Childhood dementias are a group of rare, progressive conditions involving breakdown of nerve cells in the brain. Most childhood dementias have no known cure, but clinical research and advocacy are ongoing.
“Childhood dementia” is a proposed umbrella term for a wide-ranging group of genetic brain disorders that cause progressive breakdown of nerve cells.
Dementia more often affects older adults, but in rare cases, children can be born with regressive brain disorders that cause dementia symptoms such as loss of memory, speech, or movement.
Many disorders can cause childhood dementia. In all types, children experience a sustained loss of previous developmental capabilities, followed by a steady decrease in thinking and reasoning abilities.
Even when grouped together, childhood dementias are uncommon. According to a 2023 research review, currently untreatable childhood dementias may occur in
Scientists hope that increased awareness will improve early diagnosis and bring attention to research on treatments.
The symptoms of childhood dementia can begin at any point from infancy through adolescence, depending on the disorder that is causing them. Each child who experiences one of these disorders may have unique initial symptoms and progression.
Common childhood dementia symptoms include:
- changes in behavior, personality, and sleep
- challenges related to thinking, such as difficulty concentrating or learning
- communication problems, such as speech delay, loss of speech, or difficulty understanding
- loss of vision or hearing
- difficulties with movement, such as loss of mobility, coordination, or muscle control
- challenges with feeding
- seizures
- memory loss
- confusion
- problems with specific organ systems, such as the bones, joints, heart, lungs, or liver
The hallmark of all types of childhood dementia is symptom progression, although the pace may vary. Children with these disorders will experience a progressive loss of developmental skills and functions.
The authors of a
These gene changes show up in different ways and affect a wide range of cell processes. But ultimately, they all cause the progressive brain damage characteristic of childhood dementia.
Childhood dementia disorders are present from birth, although symptoms may not appear right away.
The genetic changes that cause childhood dementia disorders are usually inherited (passed down through family generations). Occasionally, they may be sporadic, such as when a new disease-causing gene mutation arises during a baby’s development in the womb.
If your child has received a diagnosis of a childhood dementia disorder, a geneticist may be able to help you understand the risks for other members of your family.
There are
Inborn errors of metabolism
In these disorders, a child’s body has trouble breaking down, transporting, or rebuilding key cellular nutrients such as proteins, fats, and sugars. This can result in crucial deficiencies and toxin buildup. Examples include:
- Mitochondrial disorders: Mitochondria are the power-producing engines of cells. Several types of mitochondrial disease exist, all involving decreased function in the tissues that demand the most energy, such as the brain, heart, liver, and lungs.
- Urea cycle disorders: In these conditions, an inability to break down protein byproducts causes toxic ammonia levels in a child’s bloodstream.
- Disorders of organic or amino acid metabolism: Examples include phenylketonuria and Canavan disease.
- Lysosomal storage diseases: These cause abnormal buildup of fats or sugars in a child’s cells. The most common type is Gaucher disease, although Gaucher disease does not always cause childhood dementia. Other examples are Tay-Sachs disease and Sanfilippo syndrome.
- Peroxisomal diseases: In conditions such as Zellweger syndrome and X-linked adrenoleukodystrophy, a child’s cells can’t properly break down toxins and assemble crucial brain fats.
- Vitamin-responsive disorders: Examples include biotinidase deficiency and cobalamin C disease.
Other leukodystrophies
Leukodystrophies are disorders
Neurodegeneration with brain iron accumulation (NBIA)
NBIA disorders
Other childhood dementia disorders
Many childhood dementias do not fit neatly into other categories, including well-known conditions such as:
- ataxia-telangiectasia syndrome
- Rett syndrome
- juvenile Huntington’s disease
If you have a family history of childhood dementia or neurodegenerative disease, talk with a doctor when you begin planning your family.
After your baby is born, ask about their newborn blood screening results. Be sure to ask your doctor any questions you may have about feeding or development.
The Centers for Disease Control and Prevention (CDC) offers a
For families with a known history of a childhood dementia disorder, healthcare professionals might offer prenatal genetic testing.
In other cases, your child might receive a diagnosis after routine newborn blood screening.
Still, many children don’t receive a diagnosis until suggestive symptoms arise. Depending on your child’s circumstances, a doctor may recommend that you consult specialists in pediatric neurology or genetics.
A doctor might recommend:
- blood or urine tests to check organ health and look for certain biochemical abnormalities
- specific gene tests, depending on your child’s symptoms
- medical imaging, such as a brain MRI
- other specific symptom-related tests, such as an electroencephalogram to rule out seizures or an echocardiogram to assess heart condition
Effective disease-specific treatment is available for some childhood dementia disorders. Often, this treatment is most effective with early diagnosis via prenatal or newborn screening.
Childhood dementias have a wide range of causes, and treatments are also diverse, possibly including:
- dietary changes or supplementation
- enzyme replacement therapy
- gene therapy or stem cell transplantation
- small molecule drugs
For example, dietary changes and supplementation can treat urea cycle disorders, phenylketonuria, and certain acidemias.
If doctors identify biotinidase deficiency early, they may recommend high dose vitamins. You can typically manage Wilson’s disease with chelating medication.
Early identification of
But no specific treatment is available for most childhood dementia disorders. Instead, healthcare professionals focus on symptom management and family support, including:
- medications, such as those to manage seizures and help with behavioral issues
- physical, occupational, and speech therapies
- assistive devices, such as those for mobility and communication
- counseling, including foundation or community support groups
In a 2023 research review of childhood dementia in Australia, the United States, and the United Kingdom, researchers found an average life expectancy of
The availability of treatment plays a significant role in the outlook. Children who receive a prompt diagnosis of a treatable form of childhood dementia may have a nearly typical life expectancy. But the same review found that children with untreatable childhood dementia disorders had an average life expectancy of 16.3 years.
Do children grow out of childhood dementia?
No. Children are born with the genetic changes that cause childhood dementia disorders, although symptoms may not appear right away. Once symptoms begin, they generally become progressively worse.
Some childhood dementia disorders may be treatable if diagnosed in early stages. However, many of these disorders are not currently treatable.
At what age can someone receive a childhood dementia diagnosis?
The average age at symptom onset in childhood dementia disorders is
But because early symptoms can be vague or mimic those of more common diseases, children may experience a delay in diagnosis.
How rare is childhood dementia?
Specific childhood dementia disorders are all quite rare, though some are more common in certain populations.
In a 2023 review, researchers estimated that childhood dementia disorders collectively occur in
Taken together, currently untreatable childhood dementias occur in about 1 in 2,900 live births.
In the United States, experts estimate that childhood dementias are responsible for
“Childhood dementia” is a proposed term for a spectrum of rare, progressive genetic disorders that cause breakdown of nerve cells in the brain.
Childhood dementias cause children to progressively lose skills they’ve previously acquired, such as walking, talking, and remembering things. The results can be devastating for the children who experience these conditions, as well as their families and communities.
Most childhood dementias currently have no cure, although research is ongoing. For some specific childhood dementia disorders, an accurate, early diagnosis can lead to effective treatment and a typical life span.
Consult a doctor if you have a family history of childhood dementia. Monitor your child’s developmental milestones and take your child for regular checkups with a doctor. If you have any concerns about your child’s developmental progress or if your child seems to be losing capabilities, talk with their doctor right away.
If your child receives a diagnosis of a childhood dementia disorder, support is available. You can talk with your child’s healthcare team about therapy and support groups specific to their condition or contact organizations such as the Childhood Dementia Initiative for more information.