|Motor neurone disease (MND)
is a rare condition which is caused by the breakdown of the nerve
cells in the brain that control movement muscles. Unfortunately,
for now there is no cure, and most people with MND die from it
within a few years. However, much research is underway to understand
the causes, and eventually to find a cure.
MND usually begins between the ages of 50 and70, and is rare -
only about one person in 25,000 in the UK develops the disease
at some time in their lives.
It affects the movement muscles (voluntary muscles), but not the
nerves dealing with sensation, so there is no numbness or pins
and needles. The parts of the brain dealing with intelligence
and awareness also remain unaffected.
There are three main types of MND, depending on which nerves are
- Amyotrophic lateral sclerosis (ALS) which affects
about half of people with MND.
- Progressive muscular atrophy
- Bulbar palsy
The cause of MND isn't known. Some symptoms are like polio, so
it was originally thought to be due to a virus. However, no virus
has ever been discovered, and there's no evidence that it is contagious.
Some types run in families, so a genetic link has been suspected,
but even the most common genetic forms are incredibly rare, occurring
in only one in every 250,000 people.
MND is known is an autosomal recessive condition, which means
that both parents have to be carriers of the faulty gene to pass
it on to an affected child.
|MND usually begins very
gradually, and there may just be a feeling of tiredness to start
with. Clumsy fingers and a weak grip are often the first sign
of muscle problems. After a while, turning door handles becomes
difficult. This is usually followed by difficulties in speech
When the foot muscles are affected, raising the foot with each
step can be difficult, causing the feet to drag on the floor ('foot-drop').
The muscles of the chest wall may be affected, leading to breathing
difficulties and lung infections.
In bulbar palsy, the throat muscles are principally involved,
and difficulties in swallowing and speech may be the main features.
Patients with speech difficulties have a typically 'quacking'
Sometimes, the muscles can be seen to twitch (fasciculation),
and pain and stiffness can develop around any joint where the
muscles are affected.
Most people who have MND will die from it within three years.
As the disease progresses, artificial ventilation is often needed.
A tracheostomy - a surgical opening in the windpipe (trachea)
- is also sometimes carried out to make breathing easier. Despite
these measures, chest infections and pneumonia often cause complications,
and can lead to death.
There are some notable exceptions, however. Stephen Hawking, the
famous physicist, noticed his first symptoms - clumsiness in his
hands - 42 years ago.
|There is no test for MND.
The diagnosis is usually made on the basis of symptoms and what
a specialist finds when examining the nervous system. These will
rule out other conditions, such as multiple sclerosis (MS). MS
affects the nerves dealing with sensation, and sufferers often
complain of other symptoms, such as numbness and pins and needles,
as well as muscle-related problems.
Tests to help diagnose MND include:
- an electromyelogram, where the muscles are electrically
stimulated and their strength measured
- a muscle biopsy, which involves removing a small
piece of muscle under local anaesthetic, and examining it
under a microscope
|There's no cure for MND.
Riluzole (Rilutek) is the only drug currently licensed to treat
ALS, and it only slows the progress of the disease by a couple
However, several pharmaceutical companies are researching treatments
for MND at the moment.
Avenues being pursued include:
- antioxidants to 'mop up' waste molecules before
they damage nerve cells
- creatine, a chemical involved in the distribution
of energy within cells
- oxandrolone, an anabolic steroid which helps to
maintain body weight and muscle mass
|Giving people with MND enough
information to help them understand their illness and how to deal
with their disability is important. Physiotherapists, occupational
therapists and speech therapists can all offer advice and support.
Relaxation and breathing exercises regularly will help to keep
stress levels under control.
The support of family and friends is invaluable. Just being there
for the patient and helping them with their inevitable bouts of
anxiety and stress may be just as important as any help that qualified
professionals can give.
|Motor Neurone Disease (MND)
National Institute of Neurological Disorders and Stroke
Scottish Motor Neurone Disease Association
The Patients Association
|Healthwise (Health Information Resource
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Fax : (852) 2849 2900
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Homepage : http://www.healthwise.org.hk/
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