Toxicity in the brain of Motor Neurone Disease patients
3 December 2003
A project funded by Action Medical Research and supported by Britannic Management Services Ltd has found that patients with Motor Neurone Disease (MND) produce certain proteins that are toxic and which cause the motor neurones (which control movement) to die. This work is published in the January issue of the journal Acta Neuropathologica.
The proteins called immunoglobulins (IgG) are found in the blood serum of patients with MND. Too much calcium in a nerve cell can be fatal and laboratory tests showed that these proteins cause calcium levels in motor neurones to rise. The cells then degenerated and died.
When IgG from healthy individuals were used in the same experiment there was no corresponding rise in calcium and the motor neurones did not degenerate.
Simon Moore, Chief Executive of Action Medical Research said: “This is a very exciting finding. This work could potentially lead to the development of some treatments which will help these people. Early intervention is always desirable in most diseases and it would help enormously if progress could be made in this area.”
Dr Anthony Pullen at the Institute of Neurology at University College London who led the study said: “Our work has allowed us to see both the initial and end stages of how motor neurones die in the laboratory. We are just beginning to understand how immunoglobulins and calcium react in nerve cells, but we need to do a lot more work to understand the mechanism and the real significance for MND. There is a lot of exciting work going on in MND research which will hopefully lead to the development of drugs that will really help people with Motor Neurone Disease.”
MND is caused by the death of motor neurones in the brain and spinal cord that control movement. It affects around 5,000 people in the UK at any one time and there are 1000 new cases of disease each year. By the time the patient first notices that they have any muscle weakness or difficulty swallowing it is estimated that up to 50 per cent of their neurones have already died. In the majority of cases the intellect is not affected.
Symptoms of MND are muscle weakness in one or more limbs and also the muscles that supply the face and throat, causing problems with speech and difficulty chewing and swallowing. Death occurs in around three to ten years due to paralysis of the respiratory muscles.
About ten per cent of MND is genetic but 90 per cent is of uncertain cause, and is probably multifactorial. There is only symptomatic treatment to alleviate the excessive pain from muscle cramps, salivation, difficulties with swallowing and speech, and respiratory & psychological distress.
For more information or to interview Simon Moore or Dr Pullen please telephone Louise Brown on 01403-327403 or Rob Orme on 01403-327404.
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