Motor neurone disease (MND) is a fatal, rapidly progressing disease that affects the brain and spinal cord. It attacks the nerves that control movement so muscles no longer work.
It does not affect the senses but can leave people locked in a failing body, unable to move, talk and eventually breathe. Some people may experience changes in thinking and behaviour, with a proportion experiencing a rare form of dementia. Currently it is incurable.
Here, Ammar Al-Chalabi (pictured), director of King’s MND Care and Research Centre, shares his expertise on the latest research, challenges and breakthroughs for people with the condition.
The search for a treatment
The underlying aim among researchers is to understand the causes of MND. If we can work out why it happens, we might be able to find a treatment.
We’re also looking into why it progresses more rapidly in some people than others. Th is may lead to a treatment which is able to at least slow its progression down. A lot of our research is genetics-based and there are quite a few studies which give us optimism in the search for a cure.
These studies are helping us to better understand the mechanism of MND and therefore increasing our chances of being able to treat it at some point in the future.
Outlook for people with MND
MND kills a third of people within a year and more than half within two years of diagnosis. An estimated one in 300 will die of the disease. Over the last decade we’ve found survival rates have improved slightly, but this is measured in weeks and months rather than years, so it is barely noticeable.
Strong multidisciplinary teams are shown to improve outcomes. In practice, this means care is managed by neurologists in conjunction with palliative care specialists, physiotherapists, dieticians, wheelchair therapists, clinical nurse specialists, care coordinators, respiratory consultants and occupational and speech and language therapists.
In our team at King’s we also involve a volunteer who has had first-hand experience of MND through a family member or loved one. is is important because they understand what the patients are facing in a different way from healthcare professionals.
In the last 15 years we’ve started intervening more quickly with non-invasive ventilation. Previously, if someone had respiratory failure, we would give a treatment to stop them feeling distressed about being breathless but it was not straightforward to offer any practical solution to the breathing difficulty, and so they would die soon after.
Now we are able to use a portable machine that can support breathing and take away breathlessness by putting air into their lungs. is relieves their symptoms for an extended period and gives a better quality of life for longer.
There is some evidence that it may even prolong survival. We also now intervene more quickly with nutritional support. ere is a lot of research going on currently into whether this needs to be a specific type, such as a high-calorie diet, for example. Hopefully we will get a greater understanding of the impact of diet on the condition in the coming years.
MND and the brain
We are now recognising that in up to 30% of people there is some involvement of thinking. MND can affect word finding and impact on personality in quite subtle ways; o en by making people more stubborn. As a result, patients may interact with relatives differently and may also reject healthcare options.
There is a lot of exploration going on into gene therapy. Although only about 5% of people have a family history of MND, there is probably a genetic component to the condition in everyone. By affecting the genetics, we may be able to slow the disease down.
Other strategies include trying to improve strength regardless of affecting the underlying disease. Increasingly we are seeing new drug treatments designed to make muscle contractions stronger. Another approach being looked into is immune therapy. ere is some evidence that inflammation might influence the disease’s progression. If you can affect the immune system, we might be able to change the course of the disease.
MND and NHS cutbacks
MND is an extremely distressing condition and you need time to be able to deal with all the multiple problems that patients face. When people are given the diagnosis it is a life-changing and usually very distressing experience. ere are many time pressures on people in the NHS.
So we have competing demands and it can be challenging to spend enough time with our patients to support them through this difficult situation.
Although the risk of developing MND is about the same as that of developing multiple sclerosis in the UK, MND affects life expectancy, so it is not as common. It is therefore perceived as a rare disease and it can be di cult to get funding for vital research. In the UK we’re fortunate to have the support of the Motor Neurone Disease Association, which is extremely effective and supportive of patients. Crucially, it also funds a lot of our research into MND.
Generally patients are extremely motivated to take part in research. Sometimes families can be reluctant to agree to get involved if there may be a genetic basis to their family’s condition, as that can have significant implications for them.
Raising public awareness
Perceptions and awareness of MND change depending on where you are in the world. In the UK, public awareness was very low but improved a lot with the Ice Bucket Challenge.
The problem with this viral social media campaign however, was that it referred to ALS rather than MND. A lot of people in the UK didn’t realise it was the same condition and donated funds to charities in the US.
Now is the time to embrace better ways of working
By Merryn Dowson, assistant psychologist and part of the team behind rehab goal-setting platform Goal Manager
A stitch in time saves nine. Rome wasn’t built in a day. The best things take time.
We are all too aware that some of the most important parts of our lives have been crafted, carved and developed over months and years. Consider your education, for example: you may well have been to primary school, secondary school and then sixth form college. Perhaps you went on to do an undergraduate degree.
You may even have taken another leap and completed a Master’s degree or a Doctorate. This took years. You learned, revised, sat exams, sat resits, applied for places, got results, got rejected, got accepted, and made it here.
One thing is certain: compared to all of this expertise, someone who completed a two-hour online course on the same topic does not come close. We know that putting time and effort into something gives us better results than if we tried a quick approach.
We do not always lead by this example though. Despite the knowledge that great results are only achieved through hard work and perseverance, sometimes we decide just not to bother. Often, a room in our home might look cluttered, worn down and unloved and it could be made to look incredible.
The walls could be painted, clutter cleared, carpet cleaned, furniture patched up, curtains updated, but it is so much effort. We see the effort it would take and keep living with it. It does the job. It’s fine.
We heard this a lot when we began to develop our software. Goal Manager was designed from within a clinical neuropsychology service with young people with acquired brain injury, and we recognised how goal setting was becoming an intimidating concept within our service and our colleagues across the field.
To combat this, we developed an online goal-setting platform which streamlines the key processes of goal setting into one system and allows members of multi- disciplinary teams (MDTs) to collaborate on goal data remotely.
Crucially, it was designed to fill a hole. The more daunting goal setting became, the more it was shied away from, and the guidelines for goal setting that had emerged from the literature were falling to the wayside.
While we designed our platform to save time on completing all of the gold-standard processes of goal setting compared to doing them manually, we found that people had often not been completing them at all. It was all too complicated.
As a result, we recognise that adopting a software solution like Goal Manager can come with its own problems to solve. It requires relearning a lot of
what we know about a concept like goal setting, understanding properly how these key processes work and how they can be applied clinically to benefit clients.
It is only then that you can start to think of ways to make it more efficient. To help with this, our users are offered bespoke demonstrations, guided MDTs through meetings to help with the clinical application of the data, and training on assessments and goal attainment.
This takes time. Our users are often throwing out their previous guide and writing a new one. When surveyed, however, every single one who responded said that it was worth it.
This brings us back to where we started: the best things take time; Rome was not built in a day; a stitch in time saves nine. By taking time to develop an understanding of goal setting and being able to apply it to a software solution, users experience all of the benefits of best-practice goal setting outlined in the literature both for their clients and for their teams.
Clients are motivated, rehabilitation is meaningful, important areas to address are highlighted, MDTs are focussed, clinical practice is evidenced – the list continues. None of this would have been possible without the initial investment of time.
While simple enough to read, this is no doubt overwhelming to apply to your service or practice and, with this in mind, there are some key points to remember. The most significant is that there is no better time than now.
The world is slowly opening its eyes, sitting up in bed and having a good stretch after the darkness of the Covid-19 lockdown. It is not yet certain if we are going back to snooze or if we are leaping out of bed afresh.
What we do know, however, is that we are heading into a brand new day. Even for those of us who continued in practice throughout the pandemic, services have been slightly paused in one way or another, whether that be refraining from home visits or having fewer people in the office.
We are all very aware that we are heading into the “new normal” rather than our old ways. Use this time to bring new and innovative ways of working into your practice. You might completely change your filing system, consider how you approach your waiting lists, or change how you approach MDT meetings.
Whatever you have been wanting to do for you and your service for so long, now is that time.
Perhaps you decide that you are going to welcome change but not all at once. That works too! For users of Goal Manager, we often suggest that starting with one or two clients might feel more manageable than a whole caseload.
This can help get to grips with the new concepts and ways of working without feeling like everything is completely disrupted. This applies elsewhere too. If you are wary of integrating a system into your whole service, start with one corner of it, evaluate, take what you have learned and then look to apply it more widely.
Finally, remember that all time taken to improve and grow impacts more than just what you set out to do. When people lose weight, they rarely conclude
by saying they just lost weight: they often enthuse about how they feel more energised or move easier or feel more positive or experience less anxiety.
This applies to any time you invest in developing your clinical practice or your service.
While time spent learning how to use Goal Manager and establishing it within a caseload is designed to improve goal setting, that investment also leads to improved assessment processes, more effective meetings, improved digital literacy, increased patient involvement and so much more.
The potential is enormous. To motivate you to start the process, look at what you want to achieve and how that might trigger other improvements.
While the world is still trying to drag its head off the pillow to open up the lockdown curtains, look to invest in addressing those needs you have always been aware of but never felt like you could justify the time.
Walk around your “house” and look into each room: is this the best it can be or could I give it a lick of paint?
Is now the time to bring meaningful solutions into my practice? Maybe grab a tester pot and try a new shade on the walls. Sign up for a free trial. Plan to grow and improve. Start building Rome.
To invest in improving your goal setting, visit www.goalmanager.co.uk to register for a live demonstration, sign up for a free trial or request a bespoke tour through the platform and its features.
Researchers unlock key prognostic tool for brain injured patients
In 1974, leading neuroscientist Graham Teasdale co-created the Glasgow Coma Scale (GCS) while at the Institute of Neurological Sciences in Glasgow. This scale has since been used to assess coma and impaired consciousness in patients who have had a brain injury.
The scale is used to describe variations in a patient’s eye, motor, and verbal responses. Each feature is assigned numerical scores depending on the quality of the response, and total scores range from three, which is a deep coma, to 15, which is full consciousness.
The GCS is used in clinics all around the world by physicians, nurses, and emergency medical technicians; and is also applied more widely in other, more complex systems that are used in assessing acute brain damage.
However, all three features of the GCS can’t always be determined in patients. Most commonly, the verbal response can’t be tested, as it’s not possible to determine this response in patients with severe brain injury who are intubated.
When the verbal score cannot be measured, the GCS can still be used in routine assessment and communication about a patient’s condition.
“The GCS should be reported in its component parts, so there is still useful information in the motor and eye components, and the verbal score can simply be reported as not testable,” Paul Brenan, senior clinical lecturer in neurosurgery at the University of Edinburgh says.
“The missing verbal score is problematic, though ,when determining the GCS sum score (eyes + verbal + motor). The sum score is used in clinical prognostic tools, such as the GCS -pupils score, so until now, missing verbal data has prevented clinicians from using these tools.”
But now, Teasdale and Brennan, along with Gordon Murray at the University of Edinburgh, have created a tool to use to assess impaired consciousness when the verbal component of the GCS is missing.
The researchers first examined a database of GCS assessments, and found that the verbal component of GCS was missing in 12,000 patients with traumatic brain injury (TBI), which made up 11 per cent of GCS assessments. These verbal scores were most often missing in patients with low eye and motor scores.
Using GCS data recorded in a database of 54,000 patients, the researchers calculated the distribution of verbal scores for each combination of eye score and motor score. They then combined GCS eye and motor scores into EM scores, and determined the distribution of verbal scores for each EM score. Based on this, they identified a verbal score that clinicians could impute for every EM score.
“Without the verbal component of the GCS, the GCS sum score (eyes + Verbal + motor) cannot be determined, so we developed this imputation tool to enable clinicians to benefit from these prognostic tools for decision making in patients with the most severe brain injuries, where the verbal score is not testable,” Brennan says.
To test these imputed verbal scores, the researchers substituted imputed verbal scores for actual verbal scores within the framework of prognostic charts, which the authors had previously developed.
These charts take into account the total GCS score, pupil response, age of the patient, and findings of abnormalities. The charts provide predictions about patient outcomes, and are designed to help clinicians make decisions and communicate across teams.
The authors outline in their paper, ‘A practical method for dealing with missing Glasgow Coma Scale verbal component scores,’ published in the Journal of Neurosurgery, that they found that the information gleaned from imputing verbal scores according to each EM score was similar to the variations between precise eye and motor scores, and from full information on verbal, eye, and motor responses.
Imputing verbal scores doesn’t add new information, but allows clinicians to use prediction and prognostic models by filling in verbal data needed for those systems to work.
“We have developed several tools related to the GCS that enhance its ease of use and clinical application, including the GCS Aid, the GCS-pupils score and the GCS pupils Age CT prognostic charts,” Brennan tells NR Times.
“These have been designed to address specific needs. For example, the GCS Aid was developed to support training in assessment of the GCS and to enhance reproducibility of assessment. The GCS pupils score and prognostic charts provide a simple but robust prognostic tool that can be used in the clinic.”
“Prognostic scores are helpful for clinicians to get a reliable prediction of patient outcome, to inform clinical decision-making and to support communication with a patient’s family.
“We know from previous research that clinicians can tend to predict overly pessimistic outcomes for patients, particularly those with severe brain injuries, so these prediction models are designed to prevent that. With our imputation tool, the sum score can be determined and prognostic models used in real-time in the clinic.”
The researchers believe that being able to add verbal scores will help clinicians quickly determine the severity of acute brain injury and estimate patient outcomes.
“We know from the enquires we get and from the number of downloads of materials from our website, that these are very popular and are having a positive impact on clinical care around the world.
“We are confident this missing verbal score imputation tool will be just as positively received,” Brennan says.
Update:concussion in sport
A run through the latest developments in concussion in sport research and protocols.
A study published in the May 27 in the medical journal of the American Academy of Neurology, looked at a biomarker called neurofilament light chain, a nerve protein that can be detected in the blood when nerve cells are injured or die.
Levels of the protein in the blood were measured and it was found that those with three or more concussions had an average blood levels of neurofilament light 33 per cent higher than those who had never had a concussion.
“The main finding in the study is that people with multiple concussions have more of these proteins in their blood, even years after the last injury,” said study author Kimbra L. Kenney, M.D of the National Intrepid Center of Excellence.
“Additionally, these proteins may help predict who will experience more severe symptoms such as PTSD and depression. That’s exciting because we may be able to intervene earlier to help lessen the overall effects of concussions over time.”
Following on from our article on the game changing tests into concussion in children it has been found that concussions sustained by high school athletes continues to increase.
Injury data collected from 100 high schools for sports including football, volleyball and wrestling found that, between the academic years 2015 and 2017, the average amount of concussions annually increased 1.012-fold compared to the previous four academic years.
Approximately 300,000 teens suffer concussions or mild traumatic brain injuries each year while playing high school sports.
Wellington Hsu, M.D, professor of orthopedics at Northwestern University’s Feinberg School of Medicine said: “It’s understandable to think that with increased awareness among practitioners who diagnose concussions, the incidence would naturally rise; however because we’ve studied and reported on concussions for a number of years now, I feel that enough time has passed and I would have expected to see the numbers start to level out.
“What we found was that the overall average proportion of concussions reported annually in all sports increased significantly, as did the overall rate of concussions.”
The data also revealed that in gender-matched sports, girls seemingly sustain concussions at a higher rate than boys.
The effects of concussion in young people continues to be a key concern, with links between concussion and football, specifically when heading the ball leading to some big changes when it comes to training guidelines.
Coaches have been advised to update their rules connected to heading the ball in training, with no heading at all in the foundation phase for primary school children and a “graduated approach” to introduce heading training at under-12 to under-16 level. This guidance is expected to be issued across the continent later this year.
These new guidelines were recommended following a FIELD study, joint-funded by the English FA and the Professional Footballers’ Association, published in October last year, finding that professional footballers were three-and-a-half times more likely to die of a neurodegenerative disease than members of the general population of the same age.
The study did not identify a cause for this increased risk, but repeated heading of a ball and other head injuries have been identified as possible factors.
Dr Carol Routledge, director of research at Alzheimer’s Research UK, said: “Limiting unnecessary heading in children’s football is a practical step that minimises possible risks, ensuring that football remains as safe as possible in all forms.
“As such, measures to reduce exposure to unnecessary head impacts and risk of head injury in sport are a logical step. I would, however, like to see these proposals introduced as mandatory, rather than voluntary as present, and a similar approach to reduce heading burden adopted in the wider game of football, not just in youth football.”
A similar stance, that also includes restrictions during matches, has been in place in the US since 2015 after a number of coaches and parents took legal action against the US Soccer Federation.
There is clearly a need to educate coaches and athletes about the concussion recovery process while equipping physicians with quick diagnostic tools.
A partnership between Neurotechnology and brain health analytics player SyncThink and concussion education technology specialist TeachAids aims to offer the latest concussion education combined with mobile, objective measurement technology.
EYE-SYNC, which allows a clinician to use analysis to decipher between brain systems to determine whether a patient may be performing poorly or impaired, will create a brain health education and evaluation system based on the implementation of CrashCourse, an interactive educational module that teaches athletes, parents and coaches about concussions.
This implementation will be available to all SyncThink partners which include top athletic organisations and clinical partners providing medical care and education for over 10,000 high school and college athletes.
This implementation could make tracking those who receive concussion education easier while complying with sport governing bodies educational requirements.
SyncThink founder and medical advisor to TeachAids, Jamshid Ghajar said: “Using the SyncThink platform to feature the CrashCourse educational technology for athletes and coaches is brilliant.
“Now clinicians can use the Eye-Sync tests and metrics alongside CrashCourse’s latest evidence-based information on concussion.”
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