Dementia Occurs in Children
The popular perception of dementia is that it is strictly “an old person’s disease,” with the picture in our mind narrowing its focus to that of a confused senior citizen with Alzheimer’s disease, struggling to get through a life that was once much simpler, and easier to understand.
It’s an image that few people want to face for themselves, or our parents, grandparents, and other family members and friends.
But imagine this illness, with its memory loss, confusion, and inability to perform common daily functions, in a child.
Yes, a child.
Before one can imagine this horrible possibility, there are 5 main types of dementia:
· Cortical Dementia
· Subcortical Dementia
· Progressive Dementia
· Primary Dementia
· Secondary Dementia.
In the first four types, dementia is the primary disease.
With secondary dementia, there is another disease present and dementia is one of the symptoms or possible symptoms.
Dementia in children can be considered as secondary dementia in which there is a condition that leads to dementia-like symptoms.
Dementia is a neurodegenerative disorder in which there is a decline in mental and cognitive abilities.
This means that the patient had previously learned skills and abilities, but begins to lose those skills and abilities with the onset of a disease that carries dementia-like symptoms.
Some childhood diseases strike so early that there is no regression of learned abilities, but rather, conditions are present that prevent them from progressing in mental functioning and mobility.
Many types of childhood diseases and disorders can lead to dementia and we examine some of the more frequently occurring below.
Niemann-Pick Disease:
Niemann-Pick disease is an inherited disorder in children in which the metabolism malfunctions to the point that cholesterol and lipids can’t be metabolized, leading to a build-up in the liver, spleen, and brain.
Several symptoms develop after these organs are affected including
· confusion
· slurred speech
· learning problems
· memory loss
· dysphagia
· dementia
Type A of Niemann-Pick disease is always fatal, while the prognosis for those with type B is often good. Survival rates for types C and D are mixed.
Lafora Body Disease is a rare, inherited genetic disorder in which Lafora bodies (microscopic elements) are present in the brain, liver spleen, and muscle tissues of children between the ages of 6 to 19.
This often leads to seizures, immobility, and dementia. Death usually occurs within 10 years.
Batten Disease is an inherited and rare neurodegenerative disorder that develops in childhood and is, unfortunately, always fatal.
Symptoms can appear between the ages of 4 and 10 and manifest themselves with dementia-like symptoms of behavioral changes, difficulty in school and learning, clumsiness, repetitive speech, mental impairment, blindness, and immobility.
Mitochondrial myopathy is a mitochondrial disease that affects the muscle fibers of patients, leading to cognitive impairment and dementia as its major symptoms.
Other symptoms include deafness, blindness, droopy eyelids, immobility of the eyes, seizures, and vomiting.
Most cases of mitochondrial myopathy begin before the age of 20 and become evident during exercise with muscle weakness, nausea, breathlessness, and headaches.
This disorder can lead to death, but not in all cases.
Rasmussen’s encephalitis is a rare disease in which inflammation occurs in one hemisphere of the brain.
The symptoms are similar to the diseases mentioned above in that the young patient, (usually under the age of 10), will suffer from seizures, impaired mobility, speech problems, paralysis (on one side of the body), and mental and cognitive deterioration.
Although it is not fatal, the effects of this disease are usually life-long.
Recent thinking on Rasmussen’s encephalitis is that it is an autoimmune disease.
Surgery to control the seizures may also be a possible treatment approach.
Sanfilippo syndrome is an inherited disease in which the metabolism is unable to break down certain sugar molecules.
Sanfilippo syndrome belongs to a group of diseases called mucopolysaccharidoses or MPS.
There are 4 types of MPS of which Sanfilippo syndrome is an MPS III type.
Further, there are 4 types of Sanfilippo syndrome, Types A, B, C, and D.
Incidence rates place MPS III at one in every 70,000 births and symptoms often appear in the first year, with a decline in learning ability between the ages of 2 and 6.
Behavioral problems, delayed development, mental retardation, blindness, seizures, and shortened height are common symptoms.
Juvenile Huntington’s disease is the young people’s version of a mostly adult disease.
Approximately six percent of all Huntington’s disease cases begin in children and adolescents below the age of 21. Cognitive impairment might only occur in some patients.
Other types of neurodegenerative disorders that can lead to dementia-like symptoms in children include Alexander disease, Schilder’s disease, Tay Sach’s disease, Canavan disease, juvenile Huntington’s disease, Rett syndrome, and Adrenoleukodystrophy.
Alexander Disease
Alexander disease is a type of leukodystrophy characterized by the destruction of the myelin sheath (the fatty covering that acts as an insulator around nerve fiber) and abnormal protein deposits known as Rosenthal fibers.
Symptoms of the infantile form include an enlarged brain and head, seizures, stiffness in the arms and/or legs, intellectual disability, and delayed physical development.
Common problems in juvenile and adult forms of Alexander’s disease include speech abnormalities, swallowing difficulties, and poor coordination.
Schilder’s Disease
Schilder’s disease is a rare condition that usually starts in childhood. It’s most common in boys between 7 and 12 years old.
It’s a problem with the body’s myelin.
Myelin is a protective layer that covers most of the nerves in your body. It’s like the coating on electric wires.
It helps signals move faster around your body, the way electricity flows from a power source. When the myelin is damaged, signals can’t move the way they should.
Schilder’s disease is thought to be a form of multiple sclerosis. With MS, the immune system attacks the myelin, hurting it and the nerves it protects.
Tay Sach’s Disease
Tay-Sachs disease is a rare genetic disorder passed from parent to child. It’s caused by the absence of an enzyme that helps break down fatty substances.
These fatty substances, called gangliosides, build up to toxic levels in the brain and spinal cord and affect the function of the nerve cells.
In the most common and severe form of Tay-Sachs disease, signs and symptoms start to show up at about 3 to 6 months of age.
As the disease progresses, development slows and muscles begin to weaken.
Over time, this leads to seizures, vision and hearing loss, paralysis, and other major issues.
Children with this form of Tay-Sachs disease typically live only a few years.
Canavan Disease
Canavan disease is a progressive, fatal, genetic disorder affecting the central nervous system, muscles, and eyes.
Early symptoms in infancy may include increased head size, weakness, low muscle tone, and loss of head control.
Symptoms progress to seizures, blindness, inability to move voluntarily, and difficulty eating solids or swallowing liquids.
This condition is caused by changes in the ASPA gene and is inherited in an autosomal recessive pattern.
Canavan disease is diagnosed based on symptoms, laboratory testing, and genetic testing.
Juvenile Huntington’s Disease
Juvenile Huntington’s disease (HD) is a less common, early-onset form of Huntington’s disease that begins in childhood or adolescence.
It is a progressive disorder that causes the breakdown of brain cells in certain areas of the brain.
This results in uncontrolled movements, loss of intellectual abilities, and emotional disturbances.
Juvenile HD is defined by the onset of symptoms before the age of 20 years
Rett Syndrome
Rett syndrome is a rare genetic neurological and developmental disorder that affects the way the brain develops.
This disorder causes a progressive loss of motor skills and language. Rett syndrome primarily affects females.
Most babies with Rett syndrome seem to develop as expected for the first six months of life. These babies then lose skills they previously had — such as the ability to crawl, walk, communicate, or use their hands.
Over time, children with Rett syndrome have increasing problems with the use of muscles that control movement, coordination, and communication.
Rett syndrome can also cause seizures and intellectual disabilities. Unusual hand movements, such as repetitive rubbing or clapping, replace purposeful hand use.
Although there’s no cure for Rett syndrome, potential treatments are being studied.
Current treatment focuses on improving movement and communication, treating seizures, and providing care and support for children and adults with Rett syndrome and their families.
Adrenoleukodystrophy
Adrenoleukodystrophy (ALD) is a genetic condition that damages the membrane (myelin sheath) that covers nerve cells in the brain and spinal cord. Myelin acts as insulation around the nerve fibers.
When this insulating layer is damaged, nerve signals from the brain cannot communicate across the body properly, causing impaired bodily functions or paralysis.
ALD prevents the body from breaking down very long-chain fatty acids, causing these fatty acid chains to build up in the brain, nervous system, and adrenal gland. The accumulation is thought to cause inflammation in the body, damaging the myelin sheath.
There doesn’t appear to be a support organization for the umbrella concept of dementia in children, but there are specific associations and support groups for most of the diseases mentioned. These can be found online.
