average
survival for someone affected by ALS is three to five years. Fifty
percent of those affected pass away within five years of diagnosis.
A small percentage may live 10 years or more.
As the disease progresses, the patient usually experiences a
decline in speech, swallowing and limb strength and function.
Generally, ALS is not a physically painful condition, though
discomfort can result from immobility and muscle shortening. The
ALS patient usually remains alert and retains normal sensation,
vision, bowel and bladder function. While most patients do not
have loss of intellectual function, some may have subtle changes
in mood, behavior or personality. In a small minority of patients,
more significant changes in behavior and judgment suggest a form
What causes ALS? Is ALS inherited?
Approximately 10 percent of all ALS cases are inherited forms,
known as “familial ALS.” Several genes have been identified
that cause familial ALS. The remaining 90 percent of cases are
called “sporadic ALS.”
In 2011, researchers, funded in part by the Les Turner ALS
Foundation, identified a common cause of all forms of ALS: a
broken-down protein “recycling system” in the neurons of the
spinal cord and brain. Additional research is needed to
determine how best to treat this faulty pathway.
Life with ALS.....the World from a Different Angle
“I am quite often asked: How do you feel about having ALS? The answer is, not a lot. I try to lead
as normal a life as possible, and not think about my condition, or regret the things it prevents me
from doing, which are not that many.” - Stephen Hawking
cast 21 June: The Global Day for Amyotrophic Lateral Sclerosis
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