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Tuesday, November 19, 2019

Amyotrophic lateral sclerosis | Symptoms Amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis | Symptoms Amyotrophic lateral sclerosis: What is ALS? What Are the Main Types of ALS? Signs and symptoms Amyotrophic lateral sclerosis, Causes of Amyotrophic lateral sclerosis



Amyotrophic lateral sclerosis (ALS)

Amyotrophic lateral sclerosis (ALS) is the most common
degenerative disease of the motor neuron system. Although ALS is incurable and
fatal, with median survival of 3 years, treatment can extend the length and
meaningful quality of life for patients.

What Is Amyotrophic lateral sclerosis disease?


ALS is short for amyotrophic lateral
sclerosis. You might also have heard it called Lou Gehrig's disease, after the
baseball player who was diagnosed with it in the 1930s. A French doctor named
Jean-Martin Charcot discovered the condition in 1869.

ALS is a progressive
disease, which means it gets worse over time. It affects nerves in your brain and
spinal cord that control your muscles. As your muscles get weaker, it gets
harder for you to walk, talk, eat, and breathe.

What Are the Main
Types of ALS?

 

There are two types
of ALS;

  1. Sporadic ALS is
    the most common form. It affects up to 95% of people with the disease.
    Sporadic means it happens sometimes without a clear cause.
  2. Familial ALS (FALS) runs
    in families. About 5% to 10% of people with ALS have this type. FALS is
    caused by changes to a gene. Parents pass the faulty gene to their
    children. If one parent has the gene for ALS, each of their children will
    have a 50% chance of getting the gene and having the disease.


Signs
and symptoms
Amyotrophic lateral sclerosis

 

The symptoms of ALS usually
appear when a person is in their late 50s or early 60s, but it can
happen at other ages.
Progression varies between
individuals. In the early stages, signs and symptoms may be barely noticeable,
but the weakness becomes more visible over time.

What are the symptoms of amyotrophic lateral sclerosis?
12 Common
ALS symptoms

  1. difficulty carrying out daily activities,
    including walking
  2. increased clumsiness
  3. weakness in the feet, hands, legs, and
    ankles
  4. cramping and twitching in the arms,
    shoulders, or tongue
  5. difficulty maintaining good posture and
    holding the head up
  6. uncontrolled outbursts of laughing or
    crying, known as emotional lability
  7. cognitive changes
  8. slurring of speech and difficulty with
    voice projection
  9. pain
  10. fatigue
  11. problems with saliva, and mucus
  12. difficulty breathing and swallowing, in
    the later stages
Other symptoms
·        
Progressive muscle weakness
occurs in all cases of ALS, but this may not be the first indication of the
condition.
·        
Early symptoms often include
clumsiness, abnormal limb fatigue, muscle cramps and twitches, and slurred speech.
Symptoms will spread to all parts of the body as ALS progresses.
·        
Some people may have problems
with decision-making and memory, eventually leading to a form of dementia called
front temporal dementia.
·        
Emotional lability can cause
fluctuations in mood
and emotional response.



What is Motor
Neuron?


Motor neurons of the spinal
cord are part of the central nervous system (CNS) and connect to muscles,
glands and organs throughout the body.

 

ALS upper and
lower motor neurons

 

ALS
is a disease that affects your motor neurons. These nerve cells send messages
from your brain to your spinal cord and then to your muscles.

You have two main types Motor
Neuron
:
  1. Upper motor neurons: Nerve
    cells in the brain.
  2. Lower motor neurons: Nerve
    cells in the spinal cord.

These motor neurons control all your voluntary
movements the muscles in your arms, legs, and face. They tell your muscles to
contract so you can walk, run, pick up your smartphone, chew and swallow food,
and even breathe.

ALS is one of a few
motor neuron diseases. Some others include:
·        
primary lateral sclerosis (PLS)
·        
progressive bulbar palsy (PBP)
·        
pseudo bulbar palsy

































Where
does amyotrophic lateral sclerosis affect the body?

 

With ALS, motor neurons in your brain and
spinal cord break down and die. When this happens, your brain can't send
messages to your muscles anymore. Because the muscles don't get any signals,
they become very weak. This is called atrophy. In time, the muscles no longer
work and you lose control over their movement.

At first, your muscles get weak or stiff. You may have more trouble with
fine movements such as trying to button a shirt or turn a key. You may stumble
or fall more than usual. After a while, you can't move your arms, legs, head,
or body.

Eventually, people with ALS lose control of
their diaphragm, the muscles in the chest that help you breathe. Then they
can't breathe on their own and will need to be on a breathing machine. The loss
of breathing causes many people with ALS to die within 3 to 5 years after
they're diagnosed. Yet some people can live more than 10 years with the
disease.
People with ALS can still think and learn. They have all of their senses
-- sight, smell, hearing, taste, and touch. Yet the disease can affect their
memory and decision-making ability.

ALS isn't curable. Yet scientists now know
more about this disease than ever before. They are studying treatments in clinical
trials.

What Causes Amyotrophic lateral sclerosis?

 

Researchers still don't know exactly what
causes motor neurons to die with ALS. Gene changes, or mutations, are behind 5%
to 10% of ALS cases. More than 12 different gene changes have been linked to
ALS.

One change is to a gene that makes a protein called
SOD1. This protein may be toxic to motor neurons. Other gene changes
in ALS might also damage motor neurons.

Environment could also play a role in ALS.
Scientists are studying whether people who come into contact with certain
chemicals or germs are more likely to get the disease. For example, people who
served in the military during the 1991 Gulf War have gotten ALS at higher rates
than usual.

Scientists are also looking at these other possible causes;
  • Glutamate. This chemical sends signals to and from the brain
    and nerves. It's a type of neurotransmitter. With ALS, glutamate builds up
    in the spaces around nerve cells and may damage them. The medications riluzole (Rilutek)
    works by lowering glutamate levels and can help slow the development of
    the disease.
  • Immune system
    problems. 
    Your immune
    system protects your body from foreign invaders such as
    bacteria and viruses
    . In your brain, microglias are the main type of
    immune cell. They destroy germs and damaged cells. With ALS, microglia
    might also destroy healthy motor neurons.
  • Mitochondria
    problems. 
    Mitochondria
    are the parts of your cells where energy is made. A problem with them
    might lead to ALS or make an existing case worse.
  • Oxidative stress. Your cells use oxygen to make energy. Some of
    the oxygen your body uses to make energy may form into toxic substances
    called free radicals, which can damage cells. The medication edaravone (Radicava)
    is an antioxidant that can help control these free radicals.
·        
Researchers learn more about ALS every day.
What they discover will help them develop medications to treat symptoms and
improve the lives of people who have this disease.

Amyotrophic lateral sclerosis (ALS)
Diagnosis

 

No single test can diagnose ALS,
so diagnosis is based on symptoms and the results of tests to rule out other
conditions with similar symptoms.
Tests that may help diagnose ALS
are:
  1. electromyography (EMG), which detects
    electrical energy in muscles
  2. nerve conduction study (NCS), which tests
    how well the nerves send signals
These tests can help rule out
peripheral neuropathy, or peripheral nerve damage, and myopathy, or muscle
disease.
1.       
An MRI scan can detect
other problems that could be causing symptoms, such as a spinal cord tumor or
a herniated disk in the neck.
2.       
Further tests to rule out other
conditions may include blood
and urine tests
and a muscle biopsy.

Medical problems that can produce similar symptoms to
ALS include HIV, Lyme disease, multiple sclerosis (MS),
the polio virus, and West Nile virus.

Conclusion
If there are symptoms in both the upper and lower
motor neurons, ALS may be present.
Upper motor neuron symptoms
include stiffness and resistance to movement in the muscles and brisk reflexes.
Lower motor neuron symptoms include weakness, muscle atrophy, and twitching.

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