Childhood dementia
Childhood dementia results from progressive brain damage and is caused by over 100 rare genetic disorders (Elvidge et al. 2023). Collectively, childhood dementia has an estimated incidence rate of 34.5 per 100,000 births (1 in 2,900 births) and an expected prevalence rate of 5.3 per 100,000 persons in high and upper-middle income countries (Elvidge et al. 2023).
For more information, visit the Childhood Dementia Initiative.
National Disability Insurance Scheme
The National Disability Insurance Scheme (NDIS) is an Australian government program that provides funding for people under the age of 65 with a permanent and significant disability. Through this scheme children with developmental delay or disability, and their families may be provided funding to access a range of specialist support services that promote child development, family and child wellbeing and community involvement. Children diagnosed with dementia who are under the age of 65 may be eligible for support from the NDIS, but not all children with childhood dementia access support from the NDIS. These data should therefore be interpreted with caution as the data are based on services that reach a limited number of people.
This report includes the number of approved NDIS plans for people with Rett Syndrome and Juvenile Huntington’s disease. People with Juvenile Huntington’s disease includes those whose age at first NDIS plan approval was under 20, and whose primary disability was classified as Huntington’s disease according to ICD coding.
The NDIS data are updated quarterly. In this report, the number of approved plans for people with Rett Syndrome and Juvenile Huntington’s disease are reported as at 31 December 2024 (using data from the December 2024 quarter: 1 October to 31 December 2024) compared to 31 March 2023 (using data from the March 2023 quarter: 1 January to 31 March 2023).
These data come from AIHW analysis of NDIS data in the Person Level Integrated Data Asset (PLIDA) in the Australian Bureau of Statistics’ DataLab. Refer to Technical notes for further information on the NDIS and classification of childhood dementia.
Childhood dementia mortality
In consultation with an expert from the Childhood Dementia Initiative, mortality statistics of available childhood dementia specific ICD-10 codes were extracted from the National Mortality Database. It is important to note that these data do not capture all deaths with childhood dementia, for the following reasons:
- some childhood dementia conditions do not have a specific ICD-10 code
- some childhood dementia conditions are grouped under an ICD-10 code with other conditions that do not cause childhood dementia
- under-reporting of childhood dementia conditions on death certificates.
Therefore, the findings will be an underestimate of the true mortality counts.
Refer to Deaths due to dementia for a broader discussion on how the National Mortality Database is used to report on dementia deaths and what is being done to better understand dementia typing in deaths data.
Some childhood dementia conditions are grouped with multiple diseases that are not specific to younger people so analysis for those certain codes was restricted to records where people were aged less than 30 when they died (see below). For codes that only included childhood dementia diseases, there was no age limitation on the analysis.
ICD-10 codes, childhood dementia condition and age group for analysis
No age limit (age at death):
- E75.4: Neuronal ceroid lipofuscionosis (incl. Batten disease)
- Q87.83: Zellweger syndrome
- E72.5: Disorders of glycine metabolism (incl. Nonketotic hyperglycinemia)
- E75.1: GM1 gangliosidosis
- E75.0: GM2 gangliosidosis
- G11.3: Cerebellar ataxia with defective DNA repair, Ataxia telangiectasia [Louis-Bar]
- E76.0: Hurler syndrome
- E76.1: Hunter syndrome
- E77.0: Mucolipidosis type II
- F84.2: Rett syndrome
- E77.1: Defects in glycoprotein degradation
- G23.0: Pantothenate kinase-associated neurodegeneration (PKAN)
- E75.5: Cerebrotendinous xanthomatosis.
Age limit 0-29 years old (age at death):
- G10: Juvenile Huntington’s disease
- G20: Juvenile Parkinson's
- E75.2: Other sphingolipidosis disorders (incl. Niemann-Pick disease and metachromatic leukodystrophy)
- G31.8: Other specified degenerative diseases of the nervous system (incl. Leigh’s disease)
- E83.0: Disorders of Copper Metabolism (Menkes disease).
People with childhood dementia accessing NDIS supports
As at 31 December 2024, there were just under 1,300 approved National Disability Insurance Scheme (NDIS) plans for people living with Rett syndrome. The number of plans has gradually increased each quarter since 31 March 2023, with nearly 200 additional plans approved over that period. As at 31 December 2024, around 97% of NDIS plans for people living with Rett syndrome were female, and 33% were aged <20 years old.
As at 31 December 2024, there were nearly 80 approved NDIS plans for people living with Juvenile Huntington’s disease (where the person was aged 20 or younger when their first plan was approved). Around 57% of these plans were for males and 49% were for people aged <20 years old. The number of approved plans remained stable for all quarters of 2023 and 2024.
Deaths from childhood dementia
The number of deaths with a childhood dementia ICD-10 code recorded on the death certificate between 2014 and 2023 is presented in Table 14.1 (or data table S14.5).
The condition with the largest number of deaths over this 10-year period was Other sphingolipidosis disorders, including Niemann-Pick disease, followed by Rett syndrome (restricted to records where people were aged less than 30 when they died).
ICD-10 Code | Childhood dementia disease name | Number of deaths between 2014-2023 |
|---|---|---|
E75.2 | Other sphingolipidosis, including Niemann-Pick | 89 |
F84.2 | Rett Syndrome | 84 |
G31.8 | Other specified degenerative diseases of nervous system (Including Leigh's) | 48 |
E75.4 | Neuronal ceroid lipofuscinoses (Batten disease) | 35 |
E75.0 | GM2 gangliosidosis (Sandhoff disease/Tay Sachs disease & AB) | 23 |
G23.0 | Pantothenate kinase-associated neurodegeneration (PKAN) | 17 |
G11.3 | Cerebellar ataxia with defective DNA repair | 16 |
G10 | Juvenile Huntington's disease | 15 |
E83.0 | Menkes disease | 12 |
E76.1 | MPS II (Hunter syndrome) | 11 |
E77.1 | Defects in glycoprotein degradation | 10 |
E75.1 | GM1 gangliosidosis (type 1 and 2)/Mucolipidosis type IV | 9 |
E72.5 | Glycine Encephalopathy | 8 |
E74.4 | Disorders of pyruvate metabolism and gluconeogenesis | 7 |
E76.0 | MPS I (Hurler syndrome) | 7 |
E77.0 | Defects in post translational modification of lysosomal enzymes | 5 |
E75.5 | Cerebrotendinous cholesterosis | 3 |
Notes:
- This analysis is based on the underlying cause of death (UCOD) and associated causes of death (ACOD).
- There was no age cutoff, except for 'Other sphingolipidosis, including Niemann-Pick' (E75.2), 'Other specified degenerative diseases of nervous system (Including Leigh's)' (G31.8), 'Juvenile Huntington's Disease' (G10), 'Juvenile Parkinson's Disease (G20) and 'Menkes disease' (E83.0) where the age cutoff was <30.
- Childhood dementia types with a number of deaths less than 3 are suppressed for confidentiality reasons.
- Due to coding considerations, not all childhood dementia diseases are included in the analysis. Some childhood dementia diseases do not have ICD-10 codes, some are grouped with other diseases in one code, and some may not be coded due to errors or lack of understanding of rarer diseases amongst those completing death certificates. Results should be interpreted with caution.
Source: AIHW analysis of the National Mortality Database
Elvidge K, Christodoulou J, Farrar M, Tilden D, Maack M, Valeri M, Ellis M, Smith N, the Childhood Dementia Working Group (2023) ‘The collective burden of childhood dementia: a scoping review’, Brain, 146(11):4446-4455, doi.org/10.1093/brain/awad242