What Is It?
Amyotrophic lateral sclerosis, also known
as ALS, motor neuron disease or Lou Gehrig’s
disease, is a non-contagious disorder that
causes the death of neurons which control
voluntary muscles.
Motor neurons are nerve cells extending from
the brain to the spinal cord and to muscles
throughout the body.
They handle communication from the brain to
muscles that produce movements such as talking,
chewing or walking.
As ALS progresses, these neurons degenerate
and die off.
They stop sending signals to the muscles,
which, in turn, gradually weaken and become
atrophied.
In the final stages, the brain can no longer
initiate and control voluntary movement.
ALS is slightly more common in men than in
women and it usually occurs in people that
are between the ages of 40 and 70, with 55
being the average.
ALS is a truly horrifying disease, for a number
of reasons.
Sufferers gradually lose control of their
bodies and begin to struggle with the simplest
tasks.
Death by respiratory failure typically occurs
within 2 to 4 years after the onset of the
symptoms and researchers still don’t know
what causes ALS or how it can be cured.
Number 4 Mao Zedong
Mao Zedong was a Chinese communist revolutionary
who went on to become the founding father
of the People’s Republic of China.
He ruled as Chairman of China’s Communist
Party from 1949 until his death in 1976.
His theories, political policies and military
strategies are collectively referred to as
Maoism.
He is often regarded as one of the most important
and influential people in the modern history
of the world.
Mao was diagnosed with ALS in July 1974 and
gradually lost control of his motor functions
up until his death in 1976, at 82.
What Is It Caused By?
In the majority of ALS cases the cause isn’t
known, but the main suspects are genetic and
environmental factors.
There are about 20 genes associated with the
onset of familial ALS and, out of all of them,
a defect of the C9orf72 gene has been identified
in 40% of cases.
A number of other gene mutations have been
linked to ALS, including defects in how proteins
are broken down and used to build new ones.
A lot less is known about sporadic ALS, for
which there have been numerous studies yielding
various inconclusive culprits.
A 2016 study found a connection between the
disease and exposure to lead or other heavy
metals.
Other studies have cited smoking, strenuous
physical activity, head trauma and exposure
to toxic chemicals or pesticides as possible
contributing factors.
All of these potential causes have been extensively
examined and analyzed but, in most cases,
a definitive link was never proven between
them and the onset of ALS.
Number 3 Lou Gehrig
ALS is sometimes commonly referred to as Lou
Gehrig’s disease, after the American baseball
player that was diagnosed with it in 1939.
Known as the ‘The Iron Horse’ for his
batting average and longevity in the sport,
Lou Gehrig led the New York Yankees to six
World Series championships, between 1923 and
1939.
Throughout his 17 seasons in Major League
Baseball, Gehrig only played for the Yankees.
His records for most career grand slams and
for most consecutive games played went unbroken
for many years.
His game streak ended in May, 1939, when he
made the surprising decision to take himself
out of the lineup.
He had been feeling weak and unsteady.
His batting average had dropped and it was
evident that his coordination as well as his
ability to run the bases was off.
He retired from professional baseball at 36,
after he was diagnosed with the incurable
disease.
Upon his retirement, he made one of the greatest
speeches in sports history, known as the ‘Luckiest
Man on the Face of the Earth’ speech at
Yankee Stadium.
After his retirement, Gehrig wrote ‘I intend
to hold on as long as possible and if the
inevitable comes, I will accept it philosophically
and hope for the best.
That’s all we can do.’
He died at his home two years later.
When the news of his death broke out, flags
were flown at half-staff at ballparks across
the US.
For his achievements in the sport, the legendary
first-baseman would become the first MLB player
to have his uniform number retired by a team.
Today, the Lou Gehrig Memorial Award is given
each year to the MLB player who exhibits Gehrig’s
character and integrity.
How Will It Kill You?
Researchers don’t really know why neurons
die in ALS.
Neurodegeneration is believed to involve multiple
cellular and molecular processes.
They’re responsible for the death of upper
motor neurons, in the brain’s motor cortex,
and lower motor neurons, in the brainstem
and spinal cord.
Initial symptoms can include muscle twitches
in the leg, arm, shoulder or tongue.
Muscles may begin to develop cramps and become
tight or stiff.
Other symptoms involve difficulty chewing
or swallowing as well as slurred and nasal
speech.
The first sign may appear in one of the hands
and the sufferer may experience difficulty
buttoning a shirt, writing or a turning a
key.
Initial signs can also occur in a leg, with
the person tripping or stumbling more often
when they are walking or running.
As the disease progresses there will be problems
with breathing, moving, speaking or swallowing.
Although progression varies in each case,
the sufferer eventually won’t be able get
out of bed on their own, stand, walk or use
their hands and arms.
They’ll become malnourished because they
can’t swallow or chew and, also, people
with ALS burn calories faster.
The muscles responsible for respiration will
systematically become weaker, so breathing
will become more difficult.
Sufferers usually retain higher mental functions
so they’re aware of what’s happening to
them and may become depressed or anxious.
ALS is a nightmarish disease and only about
10% of people affected it by it survive more
than 10 years.
Number 2 Stephen Hillenburg
As a child in California, Stephen Hillenburg
developed a fascination for the ocean and
became interested in art.
In 1984, he began his professional career
as an instructor of marine biology at the
Orange County Marine Institute.
His passion for animation eventually led him
to enroll in the California Institute of Arts.
In 1994 he started to develop the concepts
and characters for what would become the ‘SpongeBob
SquarePants’ animated series.
The cartoon was a worldwide phenomenon and
earned Hillenburg two Emmy Awards and six
Annie Awards.
In addition to his work in animation, Hillenburg
also made efforts to raise marine life awareness.
In 2017, he disclosed in an interview with
Variety magazine, that he’d been diagnosed
with ALS.
He stated that he would continue to work on
the SpongeBob series for as long as possible.
He died at his home, in November 2018, of
cardiopulmonary failure.
His body was cremated and his ashes were scattered
in the Pacific Ocean, off the coast of California.
How to Survive?
There currently isn’t any known cure for
ALS and the treatments that exist are only
effective in managing certain symptoms or
prolonging life by a minor margin.
A medication called riluzole can extend life
by several months.
A feeding tube or mechanical ventilation may
help, but wont stop the disease from progressing.
The same is true for physical or speech therapy.
The Internet phenomenon known as the ALS Ice
Bucket Challenge managed to raise awareness
about the disease and $115 million for the
ALS Association, with the aim of funding research
into the disorder.
Scientists are currently trying to understand
the connection between ALS and some forms
of frontotemporal dementia or FTD.
In animal models, they’re studying the mutations
of specific genes associated with both disorders.
Research is being done to observe how cellular
defects affect the impairment of motor neurons.
Stem cell models are also being used to study
ALS.
These cells can become any cell type in the
body, including motor neurons and others that
may be involved in ALS.
Number 1 Stephen Hawking
British physicist, cosmologist and author
Stephen William Hawking is remembered as one
of the most brilliant scientific minds in
history.
He was diagnosed with ALS in 1963 and the
disease gradually paralyzed him.
At first he used crutches to get around but
was eventually confined to a wheelchair, for
the rest of his life.
He was able to communicate by using an electronic
speech-generating device operated by a single
functioning muscle in his cheek.
However, despite his physical limitations,
Hawking’s mind gifted the world a plethora
of scientific and mathematical theories.
Between 1979 and 2009, he was the Professor
of Mathematics at the prestigious University
of Cambridge.
He was the first to propose a theory of cosmology
through the union of quantum mechanics and
the general theory of relativity.
He worked with Sir Roger Penrose on gravitational
singularity theorems in the context of general
relativity.
He theorized that black holes emit radiation,
which would often be referred to as Hawking
radiation.
Hawking also published several works of popular
fiction, in which he discusses cosmology and
his own theories on the events that shaped
our universe.
His book, ‘A Brief History of Time’ was
aimed at non-specialist readers and was on
the Sunday Times list of best-selling books
for a record-breaking 237 weeks.
Among the numerous distinctions he received
for his work, Hawking was awarded the Presidential
Medal of Freedom, which is the highest civilian
distinction in the US.
He’s one of the very few people to have
lived with ALS for over 5 decades.
His battle with the disease ended on March
14, 2018, at age 76.
