Unveiling Lesser-Known Dementia Class We Have Surprising Limited Knowledge About

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“Childhood” and “dementia” pairings are unwelcome, yet regrettably, roughly 1,400 Australian children and youth grapple with untreatable childhood dementia.

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Generally, childhood dementia stems from over 100 rare genetic disorders. Despite differing origins from dementia onset later in life, the disease’s progression remains consistent.

Approximately half of diagnosed infants and children with childhood dementia won’t reach age ten, and the majority will pass before reaching 18.

This heartbreaking condition has suffered from lack of awareness and, importantly, the necessary research focus for pursuing treatments and a cure.

Most forms of childhood dementia result from DNA mutations. These errors lead to various rare genetic disorders, triggering childhood dementia.

Two-thirds of childhood dementia cases arise from “inborn errors of metabolism,” disrupting the body’s metabolic pathways for processing carbohydrates, lipids, fatty acids, and proteins.

Consequently, nerve pathways falter, neurons perish, and cognitive decline ensues progressively.

Initially, most children seem unaffected. However, following a period of seemingly normal development, children with childhood dementia gradually lose previously acquired skills, including speech, mobility, learning, memory, and logical thinking.

Childhood dementia also induces significant behavioral changes like aggression, hyperactivity, frequent sleep disturbances, and potential impacts on vision and hearing, alongside frequent seizures.

Symptom onset varies, influenced partly by the specific genetic disorder causing the dementia, with an average onset around age two. Symptoms result from significant, progressive brain damage.

Presently, treatments under evaluation or approved for childhood dementia target only a limited number of disorders and are available in select regions globally. These treatments encompass gene replacement, gene-modified cell therapy, and protein or enzyme replacement therapy.

Enzyme replacement therapy, available in Australia for one childhood dementia form, aims to rectify underlying issues and has shown promising outcomes.

Other experimental therapies target faulty protein production or aim to reduce brain inflammation.

Between 1997 and 2017, Australian childhood cancer mortality rates nearly halved, attributed to substantial research investment leading to multiple treatment advancements. Conversely, childhood dementia research hasn’t seen such progress.

From 2017 to 2023, childhood cancer research received over four times the funding per patient compared to childhood dementia research, despite similar annual mortality rates.

This discrepancy underscores how adequate research funding can enhance patient outcomes, as demonstrated in childhood cancer care.

Moreover, Australian childhood dementia patients face limited access to clinical trials, with only two trials recruiting participants as of December 2023, compared to 54 worldwide.

However, recent years have witnessed a slowdown in establishing clinical trials for childhood dementia globally.

Furthermore, feedback from families highlights inadequacies in current care and support systems for children with dementia and their families.

Fortunately, our research on childhood dementia secured new funding, enabling the continuation and expansion of potentially life-saving treatment studies.

In a broader context, increased funding worldwide is imperative for developing and implementing treatments for the diverse 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 of the Childhood Dementia Research Group at Flinders Health and Medical Research Institute, College of Medicine and Public Health, Flinders University; Nicholas Smith, Head of the Paediatric Neurodegenerative Diseases Research Group, University of Adelaide, and Siti Mubarokah, Research Associate at the 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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