For most of us, dementia is associated with old age, not something we would expect a child to be diagnosed with. However, “Childhood dementia” does exist among young children and is currently an untreatable disease.
Just one of more than 100 rare genetic disorders can cause childhood dementia. Though the causes differ from dementia acquired later in life, the progressive nature of the illness is the same.
Unfortunately, half of infants and children diagnosed with childhood dementia will not reach their tenth birthday, and most will die before turning 18.
There has been very little awareness about this devastating condition as well as the research required for treatment and cure.
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 failure of metabolic pathways involved in the breakdown of carbohydrates, lipids, fatty acids and proteins in the body, states a PTI report published by arrangement with the Conversation, Australia.
As a result, nerve pathways fail to function, neurons (nerve cells that send messages around the body) die, leading to progressive cognitive decline.
What happens to children with childhood dementia?
Most children initially appear unaffected. After what seems to be normal development, children who are affected 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.
Why research is important?
The importance of research is underlined by the fact that death rates for Australian children with cancer nearly halved between 1997 and 2017 thanks to
development of multiple treatments.
Unfortunately, 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 funded 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 done 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, establishment of clinical trials for childhood dementia have slowed across the world in recent years.
In addition, consultation with families reveals that current care and support systems are not meeting the needs of children with dementia and their families.
Recently, however, new funding was awarded for some research projects related to childhood dementia to develop lifesaving treatments.
More broadly, increased funding is required in Australia and around the world for research to develop and translate to treatments for the broad spectrum of childhood dementia conditions.
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