Respiratory support: the big debate on Symposium day 2

Different ways to support breathing were the main focus of the second clinical session on day two of the Symposium. Researchers from two MND Association funded studies presented their work looking at diaphragm pacing and also the withdrawal of ventilation support.

Lungs - Symp session pictureDiaphragm pacing

The NeuRx diaphragm pacing system (DPS) is a device developed to aid breathing by stimulating the large muscle that helps you to breathe – the diaphragm.

In 2011, the Food and Drug Agency (FDA) in the USA approved NeurRx DPS as a treatment for respiratory failure in motor neurone disease (MND). The treatment was not required to go through the series of clinical trials that is needed for a new drug. The FDA approved it on the basis of one small study because at the time the probable benefit to health outweighed the risk of using it.

Due to this lack of clinical evidence, this prompted further research in the USA and Europe to test its effectiveness on symptom management and survival.Read More »

Heading in the right direction: Sheffield Support Snood update


Update (February 2017): All sites are now closed and not recruiting for participants.

If you would like to be added to a waiting list for the Sheffield Support Snood, please contact Lise Sproson.


We are funding, together with the NIHR i4i (National Institute for Heath Research invention for innovation) programme, a research team in Sheffield who have developed a new type of neck support for people living with neck muscle weakness as a result of a neurological condition.

Designers, clinicians and engineers from University of Sheffield, Sheffield Hallam University, Devices for Dignity, Sheffield Teaching Hospital, Barnsley Hospital, and the Sheffield MND Research Advisory Group have worked together with people living with MND to develop the Sheffield Support Snood through an iterative design process.

The results from a small pilot study of the snood were presented at the International Symposium on ALS/MND in Brussels last year, where we reported on it.

5 b (3)

Next step – the 100 collars project

In September we attended the Sheffield Support Snood training day, run by Devices for Dignity, Sheffield Teaching Hospitals NHS Foundation Trust, and Dr. Christopher McDermott, a Clinician Scientist at the Sheffield Institute for Translational Neuroscience (SITraN) who is the Chief Investigator for the project .Read More »

Disappointing news for diaphragm pacing in MND

Results from the UK clinical trial of diaphragm pacing in MND/ALS (known as DiPALS) were published online today in the journal Lancet Neurology.

DiPALS was the first randomised clinical trial of diaphragm pacing in MND and aimed to find out whether or not diaphragm pacing was beneficial when added to the current standard treatment of non-invasive ventilation (NIV), compared to NIV treatment alone.

The trial results unfortunately show that diaphragm pacing was not beneficial when used in addition to NIV, and was in fact harmful, with people using diaphragm pacing living on average 11 months shorter than those on NIV alone.

Read More »

Breaking the Human Genome Code

Dr Johnathan Cooper-KnockProfessor Winston Hide gave his inaugural lecture on 17 March, during Brain Awareness week, entitled ‘breaking the human genome code – opening Pandora’s box’, which you can watch in full at the end of this blog post.

Professor Hide recently joined the University of Sheffield, and MND Association/ Medical Research Council (MRC) Lady Edith Wolfson Clinical Research Fellow, Dr Johnathan Cooper-Knock has written a blog below about Professor Hide’s research and how they are working together towards a world free from MND: Read More »

Gastrostomy in MND: Progress in ProGas

9 bAs well as biomedical research, we fund healthcare research to lead to better symptom management and support for people living with MND. On the final day of the symposium, Dr Stavroulakis from the Sheffield Institute for Translational Neuroscience (SITraN), presented results on his Association-funded research. 

In some people living with MND, the muscles involved in chewing and swallowing can become slow, weak and/or uncoordinated. This can cause difficulty when eating and drinking. Read More »

The importance of tissue in MND research

Courtesy of Dr Robin Highley, SITraN
Courtesy of Dr Robin Highley, SITraN

Dr Robin Highley from the Sheffield Institute of Translational Neuroscience (SITraN), which is based at the University of Sheffield, has written a special ‘guest blog’ about his research and the value of tissue donation.

I am a pathologist who recently completed a MND Association/Medical Research Council Lady Edith Wolfson fellowship.  During my research fellowship, a student and I spent many months using lasers to dissect out motor neurones from spinal cord, kindly donated by people who had died from motor neurone disease (MND). Read More »

The MND Researchers Bake off Champion!

As well as helping out with our ‘blog a day’ during MND Awareness Month, we also asked our researchers to get involved in ‘baking’ to become our first ‘MND Researchers Bake off Champion’. We received some great science-themed cakes, from zebrafish biscuits to a Nuclear Magnetic Resonance(NMR) machine cake!

Our Director of Research, Dr Brain Dickie said: “It was really tough to judge, they were all great entries! (might need to taste next year though…!). Of the seven entrants there was one that I think wins by a short head, scoring on appearance, originality and relevance to MND research, with an extra mark for sheer wackiness – the ribosome translating a C9orf72 repeat expansion cake!”

The winning cake was by Jenn Dodd, a PhD student at the Sheffield Institute for Translational Neuroscience (SITraN)! Here Jenn describes her cake and how it feels to be the MND Researchers Bake off Champion!

Jenn Dodd's winning ribosome translating C9orf72 cake!
Jenn Dodd’s winning ribosome translating C9orf72 cake!

The winner’s speech:

I decided to bake the cake, as at SITraN we have a weekly cake club and it was my turn to bake in June. I thought entering the competition would be a good way to get involved in MND awareness month and thought it would make cake club a bit different!

Small structural units called cells make up the human body. They convert food and oxygen into energy to produce chemically reactive machines and building blocks called proteins. There are thousands of different proteins made and so special templates called RNA are sent to a protein-making factory in cells called the ribosome. The ribosome makes proteins from the RNA templates in a process called translation (Read more about how cells make proteins here).

The cake shows a ribosome (yellow) translating RNA (the stripey sweets) to make a protein (the flying saucer chains). The protein that is being made is C9ORF72, a protein with an unknown function that is involved in some cases of MND.

I’d like to say thank you and I am really please to have won the bake off with my cake experiment!

Sheffield work towards a treatment for SOD1 form of MND

A number of articles were published in various news sources on 11 July 2014, highlighting how scientists in Sheffield are working towards testing a promising treatment for a rare inherited form of MND caused by the SOD1 gene. Here we write about the research and what it means for people living with MND.

The Sheffield Institute for Translational Neuroscience (SITraN) specialises in research into MND and other neurodegenerative diseases. Recently the institute received an anonymous donation of £2.2 million to help translate their research from the lab to the clinic. This is a huge amount of money into MND research and this donation will enable the researchers to further our understanding of the disease.

Laboratory PhotoThe research

We know that approximately 10% of cases of MND are inherited. This means that they are characterised by a strong family history and the disease is caused directly by a mistake in a specific gene. Of these 10% of cases, 2% are caused by the SOD1 gene (meaning that for every 100 cases of MND, 10 cases are inherited and of these, only 2 are directly caused by the faulty SOD1 gene).

Prof Mimoun Azzouz’s research at SITraN was reported in a number of news outlets, highlighting how his research is paving the way to a treatment for a rare form of MND. His research is at a relatively early stage, where he has only just begun investigating the use of a technique known as ‘gene therapy’ in mice affected by the SOD1 inherited form of MND. If the research goes to plan, he will be able to submit a proposal for regulatory approval by August 2015.Read More »

The UK Whole Genome Sequencing project

Dr Samantha Price is the Research Information Co-ordinator at the MND Association. As well as organising the ‘blog a day’ during MND Awareness Month she also communicates the latest news about MND research. Here she blogs about the MND Association’s announcement of the UK Whole Genome Sequencing project.

It’s been a brilliant Awareness Month with blogs about zebrafish research and streaking meerkats. To end on a positive research note, we’re delighted to announce that we are funding a UK Whole Genome Sequencing project to help us understand more about the causes of MND. Utilising samples from our own UK MND DNA bank; researchers in the UK will aim to sequence 1,500 genomes to help identify more of the genetic factors involved in the disease.  Read More »

Cycling to ENCALS – “on yer bike”

Medical Research Council (MRC)/ MND Association Lady Edith Wolfson Clinical Research Fellow, Dr Johnathan Cooper-Knock, is based at the Sheffield Institute for Translational Neuroscience (SITraN), at the University of Sheffield. The Tour de France ends up in Sheffield a week today, so to get you into the cycling spirit, John blogs about the Sheffield to Leuven ENCALS cycling challenge!

The great idea

A few weeks ago Richard Mead and I had a chat in the lab. We wondered whether it would be possible to cycle to Leuven for the forthcoming European Network for a Cure of ALS (ENCALS) conference (22 – 24 May 2014). Well of course it would be possible, but given finite resources, time and training could we do it? When we discovered the overnight ferry from Hull to Zeerbrugge (which we were reliably informed only resulted in sea-sickness 60% of the time) we realised the dream could become a reality. Read More »