This blog is part of the ‘Highlights from Glasgow’ collection of articles, where you can read about the content of some of the talks and posters presented at the 29th International Symposium on ALS/MND.
Exploration into cognition and frontotemporal dementia with MND/ALS continues to attract attention. At the Symposium in Glasgow 2018, we heard of several studies adding to the growing knowledge bank in this field.
A history of other mental health conditions or psychiatric disorders within the family, or for the individual, indicates a correlation with MND/FTD. There seems to be a link between this increased history and a risk of apathy. Early screening is recommended where these histories exist. This can help prepare families and enable early discussions with the person diagnosed with MND, so they can make decisions that may be important to them in the future (C41) C McHutchison.Read More »
At the end of the opening session of the International Symposium on ALS/MND, two members of the MND research community were recognised for their contribution to the field.
Professor Orla Hardiman, from Trinity College, Dublin is this year’s recipient of the Forbes Norris Award. Presented by Dee Forbes Norris, this award recognises care and compassion in the study and management of MND/ALS. It is awarded by the International Alliance of the ALS/MND in consultation with the World Federation of Neurology. Prof Hardiman accepted the award saying “I’m not normally lost for words, but to use a local expression, I’m gobsmacked”. She paid tribute to her colleagues throughout her career and in particular to what she described as as her formative years in Boston, USA, working with Professor Bob Brown Jnr.
It was a privilege to witness the first presentation of an Institute Paulo Gontijo (IPG) Young Investigator award at the Symposium. Following a moving speech from Paulo’s daughter Marcela, Professor Mamede de Carvalho, chair of the awarding panel announced that the unanimous decision was to give the award to Dr Aaron Gitler of University of Pennsylvania.
“I’m honoured and humbled to accept this award” commented Dr Gitler. “The ALS field is experiencing a revolution, with paradigm changing discoveries even in the last few months – it is good to be a part of it”.
He gave a brief overview of the research that led to this award. The work has been conducted in the most basic of organisms – yeast. Without a brain and spinal cord, looking at yeast may seem an unusual way to study MND Dr Gitler acknowledged. However, as his talk demonstrated, yeast are an excellent model for understanding a common cellular sign of many neurodegenerative diseases – accumulation of proteins. A modifier of protein that accumulates in the yeast model led the way to identifying a link with a protein called Ataxin2 in MND.
“The annals of ALS clinical trials is strewn with failed studies. Only two out of more than 70 clinical trials have been positive, and even these showed only very modest benefit. Is this dismal record strictly due to the extraordinary complexity of neurodegenerative disease in general, and ALS in particular? Or is it due to methodological flaws that could be repaired?”
Robert G Miller, Professor of Neurology, Stanford University
Although there is not much we can do about disease complexity, improving the way treatments are trialed is something that can be achieved. Imagine a world without clinical trials, where independent companies or individuals would be allowed to sell their self-made ‘drugs’ without any evidence that they were ever used on anyone with the disease, let alone that they would improve one’s condition. No one would know what the drug is (which could simply be a water solution), how it works and whether as soon as the drug is taken, we would be poisoned.
Thankfully, this is not the case and clinical trials, although not perfect, are considered the gold standard for approving any treatment. However, there are still some improvements that can be done to make trials easier to access and provide more accurate estimates of drugs’ effectiveness much faster.
In November 2018 the Home Office released a draft Guideline scope for Cannabis-based products for medicinal use in which they announced that specialist doctors (like consultant neurologists) on the Special Register of the General Medical Council will be able to prescribe cannabis-based medicinal products to some patients. Before this, the only cannabis-based medicines licensed for use in the UK were nabiximols (Sativex), used as a treatment for spasticity (where muscles are continuously contracted, causing stiffness or tightness of the muscles, interfering with normal movement and speech), in multiple sclerosis (MS).Read More »
Motor Neurone Disease (MND), as the name suggests, is known as a disease of motor neurons, a specific type of neurons that co-ordinate our voluntary movement, leading to loss of the ability to move, speak and breathe. And perhaps because the main focus often falls on the rapidly-progressing physical symptoms and their management, the way MND affects the mind has often be overlooked.
Most literature on MND states that certain behavioural and cognitive (thinking) problems affect up to 50% people with MND, out of which 15% have a co-occurring diagnosis of frontotemporal dementia (FTD). Adding to this, a recent paper by Dr Christopher Crockford and colleagues, published in the journal Neurology, found that up to 80% of people living with MND will have some form of cognitive or behavioural impairment by the final stage of their disease (or in other words, only 20% will have an intact cognitive and behavioural processing).Read More »
I usually travel to London two to three times a month for meetings and lab visits. If I’ve got any length of spare time, I head for what I call my ‘London office’ – aka the British Library. It’s close to Euston station, it’s free (!) it has a nice café for informal meetings and it has copies of all the latest textbooks and major research journals.
The way in which a cell turns its genetic instructions into the protein ‘building blocks’ it needs to function and survive is sometimes compared to a library.Read More »
Conferences and symposia are a crucial part of the research world – not only for the amount of knowledge that is communicated to large audiences but also for the exchange of ideas on a more inter-personal level. Novel ideas are created there as well establishment of collaborations that might lead to new research projects and clinical trials – all in all, putting a bunch of researchers in a venue with a projector, coffee and biscuits can only lead to good things!
One of the recent events that I had the pleasure to attend was a small-scale conference – the Mini-Symposium on generic disease mechanisms in MND and other neurodegenerative disorders. Held at the Brighton and Sussex Medical School in late June, this event was a precursor to the inauguration of a new MND Care and Research Centre for Sussex, directed by Prof Nigel Leigh.Read More »
The last of our FTD awareness week blog posts is focussing on a healthcare project looking into FTD (frontotemporal dementia) and FTD-MND (FTD when combined with MND). The project began last year and is being part-funded by us.
Professor Julie Snowden and PhD student Jennie Adams at the Cerebral Function Unit in Salford (University of Manchester) are looking into the behavioural and cognitive aspects of FTD and FTD-MND.
They are aiming to work out if there are any differences in thinking or behaviour between people who have MND-FTD and those who have FTD on its own.
For example this could be looking to see if people with FTD-MND tend to show more difficulties with language, but not have many changes relating to behaviour. Or if people with ‘pure’ FTD show more difficulties with appropriate behaviour in public, compared to organisation and planning skills.Read More »
Did you know the MND Association also supports people who have Kennedy’s disease?
In May a new clinic specialising in Kennedy’s disease opened in London at the National Hospital for Neurology and Neurosurgery.
To mark this big step in helping support and treat people with Kennedy’s disease, Katy Styles who campaigns on behalf of the Association, and whose husband Mark has Kennedy’s disease, thought it would be a great opportunity to raise awareness of this rare condition.
Katy and Mark Styles
“There is very low awareness of this disease amongst neurologists, healthcare professionals, the general public and within the Association itself. We do all we can to explain to everybody what Kennedy’s disease is and what it’s like to live with.
“Due to the rarity of Kennedy’s disease you can feel very much alone. It is so great to be part of the MND family and the Association is key to this by making us feel part of a wider community.”
What is Kennedy’s disease?
Kennedy’s disease is a condition similar to motor neurone disease (MND) which affects motor neurones. It is sometimes called spinal and bulbar muscular atrophy (SBMA).Read More »
A few days before Christmas, I hope that you’ll forgive the obvious pun. Rather than the small green vegetable that you either love or hate, here I’m talking sprouts of new shoots of talent shown by the winners of the poster prizes. They were chosen from over 300 poster presentations at the International Symposium on ALS/MND held in Brussels at the beginning of December.
It was the second year that poster prizes were a feature of the conference. The purpose of the prize was three-fold: to increase the profile of the poster sessions of the meeting; to recognise the quality of the work presented there and to reward presenters of outstanding work. Read More »