When you think of dementia and Alzheimer's disease, you usually think of adult patients. In fact, there are forms of diseases that already occur in childhood and adolescence – with a severe course.
Childhood dementia is a collective term for 250 rare, neurodegenerative diseases in children and adolescents – in contrast to the 50 forms of dementia such as Alzheimer's in adults. This leads to a decline in cognitive and physical abilities, according to the German Center for Neurodegenerative Diseases (DZNE). What makes the diseases so dramatic for children and especially for parents: In most cases, those affected initially develop completely normally and inconspicuously, showing an age-appropriate cognitive and motor development of their abilities. Depending on the form of the disease, however, they inexorably lose these abilities again. The sick children are increasingly in need of care due to dementia. How early the first symptoms appear and how quickly they progress depends on the respective form of dementia.
Dementia in children: what forms of the disease are there?
Children with dementia initially develop completely normally until the first signs such as visual disturbances and changes in character appear. (Symbolic image) © Petra Schneider/Imago
According to the German Center for Neurodegenerative Diseases (DZNE), 1 in 2,000 to 1 in 500,000 children in Germany are affected, depending on the form of the disease. The most common forms of dementia in children are:
- Neuronal ceroid lipofuscinosis (NCL), known as "Batten's disease" in English-speaking countries
- Metachromatic leukodystrophy (MLD)
- X-linked adrenoleukodystrophy (X-ALD, Addison-Schilder syndrome), which primarily affects boys
- Alpers syndrome
- Multiple sulfatase deficiency (MSD, Austin syndrome)
Neuronal ceroid lipofuscinosis (NCL) affects about half of all children and adolescents affected by dementia. Approximately 40 percent are affected by various forms of leukodystrophy, hereditary metabolic diseases of the white brain substance substantia alba.
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Dementia in children: what is the cause of early disease?
Dementia in children is caused by hereditary genetic defects, which often lead to a disturbed metabolism in the brain. As a result, important building blocks for the structure and functions of brain tissue and nerve cells are missing, which means that toxic and disease-causing metabolic products cannot be broken down and are deposited in the brain. As a result, nerve cells are damaged and die.
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Childhood dementia: are there treatment options?
For some of the neurodegenerative diseases in children, effective treatment approaches are available through which missing substances or enzymes are supplied from the outside, for example in MLD and a special form of NCL. For some forms of dementia, such as X-ALD, transplantation of hematopoietic stem cells (hematopoietic stem cells) or gene therapy may be helpful, according to the DZNE. For the majority of dementias in children, there is currently no causal therapy that could achieve a complete cure.
The diagnosis of late infantile neuronal ceroid lipofuscinosis (CLN2), a form of so-called childhood dementia, has so far meant early death for most children. Scientists at the University Medical Center Hamburg-Eppendorf (UKE) have developed the world's first therapeutic option for the treatment of CLN2. "The study has shown that this therapy is the first to develop a remedy against the rapid progression of the disease," quotes WELT Professor Ania C. Muntau, Director of the Clinic and Polyclinic for Pediatrics and Adolescent Medicine at the UKE. The scientist also led the international study on dementia in children, the results of which were published in the New England Journal of Medicine.
Dementia in children: what are the symptoms?
The first signs usually appear in children between the ages of three and six. The disease usually progresses very quickly. Mental decline begins around the age of eight. As with adults who develop dementia, affected children often perceive the changes in themselves clearly, which results in a personality change and has a severe impact on the psyche. Furthermore, the following symptoms may occur:
- Poor eyesight and poor hearing, usually as the first signs of the disease
- Aggressive behaviour
- Frequent stumbling
- Seizures, epileptic seizures
- Cardiac insufficiency
- Pronunciation gets worse, at about 13 years of age, many of the affected children can no longer speak
- Ability to write and calculate is lost
- Decline in motor skills, children can no longer walk
Often, a diagnosis is only made when the disease is already well advanced neuronally. Many doctors do not initially consider this rare disease in children, according to the Federal Guild Health Insurance Fund (BIG).
This article contains only general information on the respective health topic and is therefore not intended for self-diagnosis, treatment or medication. It is in no way a substitute for a visit to the doctor. Unfortunately, individual questions about clinical pictures may not be answered by our editors.