What are the different types of childhood dementia?
The most common types of childhood dementia include Rett syndrome, some mitochondrial disorders, nonketotic hyperglycinemia, vanishing white matter disease, Sanfilippo syndrome, Batten disease, MECP2 duplication syndrome, spastic paraplegia type 11 and juvenile Huntingtons disease. Altogether, there are over 100 different childhood dementia disorders. They are all progressive, fatal genetic conditions that damage the brain and nervous system.
Two thirds of children born with a childhood dementia condition have a genetic inborn error of metabolism (which means the body cannot properly process certain proteins, fatty acids or carbohydrates).
Within this group, the three most common sub-categories are:
- lysosomal diseases (affecting 22% of children born with a childhood dementia condition). There are over 44 lysosomal diseases that cause childhood dementia. These include Sanfilippo syndrome, Niemann-Pick disease types A and C and Batten disease.
- Mitochondrial disorders (affecting 20% of children born with a childhood dementia condition). This highly variable group of conditions includes Leigh syndrome and MELAS.
- Peroxisomal diseases (affecting 9.5% of children born with a childhood dementia condition) including X-linked adrenoleukodystrophy and Zellweger spectrum disorder.
There is also a large group of other conditions that don’t fit neatly within a category. This applies to 27% of children diagnosed with childhood dementia. Disorders in this group include Rett syndrome, ataxia-telangiectasia, Cockayne syndrome, HIBCH deficiency, spastic paraplegia type 11 and juvenile Huntington’s disease.
Aetiopathalogical classifications of childhood dementia conditions (% of childhood dementia births)
