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What is Motor Neuron Disease?

History
Motor Neurone Disease (MND) was first described in the 1850’s by French Neurologists. In 1874 Prof Jean-Martin Charcot called it Amyotrophic Lateral Sclerosis (ALS), its usual name in the USA. There it is also known as 'Lou Gehrig's Disease' named after a pre-war star of the New York Yankees baseball team. Motor Neurone Disease is the term generally used in Europe.

Motor Neurones
These are the many millions of nerve fibres which go from the cortex (grey matter) of the brain to the muscles in the trunk and limbs. There are two groups (1) Upper motor neurones which start with cell bodies in the cortex and pass down through the brain to the spinal cord and connect with the (2) Lower motor neurones which have their cell bodies in the spinal cord and brain stem and then pass out into the periphery forming nerve fibres going to muscle fibres. The strength and smoothness of movement and the bulk of muscles depend on the integrity of these nerve fibres.

Motor Neurone Disease
is due to the degeneration of these motor neurones. Degeneration of the upper motor neurones causes both weakness, stiffness and resistance to movement in the muscles with changes in the reflexes which the doctor can elicit. Degeneration of the lower motor neurones causes wasting of muscles, weakness, fasciculations (flickering of small groups of muscle fibres occurring spontaneously) and muscle cramps.

Symptoms
MND presents itself in various ways depending on the particular groups of muscle fibres which degenerate initially, Wasting and weakness of muscles of the hands, sometimes one side first, stiffness in the legs with dragging of one leg or the development of marked weakness in the legs may be the initial symptom. Sometimes the muscles of the tongue and swallowing mechanism are affected early with slurring of speech, difficulty in swallowing and coughing. The disease may remain relatively stationary for some time or may progress to other limbs to the tongue and to the breathing muscles. Death in MND is usually caused by the combined involvement of the swallowing and breathing muscles causing a severe pneumonia. Motor Neurone Disease does not affect the intellect, does not cause bladder or bowel symptoms and does not cause sensory, visual or hearing disorders.

Life Expectancy
is variable from I year up to 5 years (or occasionally longer) after onset depending on the activity of the disease and the particular muscle groups that are affected.

Who gets MND?
The majority of people with MND are aged 50 years and older but occasionally people in their 20’s and 30’s develop MND. It is not known why an individual gets MND. There is a male predominance of 2:1. There are rare familial types of the disease.

What causes MND?
This is not known at all. Suggestions as to the cause include:
(a) toxin from the environment.
(b) trauma.
(c) a virus which lies latent within the system for many years.
(d) a premature degeneration of these nerve cells caused by some in-born defect in their constitution.

How is it diagnosed?
Usually it is not difficult for the Neurologist to diagnose MND on the basis of the history and signs. Early signs may cause some initial difficulty. There is no specific laboratory test. Sometimes special tests (scans and myelograms) are necessary to exclude other diseases, Usually electrical tests on muscles and nerves are performed and a lumbar puncture may be done.

Treatment of MND
While research is continuing worldwide, there is at present no treatment which will alter the course of the disease or affect its progression in any way. Treatments given are those which help the person with MND to cope with symptoms and disabilities and may involve drugs to reduce stiffness or resistance to movement. RILUTEK is the only licensed drug, which may help to slow the progression. The involvement of physiotherapists, occupational therapists, speech therapists and the provision of aids at home are all necessary to support the person with MND and the family.

Well-known MND patients
Although in the USA almost everyone knows that 'Lou Gehrig's Disease' is actually ALS and what it is, in England and here in South Africa, if you mention MND as being what David Niven had, most people are surprised and pretty vague about the disease and what it entails. Our newsletter 'Thumbs Up' is named for the cheerful gesture used by David Niven during the later stages of his illness.

Although he is probably the most widely known MND sufferer this side of the Atlantic there have been surprisingly many well known victims of the disease considering its rarity, only 2 cases per 100,000 people worldwide.

Here are a few of them:
Chairman Mao Tse-Tung: World War II bomber ace then charity homes founder Group Capt (later Lord) Leonard Cheshire VC: actor (and father of Sinead) Cyril Cusack: from the art world legendary Australian painter 'Pro' Hart and South African art gallery owner Everard Read: footballers Leeds United and England player/manager Don Revie and Celtic and Scotland star Jimmy Johnstone: Lou Gehrig was followed at the 'Yankees' by All American pitcher Jim 'Catfish' Hunter: Director General of the BBC Sir Ian Trethowan: blues musicians Charlie Mingus and 'Leadbelly': boxer Ezzard Charles: right-to-die activist Diane Pretty.

And last but not least American University professor Morrie Schwartz, immortalised by his pupil Mitch Albom in the delightful 'Tuesdays with Morrie' which became Jack Lemmon's last movie. Read the book and rent the DVD.

We are indebted to the Irish MNDA, Dublin for permission to use some of their website content
http://www.mnda.org.za/aboutmnd.htm

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