What is Childhood Dementia? – Causes & Symptoms

childhood dementia

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Age is one of the biggest risk factors for Alzheimer’s type dementia, and typically most who are diagnosed, are over the age of 65. But sadly, children can also be affected by something called childhood dementia, resulting in a loss of abilities, altered behavior, and a life span that is dramatically shortened.

A mental condition that can affect children is called childhood dementia. This ailment cannot be placed in a particular illness category since it presents itself in the form of a variety of symptoms instead.

Childhood Dementia is a hereditary medical disorder that causes changes in the brain’s metabolism. These changes can be so severe that the disease can be deadly in children who are affected by it. Despite being considered an illness that affects a small percentage of the population, it is estimated that there are currently 700,000 children or young people worldwide living with dementia.

Neuronal ceroid lipofuscinosis (NCL) is the clinical name given to dementia that affects children. There are fourteen different kinds of disorders that fall under the NCL umbrella. Unfortunately, no treatment or cure is available for these fourteen illnesses.

Impairment of mental function is a hallmark symptom of neurodegenerative diseases, which include those that might cause dementia in children. They are complicated diseases requiring a high level of care and are the root cause of the low quality of life that permeates an entire family. The likelihood that the child will die before reaching adulthood is quite high.

The underlying cause of dementia in children can significantly impact how the symptoms of dementia manifest in that age group. If the diagnosis and treatment are put off for too long, it might lead to serious problems or even the premature death of affected children.

On the other hand, recognizing and addressing the symptoms at the earliest opportunity is of the utmost significance.

Read on to learn more about childhood dementia and its causes and symptoms.

Childhood Dementia

Childhood dementia is a kind of dementia that affects young people and is caused by a complicated combination of neurodegenerative and metabolic conditions. It is extremely uncommon.

Because of its rarity, there is a dearth of knowledge and understanding concerning this life-threatening condition. This absence of information and awareness frequently results in a delayed or incorrect diagnosis and a delay in the initiation of therapy.

In addition, a correct early diagnosis of dementia in children is difficult to achieve because the early symptoms are not particularly specific and are typically associated with other neurological conditions.

Moreover, because the brain damage associated with dementia cannot be reversed, a delayed diagnosis is extremely risky for children and frequently results in mortality at an earlier age.

It’s possible that you’re already acquainted with some of the signs of dementia in adults. However, children also experience the same symptoms, including disorientation, memory loss, loss of speech, and other symptoms.

The symptoms of dementia might vary greatly from kid to youngster. However, in certain cases, the first signs of dementia can occur in infants and very young children, and it can rapidly worsen from there.

Some children, however, might not exhibit any symptoms until they are teenagers. However, one obstacle is shared by all children diagnosed with dementia, which is that pediatric dementia is progressive.

They gradually lose abilities that they have previously gained, such as the capacity to communicate, read, write, walk, and play, as their brains continue to sustain more damage over a few months, a few years, or even many decades. As a result, their brains ultimately lose the capacity to keep the body alive and finally lose the capability to maintain the body working correctly.

Causes of Childhood Dementia

Causes of Childhood Dementia

The neuronal ceroid lipofuscinoses (NCLs) are a collection of hereditary, neurodegenerative, lysosomal storage diseases that are transmitted in an autosomal recessive manner.

They are the leading cause of childhood dementia. NCLs are the most prevalent cause of juvenile dementia worldwide, with a frequency of 7-8 per 100,000 births, making them the major cause of dementia in children.

NCLs are distinguished by the severe neurodegeneration that they cause in the central nervous system as well as the buildup of lipofuscin-like autofluorescent storage material in the neurons, microglia, macroglia, as well as other cell types.

The affected or disease-causing gene and the age at which symptoms first appeared are the two primary factors that determine the categorization of the many subtypes of NCL. To this day, 14 genes have been discovered as potential contributors to the development of NCLs.

NCLs are classified into the following kinds based on the age at which symptoms first appear as well as the clinical signs of the condition:

Congenital: a mutation in the CTSD gene is the condition’s root cause. Clinical signs include epileptic seizures and microcephaly, and the age of commencement is either before or around the time of birth.

Infantile (INCL): The clinical term for this condition is brought on by mutations in the PPT1 and KCTD7 genes. Seizures, vision loss, and a deterioration in cognitive and motor function are frequent symptoms. The earliest signs of a condition might show at any age between 6 months and 24 months.

Late-infantile (LINCL): It is a kind of NCL that mostly affects children between the ages of 2 and 7.5 years old. Mutations in the genes CLN2/TPP1, CLN5, CLN6, MFSD8, CLN8, CTSD, and PPT1 are to blame for this type of NCL, which often manifests in these children. Some clinical features include seizures, impairments in motor and cognition abilities, and visual loss.

Juvenile (JNCL): It is a type of NCL that is caused by mutations in the genes CLN3, PPT1, CLN2/TPP1, CLN9, and ATP13A2. The typical age range for the start of symptoms is between 4 and 10 years old, and clinical manifestations include impaired motor and cognitive abilities, as well as eyesight loss and seizures.

Adult types: Kufs disease and Parry disease are two prominent types of disease that manifest in adults between the ages of 15 and 50.

The most common types of NCLs are the juvenile (CLN3 illness) and late infantile varieties (CLN2 disease). For children afflicted with classical CLN2 illness, the expected lifespan ranges from 8 to 12 years.

Childhood dementia can be caused by a number of other lysosomal storage disorders in addition to NCLs. These include mucopolysaccharidoses and Niemann-Pick disease type C (NPC). The neurodegenerative condition known as NPC may strike children at any age, ranging from early childhood to puberty.

The illness is mostly caused by a mutation in the NPC1 or NPC2 genes. As a result of the body’s inability to properly process cholesterol and lipids, fatty substances accumulate in the brain, lung, spleen, and liver. Dementia is the most common symptom of NPC and frequently manifests itself in school-age children.

NPC patients frequently have dementia-related symptoms, the most prominent of which are difficulty concentrating, a lack of insight, poor judgment, impaired formation of short-term memory, and learning difficulties. When taken together, all of these symptoms result in cognitive and motor deficits and untimely mortality.

Another hereditary condition, mucopolysaccharidosis, is characterized by an inefficient disintegration of long-chain sugar molecules like mucopolysaccharides. This condition is also inherited. This leads to an aberrant buildup of sugar in the cells, blood, and connective tissue, resulting in reduced motor activity, gradual cognitive decline, and failure of many organs.

Although mucopolysaccharidosis is one of the most common causes of dementia in children, there has been a dearth of study on both the fundamental and clinical levels about the connection between the two conditions.

Leukodystrophies are a category of hereditary metabolic illnesses that can harm the brain, spine, and peripheral nervous system. Childhood dementia and leukodystrophies have been linked together before. These conditions are linked to the improper growth or destruction of myelin sheath in the central nervous system.

This leads to the gradual loss of motor control, cognitive function and development, ataxia, seizures, and premature death. Leukodystrophies are characterized by a slow decline in cognitive function over time; hence, dementia is not typically an early sign of these conditions.

Symptoms of Childhood Dementia

Symptoms of Childhood Dementia

The symptom that manifests itself is determined by the underlying etiology of dementia. Some of the early warning signs of dementia include symptoms such as poor communication skills and forgetfulness. As the dementia progresses, the child will have difficulty recognizing their family.

The following are some of the more prevalent symptoms:

1. Memory Loss

Forgetting certain things is a perfectly normal part of childhood for all children. On the other hand, if they forgets things on a consistent basis, especially significant events in their lives, this might be a sign of dementia.

However, memory loss is just one of the signs of dementia, and the fact that the kid is exhibiting this symptom does not prove that they are suffering from dementia. Before diagnosing dementia in a kid, medical professionals look for at least two abnormalities in the child’s cognitive functioning.

2. Challenges Inherent in The Language

Dementia causes cognitive decline in children, which makes it harder for them to understand language. As a result, it is more difficult for them to ingest and comprehend a number of different words and sentences.

In general, they have a more difficult time expressing themselves and understanding language, both of which are typically easy tasks for youngsters their age. Children that are affected with this condition struggle with both vocal and nonverbal forms of communication. On the other hand, this symptom is not present in every child who is impacted by the condition.

3. Reduction in Intellectual Skills

Children dealing with dementia have difficulty understanding and figuring out solutions to problems. They struggle both with forming a thought and with creatively thinking up new ideas. The severity of these symptoms tends to worsen over time, making it increasingly difficult for affected child to comprehend and commit to memory newly learned information.

4. Alterations in One’s Personality

Children who are living with dementia often display behavior that is unpredictable and constantly shifting. Examples of such shifts in personality and conduct include the following:

  1. The child frequently loses control of himself and experiences frequent shifts in mood.
  2. shows agitation, and is prone to irrational outbursts of rage and anxiety
  3. Frequently fails to remember names and faces, as well as the locations of places that are frequented.
  4. Manifests excessive behavior such as howling.
  5. Cries about insignificant matters.
  6. unable to maintain adequate levels of personal hygiene
  7. Is disorganized and unable due to a lack of ability
  8. Is anxious, afraid, and furious

Children may also have the following symptoms in addition to these:

  1. Suffer from seizures.
  2. They will lose both their sight and their hearing.
  3. They completely lose their capacity to move about.
  4. Have issues with their bones, joints, or any of their bodily systems, including their cardiovascular, digestive, and respiratory systems.


Diagnostic procedures are often carried out on children by a specialized team at a hospital that specializes in treating children. It is advisable for a parent who is concerned that their kid may be displaying indications of dementia to first seek an appointment with a primary care physician, who will then refer the child for further study.

Testing either biochemical markers or genetic markers can lead to the discovery of a diagnosis. Samples of blood or urine can be used in biochemical testing, and the process generally entails looking for unusually high concentrations of chemicals in the fluids being tested.

These compounds might include certain kinds of proteins or carbohydrates. Using this method, one may, for example, determine whether or not a child is making an adequate amount of a vital enzyme, which is something that can be discovered using this method.

The results of biochemical testing can, in many instances, either provide a diagnosis or assist medical professionals in identifying a cluster of disorders. The results of genetic testing are then used to verify a diagnosis and can provide further information on the precise alterations that have occurred in genes.

Alternatively, whole genome sequencing is utilized on occasion in order to search all of a child’s genes for alterations that might be the root cause of their disease.

Closing Thoughts

The neuronal ceroid lipofuscinoses (NCL) are a group of incurable lysosomal storage disorders that are associated with the buildup of peculiar intracellular storage material and gradual clinical deterioration. These symptoms are typically accompanied by visual loss, epilepsy, and motor decline. Children and young people most commonly develop dementia from neuronal ceroid lipofuscinoses.

The symptoms of dementia might vary greatly from child to child. There is no set age at which the signs and symptoms of infantile dementia first manifest. In certain cases, the first signs of dementia can occur in infants and very young children, and the condition can rapidly worsen from there.

However, some children may not exhibit any signs until they reach their late teens or early twenties. Like dementia in adults, infantile dementia is progressive. Children experience a gradual decline in their skills and capabilities over the course of several months, years, or even decades.

The research that has been done into treating or even just reducing the progression of dementia in children has been relatively limited due to a lack of funding. The majority of children pass away before they turn 18 years old. Our efforts to increase awareness and carry out more research will result in a better prognosis and quality of life for children who have dementia.


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