Childhood dementia

What is the cause?

Childhood dementia results from progressive brain damage and is caused by over 70 rare genetic disorders including Niemann-Pick type-C, Batten disease and Sanfilippo syndrome. Broadly, there are different groups or types of childhood dementia disorders which include:

  • Inborn errors of metabolism
  • Lysosomal disorders
  • Mucopolysaccharidoses
  • Peroxisomal disease
  • Neurodegeneration with brain iron accumulation (NBIA)
  • Leukodystrophy

More information about these conditions is available at

Around one in every 2,800 babies globally are born with a condition that causes childhood dementia. An estimated 2,300 Australians are living with childhood dementia.

What are the symptoms?

Each child’s experience with dementia is unique. Signs and symptoms of childhood dementia can appear at different ages. In some instances, dementia symptoms can appear in the youngest of children and babies and progress quickly. While for other children, symptoms may not start until they are teenagers. Like dementia in adults, childhood dementia is progressive. Over months, years or decades, children progressively lose skills and abilities.

Just like adults with dementia, children with dementia experience:

  • Memory loss
  • Confusion
  • Trouble concentrating, understanding, learning and communicating
  • Personality changes
  • Severely disturbed sleep
  • Behavioural issues such as hyperactivity
  • Emotional issues like anxiety and fear

In addition to these symptoms, children can also:

  • Suffer from seizures
  • Lose their vision and hearing
  • Lose their ability to move entirely
  • Have problems with their bones, joints, or their cardiovascular, respiratory, or digestive systems

How is childhood dementia diagnosed?

Children are usually diagnosed following tests by a specialist team at a children’s hospital. If a parent is concerned their child is showing signs of dementia, it’s best to first connect with a GP who will make a referral for further investigation. 

A diagnosis is found either through biochemical testing or genetic testing. Biochemical testing can be done using blood or urine samples and essentially involves looking for unusual levels of substances in these fluids, such as certain types of proteins or sugars. Finding that a child is missing or not producing enough of an essential enzyme, for instance, is something that can be detected in this way. Biochemical testing in many cases can either give a diagnosis, or help doctors narrow down to a group of diseases. Genetic testing is then used to confirm a diagnosis and can give more detailed information on specific changes to genes. Alternatively, sometimes whole genome sequencing is used to scan all of a child’s genes for changes that are causing their condition. 

Further information

For more information about childhood dementia, go to