Genetic Neurodegenerative Disorders
Childhood Dementia Disorders (Group of Conditions)
The information on the Rare Awareness Rare Education (RARE) Portal is intended for educational purposes only and does not replace professional advice.
Rare diseases typically display a high level of symptom complexity and variability. Individuals diagnosed with the same rare disease may be impacted differently and each person’s experience is unique. Please seek support from qualified healthcare professionals to learn more about the most suitable care and support options for you.
For further information about childhood dementia, please refer to Childhood Dementia Initiative1 and the Childhood Dementia Knowledgebase.2 Individual pages for specific childhood dementia disorders will be added to the RARE Portal in time.
If you are aware of any additional information that may benefit stakeholders with an interest in this page, or if you notice any broken links or misleading information, please let us know via the Contribute page.
This page has been co-developed with RVA Partner, Childhood Dementia Initiative.1
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Emergency Management | Clinical Care Guidelines | Synonyms | Summary | Symptoms | Cause/Inheritance | Diagnosis | Treatment | Clinical Care | Research | Rare Disease Organisation(s) | Social Services | Mental Health | Other | References
There are no collective emergency management guidelines available for childhood dementia disorders. Guidelines for individual childhood dementia disorders should be consulted where available. Similarly, each child may have guidelines regarding specific problems, like a seizure management plan or for management of movement disorders. These plans should be kept by the family and included in the medical record.
Some common considerations may include:
- The emergency department environment can be confusing and frightening for children with dementia
- Children with dementia can have an exacerbation of symptoms due to a new environment, but it can also be due to something else, such as pain or discomfort. A good general examination is vital to ensure there isnt something else occurring when there is an exacerbation
- Childhood dementia disorders are complex and affect many systems in the body in addition to the brain. Affected children will often have a ‘team’ of people caring for them, who may be specialised in one feature. The child’s specialist care team should be contacted if possible
- Children with dementia are likely to be taking multiple medications to manage their condition. Some medications are contraindicated in certain childhood dementia disorders. Expert advice should be sought before administering new medications
- Childhood dementia disorders are rare and often the parents have become the experts: their knowledge and experience should be respected
Clinical Care Guidelines
There are no clinical guidelines available for childhood dementia disorders as a collective. Clinical guidelines for individual childhood dementia disorders should be consulted where available.
Childhood dementia disorders cover a group of more than 100 different rare genetic conditions that cause progressive brain damage in children.3 Signs of childhood dementia may not always be apparent from birth, and symptoms and progression of the disease can vary. Due to the progressive nature of childhood dementia disorders, over time children will lose their skills, including their ability to write, read, talk, walk and play.1 Their brains also lose the ability to keep the body functioning properly, and eventually, to keep the body alive.1
A list of the different conditions that cause childhood dementia can be found at Childhood Dementia Initiative: Childhood dementia disorders list and the Childhood Dementia Knowledgebase.
- Childhood Dementia Knowledgebase – a relational database that provides key statistics for each of the 100+ conditions that cause childhood dementia
- Childhood Dementia Initiative: Resources for healthcare professionals
- State of Childhood Dementia 2023 – this report explores the current state of childhood dementia across policy responses, research progress and the support available for children and families
- ‘We don’t fit’: The lived experience of families affected by childhood dementia and their interactions with care and support services – developed by international management consultancy, Nous Group, the report gives insight into ‘typical’ family experiences navigating care and support services
Similar to adult-onset dementia, symptoms of childhood dementia may include memory loss, confusion, difficulties concentrating, comprehending, learning and communicating, personality changes, sleep disturbances as well as behavioural and emotional issues.1 Additionally, depending on the type of childhood dementia, children may also experience other symptoms, such as seizures, issues with bones, joints, cardiovascular, respiratory and digestive systems, and loss of vision, hearing and movement. The onset of symptoms can vary.
Childhood Dementia Knowledgebase has more information on specific symptoms and complications for a particular childhood dementia disorder.
Please speak to your medical team to learn more about the symptoms for a particular childhood dementia disorder.
Childhood Dementia can be caused by more than 100 different genetic disorders.3 These disorders are caused by pathogenic changes in at least 400 individual genes.3 The inheritance pattern may differ depending on the type of genetic disorder (please refer to the specific genetic disorder on the Childhood Dementia Knowledgebase for more information on inheritance). Some types of childhood dementia are not inherited but are caused by spontaneous changes in the DNA in early life.
If you would like to learn more about the inheritance and impact of a particular childhood dementia disorder, please ask your doctor for a referral to a genetic counsellor. Genetic counsellors are qualified allied health professionals who can provide information and support regarding genetic conditions and testing. More information on genetic counselling can be found on the National Services page and specific State and Territory Services pages on the RARE Portal.
The pathway to diagnosis varies between different childhood dementia disorders. Diagnosis may involve a combination of early clinical symptom assessment, brain imaging, detection of biochemical markers in urine and blood, and genetic testing.
Given the non-specificity of initial presenting symptoms, the rarity of the individual diseases and general lack of awareness in the medical community, the diagnosis of childhood dementia disorders is often delayed, sometimes for years after the first symptoms are noticed. Commonly, children are misdiagnosed with autism, developmental or intellectual delay, attention deficit hyperactivity disorder (ADHD) and others, before reaching a definitive diagnosis.
Please speak to your medical team to learn more about the diagnosis pathway for a particular childhood dementia disorder.
For most childhood dementia disorders, there are currently no effective treatments, and where therapies do exist, they are often limited to slowing the rate of disease progression.1
Childhood Dementia Knowledgebase has information on available treatments for some childhood dementia disorders.
It is best to speak with your medical team to learn more about any available treatment or suitable management strategies for a particular childhood dementia disorder.
Healthcare professionals involved in the treatment of childhood dementia disorders may include general practitioners (GP), paediatricians, geneticists, metabolic physicians, neurologists, and allied health professionals including occupational therapists, speech therapists, physiotherapists, dieticians, psychologists and social workers. As childhood dementia is caused by a broad range of neurodegenerative diseases, the healthcare professionals involved in the treatment of childhood dementia will vary and may extend beyond those listed here.
There may be specific clinics for some childhood dementia disorders. Information about such clinics may be available from rare disease organisations for a particular disorder.
Information for researchers can be found at Childhood Dementia Initiative: For researchers.
Please visit Australian Clinical Trials to learn about clinical trials for Childhood Dementia Disorders in Australia; there may not be any currently available.
Information regarding clinical trials for childhood dementia disorders in other countries can be found at ClinicalTrials.gov; there may not be any currently available.
It is best to discuss your interest in any clinical trials with your medical team to determine suitability and eligibility.
Rare Disease Organisation(s)
Childhood Dementia Initiative raises awareness of childhood dementia, provides information and resources for health professionals and researchers and drives systemic improvements in care, support and treatment.
Childhood Dementia Initiative’s Australian support service directory includes a list of disease-specific organisations.
Please note that RVA does not necessarily monitor or endorse each group/organisation’s operational governance.
Childhood Dementia Initiative’s Australian support services directory is a starting point for Australian families of children with dementia who are looking for access to disability, carer and palliative care services.
Please visit the National and State Services pages for other general information on social services.
Please visit the ‘Mental Health’ sections listed on the National and State Services pages.
- Childhood Dementia Initiative [Internet]. n.d. Available from: https://www.childhooddementia.org/
- Childhood Dementia Initiative Knowledgebase [Internet]. n.d. Available from: https://www.childhooddementia.org/knowledgebase
- Elvidge KL, Christodoulou J, Farrar MA, Tilden D, Maack M, Valeri M et al. The collective burden of childhood dementia: a scoping review. Brain [Internet]. 2023; awad242. Available from: https://doi.org/10.1093/brain/awad242
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