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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  • Keep Inflammation At Bay

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    How to Prevent (or Reduce) Inflammation

    You asked us to do a main feature on inflammation, so here we go!

    Before we start, it’s worth noting an important difference between acute and chronic inflammation:

    • Acute inflammation is generally when the body detects some invader, and goes to war against it. This (except in cases such as allergic responses) is usually helpful.
    • Chronic inflammation is generally when the body does a civil war. This is almost never helpful.

    We’ll be tackling the latter, which frees up your body’s resources to do better at the former.

    First, the obvious…

    These five things are as important for this as they are for most things:

    1. Get a good dietthe Mediterranean diet is once again a top-scorer
    2. Exercisemove and stretch your body; don’t overdo it, but do what you reasonably can, or the inflammation will get worse.
    3. Reduce (or ideally eliminate) alcohol consumption. When in pain, it’s easy to turn to the bottle, and say “isn’t this one of red wine’s benefits?” (it isn’t, functionally*). Alcohol will cause your inflammation to flare up like little else.
    4. Don’t smoke—it’s bad for everything, and that goes for inflammation too.
    5. Get good sleep. Obviously this can be difficult with chronic pain, but do take your sleep seriously. For example, invest in a good mattress, nice bedding, a good bedtime routine, etc.

    *Resveratrol (which is a polyphenol, by the way), famously found in red wine, does have anti-inflammatory properties. However, to get enough resveratrol to be of benefit would require drinking far more wine than will be good for your inflammation or, indeed, the rest of you. So if you’d like resveratrol benefits, consider taking it as a supplement. Superficially it doesn’t seem as much fun as drinking red wine, but we assure you that the results will be much more fun than the inflammation flare-up after drinking.

    About the Mediterranean Diet for this…

    There are many causes of chronic inflammation, but here are some studies done with some of the most common ones:

    *Type 1 diabetes is a congenital autoimmune disorder, as the pancreas goes to war with itself. Type 2 diabetes is different, being a) acquired and b) primarily about insulin resistance, and/but this is related to chronic inflammation regardless. It is also possible to have T1D and go on to develop insulin resistance, and that’s very bad, and/but beyond the scope of today’s newsletter, in which we are focusing on the inflammation aspects.

    Some specific foods to eat or avoid…

    Eat these:

    • Leafy greens
    • Cruciferous vegetables
    • Tomatoes
    • Fruits in general (berries in particular)
    • Healthy fats, e.g. olives and olive oil
    • Almonds and other nuts
    • Dark chocolate (choose high cocoa, low sugar)

    Avoid these:

    • Processed meats (absolute worst offenders are hot dogs, followed by sausages in general)
    • Red meats
    • Sugar (includes most fruit juices, but not most actual fruits—the difference with actual fruits is they still contain plenty of fiber, and in many cases, antioxidants/polyphenols that reduce inflammation)
    • Dairy products (unless fermented, in which case it seems to be at worst neutral, sometimes even a benefit, in moderation)
    • White flour (and white flour products, e.g. white bread, white pasta, etc)
    • Processed vegetable oils

    See also: 9 Best Drinks To Reduce Inflammation, Says Science

    Supplements?

    Some supplements that have been found to reduce inflammation include:

    (links are to studies showing their efficacy)

    Consider Intermittent Fasting

    Remember when we talked about the difference between acute and chronic inflammation? It’s fair to wonder “if I reduce my inflammatory response, will I be weakening my immune system?”, and the answer is: generally, no.

    Often, as with the above supplements and dietary considerations, reducing inflammation actually results in a better immune response when it’s actually needed! This is because your immune system works better when it hasn’t been working in overdrive constantly.

    Here’s another good example: intermittent fasting reduces the number of circulating monocytes (a way of measuring inflammation) in healthy humans—but doesn‘t compromise antimicrobial (e.g. against bacteria and viruses) immune response.

    See for yourself: Dietary Intake Regulates the Circulating Inflammatory Monocyte Pool ← the study is about the anti-inflammatory effects of fasting

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  • Celery vs Radish – Which is Healthier?

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    Our Verdict

    When comparing celery to radish, we picked the celery.

    Why?

    It was very close! And yes, surprising, we know. Generally speaking, the more colorful/pigmented an edible plant is, the healthier it is. Celery is just one of those weird exceptions (as is cauliflower, by the way).

    Macros-wise, these two are pretty much the same—95% water, with just enough other stuff to hold them together. The proportions of “other stuff” are also pretty much equal.

    In the category of vitamins, celery has more vitamin K while radish has more vitamin C; the other vitamins are pretty close to equal. We’ll call this one a minor win for celery, as vitamin K is found in fewer foods than vitamin C.

    When it comes to minerals, celery has more calcium, manganese, phosphorus, and potassium, while radish has more copper, iron, selenium, and zinc. We’ll call this a minor win for radish, as the margins are a little wider for its minerals.

    So, that makes the score 1–1 so far.

    Both plants have an assortment of polyphenols, of which, when we add up the averages, celery comes out on top by some way. Celery also comes out on top when we do a head-to-head of the top flavonoid of each; celery has 5.15mg/100g of apigenin to radish’s 0.63mg/100g kaempferol.

    Which means, both are great healthy foods, but celery wins the day.

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  • Can Medical Schools Funnel More Doctors Into the Primary Care Pipeline?

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    Throughout her childhood, Julia Lo Cascio dreamed of becoming a pediatrician. So, when applying to medical school, she was thrilled to discover a new, small school founded specifically to train primary care doctors: NYU Grossman Long Island School of Medicine.

    Now in her final year at the Mineola, New York, school, Lo Cascio remains committed to primary care pediatrics. But many young doctors choose otherwise as they leave medical school for their residencies. In 2024, 252 of the nation’s 3,139 pediatric residency slots went unfilled and family medicine programs faced 636 vacant residencies out of 5,231 as students chased higher-paying specialties.

    Lo Cascio, 24, said her three-year accelerated program nurtured her goal of becoming a pediatrician. Could other medical schools do more to promote primary care? The question could not be more urgent. The Association of American Medical Colleges projects a shortage of 20,200 to 40,400 primary care doctors by 2036. This means many Americans will lose out on the benefits of primary care, which research shows improves health, leading to fewer hospital visits and less chronic illness.

    Many medical students start out expressing interest in primary care. Then they end up at schools based in academic medical centers, where students become enthralled by complex cases in hospitals, while witnessing little primary care.

    The driving force is often money, said Andrew Bazemore, a physician and a senior vice president at the American Board of Family Medicine. “Subspecialties tend to generate a lot of wealth, not only for the individual specialists, but for the whole system in the hospital,” he said.

    A department’s cache of federal and pharmaceutical-company grants often determines its size and prestige, he said. And at least 12 medical schools, including Harvard, Yale, and Johns Hopkins, don’t even have full-fledged family medicine departments. Students at these schools can study internal medicine, but many of those graduates end up choosing subspecialties like gastroenterology or cardiology.

    One potential solution: eliminate tuition, in the hope that debt-free students will base their career choice on passion rather than paycheck. In 2024, two elite medical schools — the Albert Einstein College of Medicine and the Johns Hopkins University School of Medicine — announced that charitable donations are enabling them to waive tuition, joining a handful of other tuition-free schools.

    But the contrast between the school Lo Cascio attends and the institution that founded it starkly illustrates the limitations of this approach. Neither charges tuition.

    In 2024, two-thirds of students graduating from her Long Island school chose residencies in primary care. Lo Cascio said the tuition waiver wasn’t a deciding factor in choosing pediatrics, among the lowest-paid specialties, with an average annual income of $260,000, according to Medscape.

    At the sister school, the Manhattan-based NYU Grossman School of Medicine, the majority of its 2024 graduates chose specialties like orthopedics (averaging $558,000 a year) or dermatology ($479,000).

    Primary care typically gets little respect. Professors and peers alike admonish students: If you’re so smart, why would you choose primary care? Anand Chukka, 27, said he has heard that refrain regularly throughout his years as a student at Harvard Medical School. Even his parents, both PhD scientists, wondered if he was wasting his education by pursuing primary care.

    Seemingly minor issues can influence students’ decisions, Chukka said. He recalls envying the students on hospital rotations who routinely were served lunch, while those in primary care settings had to fetch their own.

    Despite such headwinds, Chukka, now in his final year, remains enthusiastic about primary care. He has long wanted to care for poor and other underserved people, and a one-year clerkship at a community practice serving low-income patients reinforced that plan.

    When students look to the future, especially if they haven’t had such exposure, primary care can seem grim, burdened with time-consuming administrative tasks, such as seeking prior authorizations from insurers and grappling with electronic medical records.

    While specialists may also face bureaucracy, primary care practices have it much worse: They have more patients and less money to hire help amid burgeoning paperwork requirements, said Caroline Richardson, chair of family medicine at Brown University’s Warren Alpert Medical School.

    “It’s not the medical schools that are the problem; it’s the job,” Richardson said. “The job is too toxic.”

    Kevin Grumbach, a professor of family and community medicine at the University of California-San Francisco, spent decades trying to boost the share of students choosing primary care, only to conclude: “There’s really very little that we can do in medical school to change people’s career trajectories.”

    Instead, he said, the U.S. health care system must address the low pay and lack of support.

    And yet, some schools find a way to produce significant proportions of primary care doctors — through recruitment and programs that provide positive experiences and mentors.

    U.S. News & World Report recently ranked 168 medical schools by the percentage of graduates who were practicing primary care six to eight years after graduation.

    The top 10 schools are all osteopathic medical schools, with 41% to 47% of their students still practicing primary care. Unlike allopathic medical schools, which award MD degrees, osteopathic schools, which award DO degrees, have a history of focusing on primary care and are graduating a growing share of the nation’s primary care physicians.

    At the bottom of the U.S. News list is Yale, with 10.7% of its graduates finding lasting careers in primary care. Other elite schools have similar rates: Johns Hopkins, 13.1%; Harvard, 13.7%.

    In contrast, public universities that have made it a mission to promote primary care have much higher numbers.

    The University of Washington — No. 18 in the ranking, with 36.9% of graduates working in primary care — has a decades-old program placing students in remote parts of Washington, Wyoming, Alaska, Montana, and Idaho. UW recruits students from those areas, and many go back to practice there, with more than 20% of graduates settling in rural communities, according to Joshua Jauregui, assistant dean for clinical curriculum.

    Likewise, the University of California-Davis (No. 22, with 36.3% of graduates in primary care) increased the percentage of students choosing family medicine from 12% in 2009 to 18% in 2023, even as it ranks high in specialty training. Programs such as an accelerated three-year primary care “pathway,” which enrolls primarily first-generation college students, help sustain interest in non-specialty medical fields.

    The effort starts with recruitment, looking beyond test scores to the life experiences that forge the compassionate, humanistic doctors most needed in primary care, said Mark Henderson, associate dean for admissions and outreach. Most of the students have families who struggle to get primary care, he said. “So they care a lot about it, and it’s not just an intellectual, abstract sense.”

    Establishing schools dedicated to primary care, like the one on Long Island, is not a solution in the eyes of some advocates, who consider primary care the backbone of medicine and not a separate discipline. Toyese Oyeyemi Jr., executive director of the Social Mission Alliance at the Fitzhugh Mullan Institute of Health Workforce Equity, worries that establishing such schools might let others “off the hook.”

    Still, attending a medical school created to produce primary care doctors worked out well for Lo Cascio. Although she underwent the usual specialty rotations, her passion for pediatrics never flagged — owing to her 23 classmates, two mentors, and her first-year clerkship shadowing a community pediatrician. Now, she’s applying for pediatric residencies.

    Lo Cascio also has deep personal reasons: Throughout her experience with a congenital heart condition, her pediatrician was a “guiding light.”

    “No matter what else has happened in school, in life, in the world, and medically, your pediatrician is the person that you can come back to,” she said. “What a beautiful opportunity it would be to be that for someone else.”

    KFF Health News is a national newsroom that produces in-depth journalism about health issues and is one of the core operating programs at KFF—an independent source of health policy research, polling, and journalism. Learn more about KFF.

    Subscribe to KFF Health News’ free Morning Briefing.

    This article first appeared on KFF Health News and is republished here under a Creative Commons license.

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  • How To Stop Binge-Eating: Flip This Switch!

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    “The Big Eating Therapist” Sarah Dosanjh has insights from both personal and professional experience:

    No “Tough Love” Necessary

    Eating certain foods is often socially shamed, and it’s easy to internalize that, and feel guilty. While often guilt is considered a pro-social emotion that helps people to avoid erring in a way that will get us excluded from the tribe (bearing in mind that for most of our evolutionary history, exile would mean near-certain death), it is not good at behavior modification when it comes to addictions or anything similar to addictions.

    The reason for this is that if we indulge in a pleasure we feel we “shouldn’t” and expect we’d be shamed for, we then feel bad, and we immediately want something to make us feel better. Guess what that something will be. That’s right: the very same thing we literally just felt ashamed about.

    So guilt is not helpful when it comes to (for example) avoiding binge-eating.

    Instead, Dosanjh points us to a study whereby dieters ate a donut and drank water, before being given candy for taste testing. The control group proceeded without intervention, while the experimental group had a self-compassion intervention between the donut and the candy. This meant that researchers told the participants not to feel bad about eating the donut, emphasizing self-kindness, mindfulness, and common humanity. The study found that those who received the intervention, ate significantly less candy.

    What we can learn from this is: we must be kind to ourselves. Allowing ourselves, consciously and mindfully, “a little treat”, secures its status as being “little”, and “a treat”. Then we smile, thinking “yes, that was a nice little thing to do for myself”, and proceed with our day.

    This kind of self-compassion helps avoid the “meta-binge” process, where guilt from one thing leads to immediately reaching for another.

    For more on this, plus a link to the study she mentioned, enjoy:

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  • What Most People Don’t Know About Hearing Aids

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    Dr. Juliëtte Sterkens, a doctor of audiology, makes things clearer in this TEDx talk:

    The sound of the future

    Half of all adults experience hearing loss by the age of 75, and by 85, that goes up to two thirds. Untreated hearing loss leads to depression, social isolation, cognitive decline, and even an increased fall risk.

    It’s not just about reduced volume though; Dr. Sterkens points out that for many (like this writer!) it’s more a matter of unequal pitch perception and difficulty in speech clarity. Most hearing aids just amplify sound, and don’t fully restore clarity, especially beyond a short range.

    However, technology keeps marching forwards there have been improvements in the move from analog to digital, and today’s bluetooth-enabled hearing aids often do a lot better, especially in the case of things like TV transmitters and clip-on microphones.

    Out and about, you might see signs sometimes saying “Hearing Loop Enabled”, and those transmit sound directly to telecoil-equipped hearing aids—venues with public address systems are legally required to provide hearing accommodations like this. Many hearing aids include telecoils, but users often aren’t informed or don’t have them activated, which is unfortunate, because telecoils improve hearing dramatically in loop-enabled venues.

    Dr. Sterkens makes a plea for us to, as applicable,

    • Activate telecoils and insist on them in new hearing aids.
    • Advocate for assistive listening systems in public venues.
    • Use available resources like the Hearing Loss Association of America for tools and information.
    • Familiarize ourselves with accessibility laws and report non-compliance.
    • Aim to make the world more accessible for people with hearing loss through advocacy, technology, and awareness.

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    Dealing With Hearing Loss

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  • Break the Cycle – by Dr. Mariel Buqué

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    Intergenerational trauma comes in two main varieties: epigenetic, and behavioral.

    This book covers both. There’s a lot more we can do about the behavioral side than the epigenetic, but that’s not to say that Dr. Buqué doesn’t have useful input in the latter kind too.

    If you’ve read other books on epigenetic trauma, then there’s nothing new here—though the refresher is always welcome.

    On the behavioral side, Dr. Buqué gives a strong focus on practical techniques, such as specific methods of journaling to isolate trauma-generated beliefs and resultant behaviors, with a view to creating one’s own trauma-informed care, cutting through the cycle, and stopping it there.

    Which, of course, will not only be better for you, but also for anyone who will be affected by how you are (e.g. now/soon, hopefully better).

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    Click here to check out Break the Cycle, and do just that!

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