What Is Childhood Dementia?

What Is Childhood Dementia?

Introduction: Childhood Dementia

The terms childhood dementia do not usually go together and may even seem contradictory. When we think of dementia we don’t usually think of children, because dementia is associated with older people.
When you think of the deteriorating brain health condition known as dementia, you probably think of older people who suffer from memory loss and other types of cognitive impairment, such as confusion and disinhibition, as they age.
However, childhood dementia shows that dementia is not always age-related. And like dementia in adults, childhood dementia limits the patient’s quality of life.
Dementia is not something most people associate with children. For some kids and their families, however, childhood dementia is a reality. Today, approximately 700,000 children worldwide suffer from childhood dementia.
Unfortunately, many people, from health professionals and researchers to the general public, are unaware of this debilitating disease in children. As a result, it’s critical to raise awareness of childhood dementia since it can be difficult to comprehend and access supportive care if others are unaware of the condition.

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Definition of Childhood Dementia

Childhood dementia refers to progressive neurodegenerative or brain damage caused by specific rare genetic disorders. More than 250 disorders affect the cognitive abilities of children and adolescents. Since these children frequently have these underlying genetic issues from birth, childhood dementia is not communicable—rather, it is ingrained in them.
Every child’s experience with childhood dementia is different due to the variety of rare genetic abnormalities that cause it. For example, symptoms of childhood dementia may appear in young children and progress rapidly. Meanwhile, other children may experience symptoms in adolescence, and the damage will happen gradually but over months or years. Whatever the age of commencement, all of these instances share the irreparable brain damage and degenerative nature of infantile dementia. To date, there is no cure for childhood dementia.
This means that as the days go by, the patient’s brain will continue to suffer damage and cognitive decline will continue. This is heartbreaking, as family members who care for the children watch as they lose newly acquired skills, such as walking, reading, speaking, or writing. The unpleasant reality is that those suffering from infantile dementia will not have the chance to explore and acquire numerous talents.
Over time, the brain loses the ability to function properly. Unfortunately, most patients with childhood dementia will not survive to adulthood.

What Causes Childhood Dementia?

Neuronal ceroid lipofuscinosis (NCL) is the leading cause of dementia in children worldwide. The worldwide prevalence of these NCLs is 7 to 8 per 100,000 births. NCL refers to a group of genetic, neurodegenerative, and lysosomal disorders that are typically inherited following an autosomal recessive inheritance pattern.

What Does This Mean?

Recessive inheritance requires two copies of the mutated gene from the mother and the father for the disease to appear in the child. Normally, both parents do not have symptoms but are carriers of the disease. If only one parent has the recessive gene, the child will not show symptoms, but may still be a carrier. Therefore, this makes this disease very rare as the child is less likely to manifest symptoms as both parents must be carriers.
However, dementia in children is still a reason for alarm. It comes from inherited genetic defects, specifically NCL, which cause poor brain function. This means that the building blocks for the structure and maintenance of neurons and brain tissue are missing or not metabolized properly. As a result, neurons in the brain become damaged and eventually die. These NCL disorders are divided into the following types based on the onset of symptoms and other clinical manifestations:
The most prevalent forms of NCL are the late infantile and juvenile forms, with a typical life expectancy of 8 to 12 years. In most cases, children and teens with childhood dementia grow up without any notable problems in their early years. They reach developmental and age-appropriate milestones. However, once the disease appears, they continue to lose their abilities steadily and steadily until their death.
In addition to NCL, X-linked adrenoleukodystrophy (X-ALD), Alpers syndrome, and multiple sulfatase deficiency are potential causes of dementia in children. Childhood dementia is also caused by lysosomal storage diseases, which include mucopolysaccharidoses and Niemann-Pick disease type C (NPC). These are inherited neurodegenerative diseases. But these cases are rare compared to NCL.

What Are The Symptoms of Childhood Dementia?

Like older adults with dementia, children with childhood dementia may experience the following:
The onset of symptoms and how quickly they worsen usually depends on the form of neurodegenerative disease that attacks the child. Patients eventually become immobile as their reliance on others for care grows. Death within the first two decades of life is not uncommon, and many children die before their 18th birthday.

What Are The Treatment Options?

Unfortunately, there are no treatments to prevent or slow childhood dementia. Instead, medications and treatments may be given to relieve symptoms. Treatment may include the following:
Children with childhood dementia require lifelong care and supervision as they suffer from extremely debilitating symptoms that cannot be reversed. Unfortunately, the younger a child is diagnosed, the worse the potential outcomes. People who are diagnosed before their first birthday are usually not expected to live more than 10 years.
To make matters worse, early diagnosis is also difficult, as early symptoms can be attributed to other diseases. Unfortunately, delays in diagnosis can lead to greater pain and premature death in children.

Why Is There So Little Knowledge About Childhood Dementia?

Most people haven’t heard of childhood dementia, so if this is the first time you’re learning more about this rare health problem in children, you’re not alone.
Because childhood dementia is caused by rare genetic conditions, it is often overlooked. Lack of information and the rarity of this life-threatening disease can also lead to a late or incorrect diagnosis. As a result, this means a delay in getting the right treatment to relieve the patient’s symptoms, as well as a delay in supportive treatment for parents who need help coping with this life-altering diagnosis. Is.
Knowing that such conditions may be a possibility for your children when you start a family, it is important to evaluate your genetic health risks before getting pregnant. Taking a family planning DNA testing kit from CircleDNA can help you find out possible hereditary diseases and disease risks that you and your partner may pass on to your future offspring. It is important to educate yourself about this aspect of family planning. In this DNA testing kit, you will find out if you have any genetic mutations that you can pass on to your children.
Many people who have a strong family history of seriously life-threatening or debilitating diseases choose not to have biological children and adopt instead. Others who have the financial means rely on IVF and embryo screening to avoid harmful and disease-causing genetic mutations in their children. It is essential to discuss family history and diseases with your partner. Getting a DNA test for family planning purposes is a responsible form of preventive health that allows you to ensure the well-being of your future children and family members.

FAQs About Childhood Dementia

Rare brain disorder in kids causing memory loss, confusion, & learning difficulties, similar to adult dementia but genetic.
Over 70 rare genetic conditions affecting how the body processes substances, damaging brain cells.
Memory loss, confusion, learning troubles, speech problems, behavior changes, vision/hearing loss, motor skill decline (varies by cause & age).
No, but treatments manage symptoms & provide support (meds, therapy, nutrition).
Raise awareness, donate to research, support families.