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.

In the September 2024 Dementia in Australia update, childhood dementia deaths have been reported for the first time. 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.

Childhood dementia data considerations

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.

Table 13.14 shows the number of deaths with a childhood dementia ICD-10 code recorded on the death certificate between 2013 and 2022. 

The condition with the largest number of deaths over 10 years was Rett syndrome, followed by Other sphingolipidosis disorders, including Niemann-Pick disease and metachromatic leukodystrophy (restricted to records where people were aged less than 30 when they died). 

Table 13.14: Number of deaths with a childhood dementia ICD-10 code recorded on the death certificate, by ICD-10 code, 2013–2022

ICD-10 Code

Childhood dementia disease name

Number of deaths between 2013–2022

F84.2

Rett syndrome

75

E75.2

Other sphingolipidosis, including Niemann-Pick

74

G31.8

Other specified degenerative diseases of nervous system (Including Leigh's)

40

E75.4

Neuronal ceroid lipofuscinoses (Batten disease)

33

E75.0

GM2 gangliosidosis (Sandhoff disease/ Tay Sachs disease & AB)

22

G23.0

Pantothenate kinase-associated neurodegeneration (PKAN)

18

G11.3

Cerebellar ataxia with defective DNA repair

17

G10

Juvenile Huntington's disease

15

E77.1

Defects in glycoprotein degradation

11

E83.0

Menkes disease

10

E72.5

Glycine encephalopathy

10

E76.1

MPS II (Hunter syndrome)

10

E75.1

GM1 Gangliosidosis (type 1 and 2)/Mucolipidosis type IV

10

E74.4

Disorders of pyruvate metabolism and gluconeogenesis

8

E77.0

Defects in post-translational modification of lysosomal enzymes

7

E75.5

Cerebrotendinous cholesterosis

6

E76.0

MPS I (Hurler Syndrome)

6

SeeTable S13.14 for notes.

Source: AIHW analysis of the National Mortality Database

In the December 2023 quarter, just over 1,100 people living with Rett syndrome were approved for a National Disability Insurance Scheme (NDIS) plan. This number remained stable in all quarters of 2023. Around 98% of people approved for a NDIS plan living with Rett syndrome were female, and 35% were aged <20 years old. In the same quarter, 80 people living with Juvenile Huntington’s disease (aged 20 or younger) were approved for an NDIS plan, of which approximately 58% were male. Similarly, the number of people approved remained stable in each quarter.

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