A Tasmanian researcher is at the forefront of research into understanding the genetic changes that cause childhood dementia.

Tony Cook works out of the Wicking Dementia Research and Education Centre at the University of Tasmania.

"Childhood dementia, much like adult-onset dementia, is a loss of previously acquired skills," Mr Cook said.

"However, the cause childhood dementia is purely genetic, and is frequently the result of having pathological gene variants in each copy of a key gene, where one variant copy is inherited from each parent.

"This is different to diseases like Alzheimer's disease where although genes can contribute to disease, there can also be a large contribution from lifestyle and environmental risk factors such as smoking, hearing loss, or cardiometabolic factors.

"What childhood dementia and adult-onset dementia have in common is the decline in previously acquired skills including in cognitive ability, amongst other features like memory loss, that are brought about through the degeneration of brain cells."

Mr Cook said many Tasmanian children were living with dementia but the exact number was difficult to determine to a range of factors, such as a general lack of awareness of the condition.

"One of the most exciting therapies has been a drug called Brineura.

"This has been shown to alter the disease course in children with one form of Batten disease which causes childhood dementia.

"However, this is not a cure, and there is still a lot we need to learn about this drug and when in the disease course it is most appropriate to use."

Mr Cook's research was focused on understanding the condition more.

"We have several research projects focused on understanding how the genetic changes that cause childhood dementia lead to accumulation of fats and proteins in brain cells that affect the normal functions of nerves, causing them to degenerate," he said.

"In related projects, we are conducting pre-clinical research to test potential new therapies.

"For example, one current project funded by the Medical Research Future Fund seeks to test a new way of reducing the accumulation of specific types of fats in brain cells; this work is a collaboration with Murdoch University in Perth, Western Australia.

"If our pre-clinical work shows promise, we will work with childhood dementia advocacy groups to plan a clinical trial."

Mr Cook said the condition transformed the lives of children affected by it.

"Childhood dementia affects the child's ability to learn, play and recognise family and friends. Most of these result in childhood death and do not have disease-modifying treatment options.

"The burden of childhood dementia is largely hidden, and much of it is borne by parents and siblings."

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