What Is Childhood Dementia?

Childhood dementia refers to progressive brain damage caused by certain rare genetic disorders. Children with childhood dementia are born with the underlying condition; it is not contagious.

Symptoms of childhood dementia may include confusion, loss of memory, or the inability to communicate. The symptoms are progressive, meaning they get worse over time. Symptoms may progress quickly or slowly depending on the cause. 

This article will provide an overview of childhood dementia, including its symptoms, causes, and treatment options.

Types of Childhood Dementia

Childhood dementia is usually related to neuronal ceroid lipofuscinoses (NCL), a group of rare, fatal genetic conditions.  

The three types of NCL are:

• Late infantile: Diagnosed in infants and includes Jansky-Bielschowsky disease
• Juvenile: Diagnosed in children and adolescents and includes Batten disease (the most common form of NCL)
• Adult: Diagnosed in adults and includes Parry disease
  

Childhood Dementia Symptoms

The symptoms of childhood dementia are caused by brain damage that worsens over time. It is common for children to lose skills that they previously developed or mastered.

Childhood dementia symptoms may include:

• Memory loss
• Confusion
• Inability to concentrate
• Personality changes
• Hyperactivity
• Inability to regulate emotions

Depending on the underlying cause, children with childhood dementia may also experience the following:

• Seizures
• Loss of vision
• Loss of hearing
• Immobility 

Causes

Childhood dementia occurs when nerve cells in the brain (neurons) lose connections to each other and start to die. A group of rare genetic conditions causes this brain damage.

NCL causes a substance called lipofuscin to build up in the brain, leading to brain damage. These diseases are genetic and caused by autosomal recessive traits. That means both parents must pass on the mutated gene for the child to contract the disease.

Diagnosis

The diseases that cause childhood dementia may be diagnosed at birth or later in childhood. If your medical team is concerned that your child is at risk for NCL, they may recommend the following diagnostic tests:

• Magnetic resonance imaging (MRI) of the brain
• Computed tomography (CT) scan of the brain
• Electroencephalogram (EEG)
• Genetic testing 
• Skin biopsy (removing a sample of skin to be sent to a lab for testing)
• Autofluorescence (a noninvasive test assessing the function of the retinal pigment epithelium, the layer of cells underneath the retina in the eye)

Treatment

No treatments exist to stop or slow dementia caused by neurodegenerative diseases. Treatment for NCL usually targets the symptoms. There is no cure for NCL genetic diseases.

Treatment options may include:

• Muscle relaxants to treat spasticity (muscle stiffness or inflexibility)
• Sleep aides to treat insomnia
• Antiseizure drugs to treat epilepsy
• Antidepressant medications to treat behavior and personality changes

Children with childhood dementia likely require lifelong care. Therapies in their treatment plan may include:

• Physical therapy
• Occupational therapy
• Speech therapy
• Mental health therapy
• Nutrition care

Prognosis 

Childhood dementia is a severe condition. It cannot be reversed, and most children require constant care and supervision. The genetic diseases that cause childhood dementia are fatal.

The younger the child is diagnosed, the worse the prognosis usually is. Children diagnosed with NCL in their first year of life are not expected to live beyond age 10. Talk with your healthcare provider about your child’s prognosis. 

Coping 

Parents and caregivers of children with childhood dementia need support in all areas of their lives. A support group may be helpful for learning about resources and connecting with others. 

Organizations that offer support groups for caregivers of children with NCL include:

• Genetic and Rare Diseases Information Center
• Batten Disease Support and Research Association

Summary 

Childhood dementia is a progressive brain disorder caused by a rare genetic disease. There is no cure for childhood dementia, and the symptoms worsen over time. Possible symptoms include memory loss, confusion, and the inability to speak.

To diagnose childhood dementia, your healthcare team will focus on diagnosing the underlying disease. Your child may require a brain imaging test to confirm the diagnosis.

There is no cure for childhood dementia. Treatment options may include medications, physical, occupational, speech, and mental health therapy, and nutrition services.