Motor Neurone Disease
What is motor neurone disease?
Motor neurone disease (MND) is the name
given to a group of related diseases in which there is progressive degeneration
of the motor neurones in the brain and spinal cord. Motor neurones are the nerve
cells that control muscles, and their degeneration therefore leads to weakness
and wasting of the muscles.
This wasting generally occurs in the arms
and legs initially, with some groups of muscles being affected more than
others. Some patients with a particular type of MND may develop weakness and
wasting in the muscles supplying the face and
throat. This can lead to problems with
speech, chewing and swallowing.
Amyotrophic lateral sclerosis (ALS) is the
most common form of MND, this is commonly referred to as “Lou Gehrig’s disease”
in the US.
What are the symptoms of MND?
MND can present itself in a number of ways,
depending on the particular group of muscles which are initially affected.
Initial symptoms can include wasting and weakness of muscles of the hands or
weakness and stiffness in the legs with a dragging of one leg.
Well-known scientist, Stephen Hawking who
has motor neurone disease.
There may also be difficulty with speech
and swallowing early on. The disease may remain stationary for some time or may
progress to other limbs, to the tongue and to the breathing muscles. The rate
at which MND progresses varies from one person to another. Death in MND is
usually caused by the combined involvement of swallowing and breathing muscles,
causing severe pneumonia.
Does MND affect intellect?
No, MND does not affect intellect. It also
does not affect touch, taste, sight, smell hearing, nor directly bladder, bowel
or sexual function.
Who gets MND?
Both males and females can develop MND,
however men are twice as likely to get it compared to women. The disease most
commonly occurs in people aged 50 or over, although occasionally, people in their
20s and 30s develop it. Around one in 50,000 people will develop MND in any one
year, with 250 people with MND in Ireland at any one time.
What causes MND?
The cause of MND is unknown and research is
continuing. Theories include:
- Toxins from the environment
- Metabolic disturbances
- Genetic defects.
There is some evidence that MND can run in
families, however the majority of people with MND have no family history of the
How is MND diagnosed?
There is no specific laboratory test for
MND, however usually a neurologist can diagnose the disease on the basis of the
patient's history and signs.
Sometimes extra tests are carried out,
including a brain and spine MRI (magnetic resonance imaging), in order to
exclude other diseases. Usually electrical tests on muscles and nerves are
performed and a lumbar puncture and muscle biopsy may also be carried out.
Are there any treatments for MND?
There is no known cure at present for motor
neurone disease. Survival time varies from around 3-5 years; however, some
people may survive for much longer. A modest improvement in life expectancy may
be achieved using the drug riluzole – the first and to date, only drug licensed
for the treatment of ALS, the most common type of MND.
The drug is not a cure and it does not
prevent, halt or reverse the disease. However it has been shown to slow
progression of the condition and increase the life expectancy of some patients
Apart from this, many of the symptoms and
subsequent problems can be effectively alleviated by your consultant or GP. In
addition, other professionals, such as physiotherapists, occupational
therapists and speech therapists can be a great source of help.
What can a person with MND do if they
develop swallowing problems?
If a person with MND is showing signs of
having difficulties swallowing, they should be referred to a speech and
language therapist for a full assessment and ongoing management. Signs of
swallowing problems, which is also known as dysphagia, include:
- A difficulty with chewing food
- Problems initiating a swallow
- Requiring a long period of time to finish
- Weight loss
- Excessive drooling or leaking of saliva,
food or drink from the mouth
- Repeated chest infections.
Following full assessment by a speech
therapist, management of swallowing difficulties may include:
- Dietary changes, as some foods may be
easier to eat than others
- Posture changes, to ensure that you are as
upright as possible when eating or drinking
- Special head positions when eating.
It is extremely rare for people with MND to
choke when eating and drinking.
Are there any organisations that can help?
The Irish Motor Neurone Disease Association
was founded in 1985 by a small group of friends, relatives and carers of people
with MND. The association functions mainly as a support organisation for people
who have MND, as well as their carers and families. This work entails home
visiting, financial assistance for home nursing and the loan of specialised
equipment to patients.
The association also supports research into
the causes and treatment of MND. You can contact the organisation on Freephone:
1800 403 403 or Email: firstname.lastname@example.org.
The organisation also has a website at www.imnda.ie