Understanding childhood dementia
Here you'll find education resources and information about childhood dementia specifically for health and care professionals. They’re designed to give you a broad understanding of childhood dementia.
What is childhood dementia?
The term childhood dementia is an umbrella term, representing a group of genetic conditions which cause neurocognitive decline. You can see the list of conditions known to cause dementia here.
Developmental trajectories of individuals with typical development, intellectual disability and childhood dementia.
The trajectory of childhood dementia classically proceeds in line with normal development for a long or short interval then decelerates and ultimately regresses (adapted from Haugen et al., 2019).
Symptoms of childhood dementia
The symptomatology of childhood dementia disorders is highly variable with considerable clinical and phenotypic heterogeneity. Childhood dementia does, however share many similarities with the hallmark features of adult-onset dementias, including:
- Decline in cognitive ability
- Problems with attention and concentration
- Memory loss and learning difficulties
- Problems with thinking and reasoning
- Confusion and disorientation
- Uncooperative and disruptive behaviour
- Wandering and restlessness
- Emotional disturbance (anxiety, fear, panic attacks, etc)
- Personality and behavioural changes (aggression, irritability, hyperactivity, etc)
- Sleep disturbance (often severe)
- Deterioration of social skills and socially appropriate behaviour
- Psychotic symptoms and hallucinations
- Loss of speech
- Incontinence
In contrast to most adult-onset dementias, however, and in addition to these cognitive, neuropsychological and behavioural manifestations, childhood dementia disorders are commonly associated with seizures, sensory decline (vision and hearing), movement disorders including ataxia, spasticity, dyskinesia, dystonia, gait disturbances, muscle weakness and abnormal muscle tone, and progressive neuromotor decline. Some childhood dementia disorders also involve other organs and physiological systems in addition to the central nervous system, including, peripheral nerve disease, visceromegaly (enlargement of abdominal organs), liver disease, growth retardation, gastrointestinal disease, bone and joint anomalies, and cardiac involvement.
Three paediatric neurologists describe the symptoms of childhood dementia.
This video features Professor Michelle Farrar, Dr Nicholas Smith and Dr Alex Johnson.
Research on childhood dementias revealed that symptoms extend beyond cognitive impairment, affecting behaviour, sleep, motor functions, and eating. Additionally, organ systems outside the brain, such as the liver and kidneys, are often involved. Early symptoms are followed by a decline in skills, leading to a reliant end-stage where children may require feeding tubes, seizure medication, and assistance with mobility. Some children experience premature death, while others present with neurological issues in their teenage years. The spectrum of these diseases encompasses both rapid and slow progression.
Childhood dementia facts and statistics
1 in every 2,900 babies
is born with a condition that causes childhood dementia
CHILDHOOD DEMENTIA RESULTS FROM PROGRESSIVE BRAIN DAMAGE CAUSED BY 100+ GENETIC CONDITIONS
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
HALF OF ALL CHILDREN WITH DEMENTIA DIE BY THE AGE OF 10.
Childhood dementia is progressive.
Children lose their ability to talk, walk, read, write and play, and eventually the body loses its ability to function.
Symptoms can vary and progress over months, years or even decades, impacting both the child and their family.
Estimated in Australia every year:
91 deaths in people with childhood dementia
92 deaths from childhood cancer (0-14 yrs)
Estimated in the USA every year:
1,077 deaths in people with childhood dementia
1,050 deaths from childhood cancer (0-14 yrs)
Estimated in the UK every year:
204 deaths in people with childhood dementia
260 deaths from childhood cancer (0-14 yrs)
Statistics from: Elvidge KL, Christodoulou J, Farrar MA, Tilden D, Maack M, Valeri M, Ellis M, Smith NJC; Childhood Dementia Working Group. The collective burden of childhood dementia: a scoping review. Brain. 2023 Jul 20:awad242. doi: 10.1093/brain/awad242. PMID: 37471493. https://doi.org/10.1093/brain/awad242
Resources
What is childhood dementia?
This 3-minute explainer video provides and overview of the causes, symptoms, impacts, and prevalence of childhood dementia.
An introduction to childhood dementia
This 35-minute presentation provides an overview of childhood dementia including prevalence, incidence, symptoms, and mortality.
You can download the presentation from the video below, along with a facilitator's guide to help you deliver a DIY information session for your team.
Childhood Dementia Knowledgebase
This free database contains essential information about the conditions that cause childhood dementia, gathered from the literature with expert input.
Childhood dementia disorders
View the list of over 100 rare neurodegenerative genetic disorders that have been identified as causing childhood dementia.
The collective burden of childhood dementia
This review provides information on the conditions that cause childhood dementia, how common they are and their characteristics.
References
Elvidge KL, Christodoulou J, Farrar MA, Tilden D, Maack M, Valeri M, Ellis M, Smith NJC; Childhood Dementia Working Group. The collective burden of childhood dementia: a scoping review. Brain. 2023 Jul 20:awad242. doi: 10.1093/brain/awad242. Epub ahead of print. PMID: 37471493. https://doi.org/10.1093/brain/awad242
Haugen PK, von Tetzchner S, Oxley JD, Elmerskog B. Dementia in Adulthood and Childhood. In: von Tetzchner S, Elmerskog B, Tøssebro A-G, Rokne S, editors.Juvenile Neuronal Ceroid Lipofuscinosis, Childhood Dementia and Education: Intervention, education and learning strategies in a lifetime perspective. Norway: Snøfugl Forlag; 2019. p. 76
Nunn K, Williams K, Ouvrier R. The Australian Childhood Dementia Study. Eur Child Adolesc Psychiatry 2002; 11: 63–70.
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