What is childhood dementia? And how could new research help?
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“Childhood” and “dementia” are two words we wish we didn’t have to use together. But sadly, around 1,400 Australian children and young people live with currently untreatable childhood dementia.
Broadly speaking, childhood dementia is caused by any one of more than 100 rare genetic disorders. Although the causes differ from dementia acquired later in life, the progressive nature of the illness is the same.
Half of infants and children diagnosed with childhood dementia will not reach their tenth birthday, and most will die before turning 18.
Yet this devastating condition has lacked awareness, and importantly, the research attention needed to work towards treatments and a cure.
More about the causes
Most types of childhood dementia are caused by mutations (or mistakes) in our DNA. These mistakes lead to a range of rare genetic disorders, which in turn cause childhood dementia.
Two-thirds of childhood dementia disorders are caused by “inborn errors of metabolism”. This means the metabolic pathways involved in the breakdown of carbohydrates, lipids, fatty acids and proteins in the body fail.
As a result, nerve pathways fail to function, neurons (nerve cells that send messages around the body) die, and progressive cognitive decline occurs.
What happens to children with childhood dementia?
Most children initially appear unaffected. But after a period of apparently normal development, children with childhood dementia progressively lose all previously acquired skills and abilities, such as talking, walking, learning, remembering and reasoning.
Childhood dementia also leads to significant changes in behaviour, such as aggression and hyperactivity. Severe sleep disturbance is common and vision and hearing can also be affected. Many children have seizures.
The age when symptoms start can vary, depending partly on the particular genetic disorder causing the dementia, but the average is around two years old. The symptoms are caused by significant, progressive brain damage.
Are there any treatments available?
Childhood dementia treatments currently under evaluation or approved are for a very limited number of disorders, and are only available in some parts of the world. These include gene replacement, gene-modified cell therapy and protein or enzyme replacement therapy. Enzyme replacement therapy is available in Australia for one form of childhood dementia. These therapies attempt to “fix” the problems causing the disease, and have shown promising results.
Other experimental therapies include ones that target faulty protein production or reduce inflammation in the brain.
Research attention is lacking
Death rates for Australian children with cancer nearly halved between 1997 and 2017 thanks to research that has enabled the development of multiple treatments. But over recent decades, nothing has changed for children with dementia.
In 2017–2023, research for childhood cancer received over four times more funding per patient compared to funding for childhood dementia. This is despite childhood dementia causing a similar number of deaths each year as childhood cancer.
The success for childhood cancer sufferers in recent decades demonstrates how adequately funding medical research can lead to improvements in patient outcomes.
Another bottleneck for childhood dementia patients in Australia is the lack of access to clinical trials. An analysis published in March this year showed that in December 2023, only two clinical trials were recruiting patients with childhood dementia in Australia.
Worldwide however, 54 trials were recruiting, meaning Australian patients and their families are left watching patients in other parts of the world receive potentially lifesaving treatments, with no recourse themselves.
That said, we’ve seen a slowing in the establishment of clinical trials for childhood dementia across the world in recent years.
In addition, we know from consultation with families that current care and support systems are not meeting the needs of children with dementia and their families.
New research
Recently, we were awarded new funding for our research on childhood dementia. This will help us continue and expand studies that seek to develop lifesaving treatments.
More broadly, we need to see increased funding in Australia and around the world for research to develop and translate treatments for the broad spectrum of childhood dementia conditions.
Dr Kristina Elvidge, head of research at the Childhood Dementia Initiative, and Megan Maack, director and CEO, contributed to this article.
Kim Hemsley, Head, Childhood Dementia Research Group, Flinders Health and Medical Research Institute, College of Medicine and Public Health, Flinders University; Nicholas Smith, Head, Paediatric Neurodegenerative Diseases Research Group, University of Adelaide, and Siti Mubarokah, Research Associate, Childhood Dementia Research Group, Flinders Health and Medical Research Institute, College of Medicine and Public Health, Flinders University
This article is republished from The Conversation under a Creative Commons license. Read the original article.
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A drug that can extend your life by 25%? Don’t hold your breath
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Every few weeks or months, the media reports on a new study that tantalisingly dangles the possibility of a new drug to give us longer, healthier lives.
The latest study centres around a drug involved in targeting interleukin-11, a protein involved in inflammation. Blocking this protein appeared to help mice stave off disease and extend their life by more than 20%.
If only defying the ravages of time could be achieved through such a simple and effort-free way – by taking a pill. But as is so often the case, the real-world significance of these findings falls a fair way short of the hype.
Halfpoint/Shutterstock The role of inflammation in disease and ageing
Chronic inflammation in the body plays a role in causing disease and accelerating ageing. In fact, a relatively new label has been coined to represent this: “inflammaging”.
While acute inflammation is an important response to infection or injury, if inflammation persists in the body, it can be very damaging.
A number of lifestyle, environmental and societal drivers contribute to chronic inflammation in the modern world. These are largely the factors we already know are associated with disease and ageing, including poor diet, lack of exercise, obesity, stress, lack of sleep, lack of social connection and pollution.
While addressing these issues directly is one of the keys to addressing chronic inflammation, disease and ageing, there are a number of research groups also exploring how to treat chronic inflammation with pharmaceuticals. Their goal is to target and modify the molecular and chemical pathways involved in the inflammatory process itself.
What the latest research shows
This new interleukin-11 research was conducted in mice and involved a number of separate components.
In one component of this research, interleukin-11 was genetically knocked out in mice. This means the gene for this chemical mediator was removed from these mice, resulting in the mice no longer being able to produce this mediator at all.
In this part of the study, the mice’s lives were extended by over 20%, on average.
Another component of this research involved treating older mice with a drug that blocks interleukin-11.
Injecting this drug into 75-week old mice (equivalent to 55-year-old humans) was found to extend the life of mice by 22-25%.
These treated mice were less likely to get cancer and had lower cholesterol levels, lower body weight and improved muscle strength and metabolism.
From these combined results, the authors concluded, quite reasonably, that blocking interleukin-11 may potentially be a key to mitigating age-related health effects and improving lifespan in both mice and humans.
Why you shouldn’t be getting excited just yet
There are several reasons to be cautious of these findings.
First and most importantly, this was a study in mice. It may be stating the obvious, but mice are very different to humans. As such, this finding in a mouse model is a long way down the evidence hierarchy in terms of its weight.
Research shows only about 5% of promising findings in animals carry over to humans. Put another way, approximately 95% of promising findings in animals may not be translated to specific therapies for humans.
Second, this is only one study. Ideally, we would be looking to have these findings confirmed by other researchers before even considering moving on to the next stage in the knowledge discovery process and examining whether these findings may be true for humans.
We generally require a larger body of evidence before we get too excited about any new research findings and even consider the possibility of human trials.
Third, even if everything remains positive and follow-up studies support the findings of this current study, it can take decades for a new finding like this to be translated to successful therapies in humans.
Until then, we can focus on doing the things we already know make a huge difference to health and longevity: eating well, exercising, maintaining a healthy weight, reducing stress and nurturing social relationships.
Hassan Vally, Associate Professor, Epidemiology, Deakin University
This article is republished from The Conversation under a Creative Commons license. Read the original article.
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Tuna Steak with Protein Salad
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Yes, it’s protein on protein today, and it’s all healthy.
You will need (per person)
- 1 tuna steak
- 1 400g/12oz can mixed beans, drained & rinsed
- 1 tsp capers
- 2 tsp black pepper, coarse ground
- 1 red chili, chopped
- 1 lime, cut into wedges
- ½ tsp white wine vinegar
- Extra virgin olive oil, for cooking
- Garnish: chopped parsley
Method
(we suggest you read everything at least once before doing anything)
1) Put the beans in a bowl, mixing in the capers, vinegar, and 1 tsp of the black pepper
2) Gently rub a little olive oil onto each side of the tuna steak, and season with the remainder of the black pepper (as in, the other tsp, not the rest of what you have in the house).
3) Heat a ridged grill pan until hot, and then cook the tuna for around 3 minutes on each side. Do not jiggle it! Do not slide it, and definitely do not stir it. Just gently turn it over when necessary. The edges should be cooked, and the inside should still be pink (it’s easy to forget when it comes from a can, but remember tuna is usually eaten raw)
4) Serve, sprinkling with the chopped chili and garnishing with the parsley. The lime wedges go on the side for squeezing at the table.
Enjoy!
Want to learn more?
For those interested in some of the science of what we have going on today:
- Protein: How Much Do We Need, Really?
- Salmon vs Tuna – Which is Healthier?
- Cilantro vs Parsley – Which is Healthier?
- Our Top 5 Spices: How Much Is Enough For Benefits?
Take care!
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The Orchid That Renovates Your Gut (Gently)
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The Orchid That Renovates Your Gut (Gently)
Dendrobium officinale is an orchid that’s made its way from Traditional Chinese Medicine into modern science.
Read: Traditional Uses, Phytochemistry, Pharmacology, and Quality Control of Dendrobium officinale
To summarize its benefits, we’ll quote from Dr. Paharia’s article featured in our “what’s happening in the health world” section all so recently:
❝Gut microbes process Dendriobium officinale polysaccharides (DOPs) in the colon, producing short-chain fatty acids (SCFAs) and oligosaccharides that alter gut microbial composition and improve human health.
DOPs have been shown to decrease harmful bacteria like E. coli and Staphylococcus while promoting beneficial ones like Bifidobacterium.❞
We don’t stop at secondary sources, though, so we took a look at the science.
Dr. Wu et al. found (we’ll quote directly for these bullet points):
- DOPs have been shown to influence the gut microbiota, such as the abundance of Lactobacillus, Bifidobacterium, Akkermansia, Bacteroides, and Prevotella, and provide different benefits to the host due to structural differences.
- The dietary intake of DOPs has been shown to improve the composition of the gut microbiome and offers new intervention strategies for metabolic diseases such as obesity and type 2 diabetes as well as inflammatory diseases such as chronic obstructive pulmonary disease and colitis.
- Compared to drug therapy, intervention with DOPs is not specific and has a longer intervention duration
This is consistent with previous research on Dendrobium officinale, such as last year’s:
❝DOP significantly increased benign intestinal microbe proportion (Lactobacillus, etc.), but reduced harmful bacteria (Escherichia shigella) (P < 0.05), and significantly increased butyric acid production (P < 0.05)❞
In summary…
Research so far indicates that this does a lot of good for the gut, in a way that can “kickstart” healthier, self-regulating gut microbiota.
As to its further prospects, check out:
Very promising!
Where can I get it?
We don’t sell it, but for your convenience here’s an example product on Amazon
Be warned, it is expensive though!
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Super-Nutritious Shchi
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Today we have a recipe we’ve mentioned before, but now we have standalone recipe pages for recipes, so here we go. The dish of the day is shchi—which is Russian cabbage soup, which sounds terrible, and looks as bad as it sounds. But it tastes delicious, is an incredible comfort food, and is famous (in Russia, at least) for being something one can eat for many days in a row without getting sick of it.
It’s also got an amazing nutritional profile, with vitamins A, B, C, D, as well as lots of calcium, magnesium, and iron (amongst other minerals), and a healthy blend of carbohydrates, proteins, and fats, plus an array of anti-inflammatory phytochemicals, and of course, water.
You will need
- 1 large white cabbage, shredded
- 1 cup red lentils
- ½ lb tomatoes, cut into eighths (as in: halve them, halve the halves, and halve the quarters)
- ½ lb mushrooms sliced (or halved, if they are baby button mushrooms)
- 1 large onion, chopped finely
- 1 tbsp rosemary, chopped finely
- 1 tbsp thyme, chopped finely
- 1 tbsp black pepper, coarse ground
- 1 tsp cumin, ground
- 1 tsp yeast extract
- 1 tsp MSG, or 2 tsp low-sodium salt
- A little parsley for garnishing
- A little fat for cooking; this one’s a tricky and personal decision. Butter is traditional, but would make this recipe impossible to cook without going over the recommended limit for saturated fat. Avocado oil is healthy, relatively neutral in taste, and has a high smoke point for caramelizing the onions. Extra virgin olive oil is also a healthy choice, but not as neutral in flavor and does have a lower smoke point. Coconut oil has far too strong a taste and a low smoke point. Seed oils are very heart-unhealthy. All in all, avocado oil is a respectable choice from all angles except tradition.
Note: with regard to the seasonings, the above is a basic starting guide; feel free to add more per your preference—however, we do not recommend adding more cumin (it’ll overpower it) or more salt (there’s enough sodium in here already).
Method
(we suggest you read everything at least once before doing anything)
1) Cook the lentils until soft (a rice cooker is great for this, but a saucepan is fine); be generous with the water; we are making a soup, after all. Set them aside without draining.
2) Sauté the cabbage, and put it in a big stock pot or similar large pan (not yet on the heat)
3) Fry the mushrooms, and add them to the big pot (still not yet on the heat)
4) Use a stick blender to blend the lentils in the water you cooked them in, and then add to the big pot too.
5) Turn the heat on low, and if necessary, add more water to make it into a rich soup
6) Add the seasonings (rosemary, thyme, cumin, black pepper, yeast extract, MSG-or-salt) and stir well. Keep the temperature on low; you can just let it simmer now because the next step is going to take a while:
7) Caramelize the onion (keep an eye on the big pot, stirring occasionally) and set it aside
8) Fry the tomatoes quickly (we want them cooked, but just barely) and add them to the big pot
9) Serve! The caramelized onion is a garnish, so put a little on top of each bowl of shchi. Add a little parsley too.
Enjoy!
Want to learn more?
For those interested in some of the science of what we have going on today:
- Level-Up Your Fiber Intake! (Without Difficulty Or Discomfort)
- The Magic Of Mushrooms: “The Longevity Vitamin” (That’s Not A Vitamin)
- Easily Digestible Vegetarian Protein Sources
- The Bare-Bones Truth About Osteoporosis
- Some Surprising Truths About Hunger And Satiety
Take care!
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Which Vitamin Brands Are Effective?
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It’s Q&A Day at 10almonds!
Have a question or a request? You can always hit “reply” to any of our emails, or use the feedback widget at the bottom!
In cases where we’ve already covered something, we might link to what we wrote before, but will always be happy to revisit any of our topics again in the future too—there’s always more to say!
As ever: if the question/request can be answered briefly, we’ll do it here in our Q&A Thursday edition. If not, we’ll make a main feature of it shortly afterwards!
So, no question/request too big or small
❝As far as specific brands of vitamin…some are good some not. I don’t like being told what buy but I guess I want to know which are effective. Could there be some brands recognized as good given to us?❞
The most reliable brands are generally those with the most transparency:
- They tell you what is in the supplement; not just the active ingredient(s), with doses, but also any buffers etc.
- They tell you, in the case of ingredients that can have various different sources, what the source is.
- They are, ideally, well-certified and independently tested.
Our previous sponsor Ora is a good example of a company that does this.
Additionally, in terms of bioavailability, generally speaking the order of preference goes liquid > capsule/softgel > tablet, so that’s something to look out for, too.
Note: “liquid” includes powders that are ingested when dissolved/suspended in water, and also includes tablets that become a liquid when dissolved/dispersed in water and ingested that way.
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Intuitive Eating – by Evelyn Tribole and Elyse Resch
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You may be given to wonder: if this is about intuitive eating, and an anti-diet approach, why a whole book?
There’s a clue in the other part of the title: “4th Edition”.
The reason there’s a 4th edition (and before it, a 3rd and 2nd edition) is because this book is very much full of science, and science begets more science, and the evidence just keeps on rolling in.
While neither author is a doctor, each has a sizeable portion of the alphabet after their name (more than a lot of doctors), and this is an incredibly well-evidenced book.
The basic premise from many studies is that restrictive dieting does not work well long-term for most people, and instead, better is to make use of our bodies’ own interoceptive feedback.
You see, intuitive eating is not “eat randomly”. We do not call a person “intuitive” because they speak or act randomly, do we? Same with diet.
Instead, the authors give us ten guiding principles (yes, still following the science) to allow us a consistent “finger on the pulse” of what our body has to say about what we have been eating, and what we should be eating.
Bottom line: if you want to be a lot more in tune with your body and thus better able to nourish it the way it needs, this book is literally on the syllabus for many nutritional science classes, and will stand you in very good stead!
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