Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:

 

Motor neurone disease: devastating, mysterious and few treatments available

15.06.2007
Motor neurone disease (MND), also known as amyotrophic lateral sclerosis, is a degenerative disease of unknown cause which causes patients to lose basic motor functions and has a devastating effect on them and their families. But work is ongoing on new treatments to prolong life expectancy and raise quality of life for patients, say the authors of a Seminar published in this week’s edition of The Lancet.

And soldiers, Italian professional footballers and the Chamorro population of the Pacific Island of Guam have been reported to be more at risk of MND than the general population.

Professor Douglas Mitchell, Motor Neurone Disease Care and Research Centre, Lancashire Teaching Hospitals NHS Foundation Trust, Preston, UK and Professor Gian Borasio, Motor Neurone Research Group, Department of Neurology, Munich University, Germany, comprehensively reviewed literature published on MND since 2000 to prepare the Seminar.

MND is one of the major neurodegenerative diseases alongside Alzeheimer’s disease and Parkinson’s disease. It is a progressive disorder that involves degeneration of motor functions at all levels as the motor neurones of the nervous system are destroyed.

The authors: “The course of the disorder is inexorably progressive, with 50% of patients dying within three years of onset.”

Symptoms generally begin in one or more of three regions – the throat/jaw area (bulbar), the arm area (cervical) or the leg area (lumbar). Bulbar-onset patients can struggle to swallow, have slurred speech, and/or facial weakness. Cervical-onset patients have problems with the use of their arms and hands and lumbar-onset patients have difficulty with walking.

Progression of the disease is marked by loss of speech, chronic nocturnal hypoventilation, daytime fatigue, general loss of strength and motor functions. Up to 73% of MND patients complain of pain. Psychosocial care is also vital for patients, with 100% of patients in one study mentioning family as important to their quality of life.

Men are more likely to get MND than women (approximate ratio 1.6:1) and MND has been more common in certain populations, such as the Chamorro population on the Pacific island of Guam. It has been suggested that this is because the diet of these people includes the fruit bat, which feeds on cycad seeds. These seeds contain methylaminoalanine, which is thought to have a role in MND. An increased risk of MND has also been reported in military personnel and Italian professional football players.

Heavy-metal toxic effects, and environmental and occupational exposures, have also been proposed as causes of MND. But the authors say: “Despite extensive research, the disorder remains poorly understood in terms of a unifying causal hypothesis, and, indeed, might turn out to be a common end-stage phenotype of diverse causes.”

Many treatments have been tried for MND, but few have had success. Riluzole is the only drug licensed to treat MND, and has been shown to extend the lives of MND patients by an average of three months.

The authors are sceptical about the use of stem cell treatment, saying much work needs to be done before stem-cell treatment can ever be regarded as even an experimental therapeutic treatment. An earlier trial of injecting stem-cells into the spinal cord proved ineffective in relieving MND symptoms.

Use of viral vectors for drug delivery could be a pioneering MND treatment, since it extended the life expectancy of mice it was tested in by 30%. The need to deliver disease-modifying treatments direct to the site of disease process – i.e. the motor neurone – is a recurring theme of treatment discussions.

The authors conclude: “Because of the shortage of satisfactory disease-modifying treatments, early diagnosis of amyotrophic lateral sclerosis has traditionally not been imperative. It will, however, be increasingly important for any attempts to develop more effective treatments.”

Tony Kirby | alfa
Further information:
http://www.thelancet.com/webfiles/images/clusters/thelancet/press_office/Seminar.pdf

More articles from Health and Medicine:

nachricht Collagen nanofibrils in mammalian tissues get stronger with exercise
14.12.2018 | University of Illinois College of Engineering

nachricht New discoveries predict ability to forecast dementia from single molecule
12.12.2018 | UT Southwestern Medical Center

All articles from Health and Medicine >>>

The most recent press releases about innovation >>>

Die letzten 5 Focus-News des innovations-reports im Überblick:

Im Focus: Data use draining your battery? Tiny device to speed up memory while also saving power

The more objects we make "smart," from watches to entire buildings, the greater the need for these devices to store and retrieve massive amounts of data quickly without consuming too much power.

Millions of new memory cells could be part of a computer chip and provide that speed and energy savings, thanks to the discovery of a previously unobserved...

Im Focus: An energy-efficient way to stay warm: Sew high-tech heating patches to your clothes

Personal patches could reduce energy waste in buildings, Rutgers-led study says

What if, instead of turning up the thermostat, you could warm up with high-tech, flexible patches sewn into your clothes - while significantly reducing your...

Im Focus: Lethal combination: Drug cocktail turns off the juice to cancer cells

A widely used diabetes medication combined with an antihypertensive drug specifically inhibits tumor growth – this was discovered by researchers from the University of Basel’s Biozentrum two years ago. In a follow-up study, recently published in “Cell Reports”, the scientists report that this drug cocktail induces cancer cell death by switching off their energy supply.

The widely used anti-diabetes drug metformin not only reduces blood sugar but also has an anti-cancer effect. However, the metformin dose commonly used in the...

Im Focus: New Foldable Drone Flies through Narrow Holes in Rescue Missions

A research team from the University of Zurich has developed a new drone that can retract its propeller arms in flight and make itself small to fit through narrow gaps and holes. This is particularly useful when searching for victims of natural disasters.

Inspecting a damaged building after an earthquake or during a fire is exactly the kind of job that human rescuers would like drones to do for them. A flying...

Im Focus: Topological material switched off and on for the first time

Key advance for future topological transistors

Over the last decade, there has been much excitement about the discovery, recognised by the Nobel Prize in Physics only two years ago, that there are two types...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

VideoLinks
Industry & Economy
Event News

ICTM Conference 2019: Digitization emerges as an engineering trend for turbomachinery construction

12.12.2018 | Event News

New Plastics Economy Investor Forum - Meeting Point for Innovations

10.12.2018 | Event News

EGU 2019 meeting: Media registration now open

06.12.2018 | Event News

 
Latest News

Data use draining your battery? Tiny device to speed up memory while also saving power

14.12.2018 | Power and Electrical Engineering

Tangled magnetic fields power cosmic particle accelerators

14.12.2018 | Physics and Astronomy

In search of missing worlds, Hubble finds a fast evaporating exoplanet

14.12.2018 | Physics and Astronomy

VideoLinks
Science & Research
Overview of more VideoLinks >>>