(Tuesday, 23rd Sep, 2014)
Motor neurone disease
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.
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.
No, MND does not affect intellect. It also does not affect touch, taste, sight, smell hearing, nor directly bladder, bowel or sexual function.
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.
The cause of MND is unknown and research is continuing. Theories include:
There is some evidence that MND can run in families, however the majority of people with MND have no family history of the disease.
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.
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 with MND.
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.
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:
Following full assessment by a speech therapist, management of swallowing difficulties may include:
It is extremely rare for people with MND to choke when eating and drinking.
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
Reviewed: December 8, 2006
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Last Reviewed: 8th December 2006