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Childhood Dementia is a Result of Progressive Brain Damage That Can be Caused by Over 70 Rare Genetic Disorders.

Childhood dementia is a neurological disorder that affects the metabolism of the brain. Typical symptoms include retinal degeneration, epileptic seizures, visual loss, deafness, cognitive deterioration, impaired motor functions, and an increased risk of death. Do you know what Childhood Dementia is?

Dementia in childhood. It is caused by over 70 rare genetic disorders and is the result of progressive brain damage. It is estimated that 700,000 children suffer from some form of childhood dementia. Each year, 48,300 children die as a result of childhood dementia.

What is Childhood Dementia?

Childhood dementia is a rare genetic disease caused by complex neurodegenerative and metabolic disorders. Because of its rarity, there is a lack of information and awareness about this potentially fatal disorder, frequently leading to late or incorrect diagnosis and delayed treatment initiation.

Furthermore, early diagnosis of childhood dementia is difficult because the initial symptoms are not very specific and are often confused with other neurological disorders. Because dementia-related brain damage is irreversible, delaying diagnosis is especially dangerous for children and frequently results in premature death. (Source: Medical News)

What Causes Childhood Dementia?

Neuronal Ceroid Lipofuscinoses (NCLs) are a group of genetic, neurodegenerative, lysosomal storage disorders inherited in an autosomal recessive fashion. NCLs are the leading cause of childhood dementia worldwide, with a global prevalence of 7-8 per 100,000 births.

NCLs are distinguished by severe central nervous system neurodegeneration and the accumulation of lipofuscin-like autofluorescent storage material in neurons, microglia, macroglia, and other cell types. The classification of different types of NCLs is primarily determined by the affected or disease-causing gene and the age at which the disease manifests itself. To date, 14 genes have been identified as being responsible for NCLs. (Source: Medical News)

Other Causes of Childhood Dementia

Aside from NCLs, other lysosomal storage diseases that cause childhood dementia include Niemann-Pick type C (NPC) and mucopolysaccharidoses. NPC is yet another inherited neurodegenerative disorder that can affect children at any age, from infancy to adolescence.

A genetic mutation in the NPC1 or NPC2 genes primarily causes this disease. It occurs due to impaired cholesterol and lipid metabolism in the body, resulting in fatty substance accumulation in the brain, liver, spleen, and lungs. The primary symptom of NPC is dementia, which usually manifests in school-aged children.

Poor concentration, lack of insight, poor judgment, impaired short-term memory formation, and learning difficulties are the most common dementia-related symptoms of NPC. All of these symptoms, when combined, result in severe cognitive and motor disabilities as well as premature death. (Source: Medical News

Other Diseases Linked to Childhood Dementia

Mucopolysaccharidosis is another inherited disease characterized by an inability to break down long-chain sugar molecules such as mucopolysaccharides. This causes abnormal sugar accumulation in cells, blood, and connective tissue, resulting in impaired motor functioning, progressive cognitive decline, and multiple organ failure. 

Even though mucopolysaccharidosis is a leading cause of childhood dementia, primary research and clinical studies on the relationship between mucopolysaccharidosis and childhood dementia are lacking.

Childhood dementia is also linked to leukodystrophies, a group of inherited metabolic disorders affecting the brain, spinal cord, and peripheral nervous system. These disorders are associated with abnormal myelin sheath (nerve covering) development or destruction in the central nervous system, resulting in progressive loss of motor control and cognitive function/development, ataxia, seizures, and premature death. Because cognitive function declines gradually in leukodystrophies, dementia is not an early symptom. (Source: Medical News

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