Motor Neurone Disease

        Steven Hawking, (pictured below), suffers from this disorder.  

You do not normally get this before you reach the age of forty and it is more common in men than women. But 5 to 10 per cent of incidents of the disease are inherited, and in some of these there is known to be a gene defect that alters the function of an enzyme known as super oxide dismutase. Affected muscles twitch, become progressively weaker. The muscles that control eye movement, and sphincter muscles, which surround the openings of organs such as the bowel, are unaffected. Deterioration of the long spinal-cord tracts and nerve cells concerned with movement results in wasting and weakness of the small muscles in the persons hands, spreading to the forearms, and there is increased muscle tension in the legs. When the disease affects the nerves emerging from the brain stem it causes progressive difficulty in swallowing and speaking and a tendency to inhale ingested food. There is hoarseness, reduction in the strength of the voice, and wasting of the tongue. Eventually, speech becomes impossible. Lucent Technologies are a company which provide a voice synthesis system. Advances in electronic control and communication and in electromechanical technology have enabled sufferers of the disease to achieve some measure of independence. Control of electrically operated wheelchairs is possible by applying light pressure, blowing, head-nodding, or even blinking.

By Peter Hales   10KE