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What is childhood dementia and how is it treated? Niemann-Pick disease and its types

It has been determined that Niemann-pick, also known as childhood dementia, is spreading rapidly in the world. Millions began researching the childhood disease known to be a genetic disease. What is childhood dementia and how is it treated? What are Niemann-Pick disease and its types? In this news, we have included details about the life-threatening Nieman-pick disease.

Niemann-Pick disease arising in certain tissues; reticuloendothelial
in cells, from the group of congenital lipidosis, in which sphingolipids accumulate
It is an autosomal recessive disease. So it’s genetic. The disease (inherited gene), popularly known as childhood dementia, is seen in 4 different basic types according to the types of signs and symptoms. These are type A, type B, type C1 and type C2. Hepatosplenomegaly Type A is the most severe type, beginning in early childhood. Childhood dementia disease, which was found to be spreading rapidly around the world recently, was also found in Turkey. Experts warned about the fatal childhood dementia and provided guidelines for early detection and treatment.

childhood dementia


The most severe form of Niemann-Pick disease that begins in early childhood. It was determined that hepatosplenomegaly Type A is generally seen in Jewish families.

What is childhood dementia


Niemann-Pick disease is an inherited metabolic disease. This affects many body systems. Fatty substances called lipids are distributed in harmful amounts to the spleen, liver, lungs, bone marrow and brain. Therefore, lack of muscle activities, brain degeneration, learning disorder, loss of muscle tone, increased sensitivity to contact, spasticity (the condition where certain muscles become overactive and cause muscle stiffness, stiffness or spasms), feeding and swallowing difficulties, speech disorder, spleen and symptoms such as liver enlargement occur. The disease is transmitted as autosomal recessive (functional changes occurring in both gene pairs at the same locus).


It is caused by genetic, metabolic, infectious, toxic or degenerative factors. These can be explained as:

  • Genetic disorders:Genetic disorders such as Down syndrome, Fragile X syndrome, Rett syndrome can be the cause of childhood dementia.
  • Metabolic disorders:Metabolic diseases such as phenylketonuria and organic acidemia can cause childhood dementia by affecting brain functions.
  • Infections:Brain infections, particularly viral infections (eg, encephalitis), can sometimes lead to childhood dementia.
  • Toxic effects:Certain toxins, excessive alcohol consumption, lead poisoning, or certain medications can cause childhood dementia.

What are the symptoms of Niemann-pick disease


TYPE A: Weakness, larger-than-normal liver and spleen, swollen lymph nodes, deep brain damage is observed. At the same time, children with this disease have an eye abnormality called ‘cherry red spot’, which can be detected by eye examination.

Children with the most dangerous type A Nieckmann disease rarely live more than 18 months.

TYPE B: Symptoms of muscle coordination disorder and peripheral neuropathy (dysfunction of motor, sensory and autonomic peripheral nerves) occur in the pre-adolescent period. There are also enlarged spleen and liver (hepatosplenomegaly), short stature, recurrent liver infections and low level of platelets in the blood (thrombocytopenia). It has been determined that those with the TYPE B type of the disease live until adulthood.


Toxic amount of sphingomyelin in type A and B types as a result of deficiency in sphingomyelinase enzyme activity (a fatty substance found in each body cell)formation is observed.

Types C1 and C2: The symptoms of these types are very similar. They differ from each other only for genetic reasons. It occurs as a result of deficiency of NPC1 and NPC2 proteins. Niemann Pick Types C1 and C2 disease usually begin in childhood, but can show symptoms at any time in life. In individuals with these types of the disease, muscle coordination disorder (ataxia), inability to move the eyes vertically (VSGP), weak muscle tone (dystonia), severe liver disease, and interstitial lung disease are observed. Difficulty in swallowing and walking, which worsens over time, appears. Individuals with the disease can live into adulthood.

How to treat Niemann-Pick disease


First, the underlying cause must be determined. Accordingly, the treatment method should be shaped. Treatment often requires a multidisciplinary approach and should be tailored to the needs of the child. Here are some methods used in the treatment of childhood dementia:

  • Treatment of the underlying cause: If the cause of childhood dementia is due to a specific condition, such as a genetic disorder, metabolic disease, or infection, treatment should focus on the management of that underlying cause. This may include different modalities such as genetic counseling, dietary changes, medication or specific treatment of the infection.
  • Symptomatic treatment: Managing the child’s symptoms is important. For example, behavioral therapy or medication may be used for behavior problems. For sleep problems, sleep regulation and sleep aids can be used.
  • Supportive therapy: Children with childhood dementia will often benefit from a variety of therapy and rehabilitation services. Therapies such as physiotherapy, speech therapy and preschool/special education can help develop a child’s motor skills, communication skills and activities of daily living.
  • Family support: Caring for children with this disease can be challenging and place significant responsibilities on families. It is important to provide emotional support, counseling and information to families. Joining support groups and sharing experiences with other families can also be helpful.


​As every child’s situation is different, the treatment plan should be individualized. It should be noted that children with childhood dementia will require long-term care and follow-up. Treatment and support should be reviewed regularly according to the child’s age, symptoms, and needs.

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