ALS
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Amyotrophic lateral sclerosis (ALS) - a motor neurone disease is a rare condition that progressively
damages parts of the nervous system. This leads to muscle weakness, often with
visible wasting.
Motor neurone disease, also known as
amyotrophic lateral sclerosis (ALS), occurs when specialist nerve cells in the
brain and spinal cord called motor neurones stop working properly. This is
known as neurodegeneration.
Motor neurones control important muscle activity,
such as:
Gripping
Walking
Speaking
Swallowing
Breathing
As the condition progresses, people with
motor neurone disease will find some or all of these activities increasingly
difficult. Eventually, they may become impossible.
C
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auses of amyotrophic lateral sclerosis
It's not clear what causes motor neurones
to stop working properly. In about 5% of cases there's a family history of
either motor neurone disease or a related condition called frontotemporal
dementia. This is known as familial motor neurone disease. In most of these
cases, faulty genes have been identified as making a major contribution to the
development of the condition.
D
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iagnosis of amyotrophic lateral sclerosis
There's no single test to diagnose motor
neurone disease and diagnosis is based mainly on the opinion of a brain and
nervous system specialist (a neurologist). The diagnosis of motor neurone
disease is usually clear to an experienced neurologist, but sometimes
specialised tests are needed to rule out other conditions with similar
features.
S
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Ymptoms of amyotrophic lateral sclerosis:
Disease begin gradually over weeks and
months, usually on one side of the body initially, and get progressively worse.
Common early symptoms include:
A weakened grip, which can cause difficulty
picking up or holding objects
Weakness at the shoulder that makes lifting
the arm difficult
A "foot drop" caused by weak
ankle muscles
Dragging of the leg
Slurred speech (dysarthria)
The condition isn't usually painful.
As damage progresses, symptoms spread to
other parts of the body and the condition becomes more debilitating.
Eventually, a person with motor neurone
disease may be unable to move. Communicating, swallowing and breathing may also
become very difficult.
In up to 15% of cases, motor neurone
disease is associated with a type of dementia that can affect personality and
behaviour. This is called frontotemporal dementia, and is often an early
feature when it occurs in motor neurone disease. The affected person may not
realise that their personality or behaviour is different.
T
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reatment of amyotrophic lateral sclerosis.
There's currently no cure for motor neurone
disease. Treatment aims to:
Make the person feel comfortable and have
the best quality of life possible
Compensate for the progressive loss of
bodily functions such as mobility, communication, swallowing and breathing
For example, a breathing mask can greatly
help the symptoms of breathing and weakness, and a feeding tube (called a
gastrostomy) helps maintain nutrition and overall comfort. If necessary,
medication can be used to help control excessive drooling.
P
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A medication called riluzole has shown a
very small improvement in patients' overall survival, but it isn't a cure and
doesn't stop the condition progressing.
rognosis of amyotrophic lateral sclerosis
Motor neurone disease is a severely
life-shortening condition for most people. Life expectancy for about half of
those with the condition is three years from the start of symptoms. However,
some people may live for up to 10 years, and in rarer circumstances even
longer.
Living with motor neurone disease is extremely
challenging and often a terrifying possibility before the diagnosis is made.
However, it's not necessarily as bleak as people imagine.
With strong community and specialist support,
many people can maintain some independence for a significant part of the
condition's course, and experience a quality of life they may not have imagined
was possible at the time of their diagnosis.
The end of life for someone with motor neurone
disease isn't usually distressing and is most often in their own home. In most
cases, a person with the condition will die in their sleep as the end stage of
gradual weakness in their breathing muscles. Although some people with the
condition will have swallowing problems, they won't choke to death.
