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.

A father with his son on his shoulders in a park.
Childhood dementia is linked to rare genetic disorders. maxim ibragimov/Shutterstock

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.

An old woman holds a young girl on her lap.
Dementia is not just a disease of older people. Miljan Zivkovic/Shutterstock

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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  • Maca Root’s Benefits For The Mood And The Ability

    10almonds is reader-supported. We may, at no cost to you, receive a portion of sales if you purchase a product through a link in this article.

    Maca Root: What It Does And Doesn’t Do

    Maca root, or Lepidium meyenii, gets thought of as a root vegetable, though it’s in fact a cruciferous vegetable and more closely related to cabbage—notwithstanding that it also gets called “Peruvian ginseng”.

    • Nutritionally, it’s full of all manner of nutrients (vitamins, minerals, fiber, and a wide array of phytochemicals)
    • Medicinally, it’s long enjoyed traditional use against a wide variety of illnesses, including respiratory infections and inflammatory diseases.

    It’s also traditionally an aphrodisiac.

    Is it really anti-inflammatory?

    Probably not… Unless fermented. This hasn’t been studied deeply, but a 2023 study found that non-fermented and fermented maca root extracts had opposite effects in this regard:

    Anti-Inflammation and Anti-Melanogenic Effects of Maca Root Extracts Fermented Using Lactobacillus Strains

    However, this was an in vitro study, so we can’t say for sure that the results will carry over to humans.

    Is it really an aphrodisiac?

    Actually yes, it seems so. Here’s a study in which 45 women with antidepressant-induced sexual dysfunction found it significantly improved both libido and sexual function:

    ❝In summary, maca root may alleviate antidepressant-induced sexual dysfunction as women age, particularly in the domain of orgasm❞

    ~ Dr. Christina Dording et al.

    Read in full: A Double-Blind Placebo-Controlled Trial of Maca Root as Treatment for Antidepressant-Induced Sexual Dysfunction in Women

    As for men, well these mice (not technically men) found it beneficial too:

    Effects of combined extracts of Lepidium meyenii and Allium tuberosum Rottl. on [e-word] dysfunction

    (pardon the censorship; we’re trying to avoid people’s spam filters)

    It did also improve fertility (and, actually in real men this time):

    Does Lepidium meyenii (Maca) improve seminal quality?

    Oh, to be in the mood

    Here’s an interesting study in which 3g/day yielded significant mood improvement in these 175 (human) subjects:

    Acceptability, Safety, and Efficacy of Oral Administration of Extracts of Black or Red Maca (Lepidium meyenii) in Adult Human Subjects: A Randomized, Double-Blind, Placebo-Controlled Study

    And yes, it was found to be “well-tolerated” which is scientist-speak for “this appears to be completely safe, but we don’t want to commit ourselves to an absolutist statement and we can’t prove a negative”.

    Oh, to have the energy

    As it turns out, maca root does also offer benefits in this regard too:

    The improvement of daily fatigue in women following the intake of maca (Lepidium meyenii) extract containing benzyl glucosinolate

    (that’s not an added ingredient; it’s just a relevant chemical that the root naturally contains)

    Want to try some?

    We don’t sell it, but here for your convenience is an example product on Amazon 😎

    Enjoy!

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  • Coconut vs Avocado – Which is Healthier?

    10almonds is reader-supported. We may, at no cost to you, receive a portion of sales if you purchase a product through a link in this article.

    Our Verdict

    When comparing coconut to avocado, we picked the avocado.

    Why?

    In terms of macros, avocado is lower in carbs and also in net carbscoconut’s a little higher in fiber, but not enough to make up for the difference in carbs nor, when it comes to glycemic index and insulin index, the impact of coconut’s much higher fat content on insulin responses too. On which note, while coconut’s fats are broadly considered healthy (its impressive saturated fat content is formed of medium-chain triglycerides which, in moderation, are heart-healthy), avocado’s fats are even healthier, being mostly monounsaturated fat with some polyunsaturated (and about 15x less saturated fat). All in all, a fair win for avocado on the macros front, but coconut isn’t bad in moderation.

    When it comes to vitamins, avocados are higher in vitamins A, B1, B2, B3, B5, B6, B9, C, E, K, and choline. Most of those differences are by very large margins. Coconuts are not higher in any vitamins. A huge, easy, “perfect score” win for avocados.

    In the category of minerals, however, it’s coconut’s turn to sweep with more calcium, copper, iron, magnesium, manganese, phosphorus, zinc, and selenium—though the margins are mostly not nearly as impressive as avocado’s vitamin margins. Speaking of avocados, they do have more potassium than coconuts do, but the margin isn’t very large. A compelling win for coconut’s mineral content.

    Adding up the sections, we get to a very credible win for avocados, but coconuts are also very respectable. So, as ever, enjoy both (although we do recommend exercising moderation in the case of coconuts, mainly because of the saturated fat content), and if you’re choosing between them for some purpose, then avocado will generally be the best option.

    Want to learn more?

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    Take care!

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  • Unfuck Your Brain – by Dr. Faith Harper

    10almonds is reader-supported. We may, at no cost to you, receive a portion of sales if you purchase a product through a link in this article.

    This book takes a trauma-informed care approach, which is relatively novel in the mental health field and it’s quickly becoming the industry standard because of its effectiveness.

    The basic premise of trauma-informed care is that you had a bad experience (possibly even more than one—what a thought!) and that things that remind you of that will tend to prompt reactivity from you in a way that probably isn’t healthy. By identifying each part of that process, we can then interrupt it, much like we might with CBT (the main difference being that CBT, for all its effectiveness, tends to assume that the things that are bothering you are not true, while TIC acknowledges that they might well be, and that especially historically, they probably were).

    A word of warning: if something that triggers a trauma-based reactivity response in you is people swearing, then this book will either cure you by exposure therapy or leave you a nervous wreck, because it’s not just the title; Dr. Harper barely gets through a sentence without swearing. It’s a lot, even by this (European) reviewer’s standards (we’re a lot more relaxed about swearing over here, than people tend to be in America).

    On the other hand, something that Dr. Harper excels at is actually explaining stuff very well. So while it sometimes seems like she’s “trying too hard” style-wise in terms of being “not like other therapists”, in her defence she’s nevertheless a very good writer; she knows her stuff, and knows how to communicate it clearly.

    Bottom line: if you don’t mind a writer who swears more than 99% of soldiers, then this book is an excellent how-to guide for self-administered trauma-informed care.

    Click here to check out Unfuck Your Brain, and indeed unfuck it!

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    The author argues that often, the problem with our bodies is not actually our bodies. If we have cancer, or diabetes, then sure, that’s a problem with the body. But most of the time, the “problem with our bodies” is simply society’s rejection of our “imperfect” bodies as somehow “less than”, and something we must invest time and money to correct. Hence, the need for a radical uprooting of ideas, to fix the real problem.

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  • Exercise with Type 1 Diabetes – by Ginger Vieira

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  • Nudge – by Richard Thaler & Cass Sunstein

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    How often in life do we make a suboptimal decision that ends up plaguing us for a long time afterwards? Sometimes, a single good or bad decision can even directly change the rest of our life.

    So, it really is important that we try to optimize the decisions we do make.

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