- Source: Childhood dementia
Childhood dementia is an umbrella group of rare, mostly untreatable neurodegenerative disorders that show symptoms before the age of 18. These conditions cause progressive deterioration of the brain and the loss of previously acquired skills, including: talking, walking, and playing.
Other symptoms and complications are the loss of movement, vision, and hearing; seizures; and cardiorrespiratory, bone, and joint problems. As the conditions progress, so does their impact on life expectancy, quality of life. Due to this, most conditions in the group have a poor prognosis and cause a high degree of dependence as they progress.
Childhood dementia is genetic and progressive, distinguishing it from other sources of cognitive decline like traumatic brain injury and nutrient deficiencies.
Classification and terminology
Childhood dementias are a heterogenous group of genetic neurodegenerative disorders, that present symptoms before the age of 18. They are typically monogenic (caused by mutations of a single gene).
Their main characteristics are chronic and widespread cognitive decline; loss of previously acquired developmental skills after a period of typical development; and behaviours and psychological symptoms of dementia (BPSD).
Childhood dementias are distinct from sources of intellectual disability in childhood that are non-progressive (e.g traumatic brain injury) or acquired (e.g nutritional deficiencies or encephalitis).
Prognosis
The prognosis for childhood dementia is generally poor, with most children experiencing a significant decline in cognitive and motor function. Life expectancy varies depending on the underlying cause, but it is often significantly reduced. Studies show that only 25–29% of affected individuals survive to adulthood, and only 10% reach the age of 50.
The median life expectancy is around 9 years, whereas the average life expectancy is 16.3 years.
Signs and symptoms
By their usual definitions, childhood dementias always cause global neurocognitive decline, typically beginning after a period of seemingly normal development.
This progressive decline causes difficulty concentrating, memory loss, confusion, and learning difficulties, in addition to the loss of developmental skills acquired previously, such as: walking, talking, writing, reading, and playing. Eventually the body loses its ability to function, leading to an early death.
Other symptoms and complications can occur depending on the subtype.
Other symptoms:
Behavioral changes: Changes in one's personality, aggression, and hyperactivity.
Motor decline: Loss of coordination, balance, and movement abilities.
Seizures: Frequent in many forms of childhood dementia.
Other complications:
Loss of vision or hearing.
Cardiovascular, respiratory, and/or digestive problems
Bone or joint problems.
Causes
The majority of childhood dementia cases are caused by genetic mutations that lead to neurodegenerative diseases. The most frequent cause is neuronal ceroid lipofuscinoses (NCL), a family of lysosomal storage disorders. Other causes include mitochondrial diseases, peroxisomal disorders, and other genetic disorders affecting brain function.
Diagnosis
Diagnosis typically involves a combination of biochemical testing and genetic testing, often performed around the age of four. Early diagnosis is crucial for managing symptoms and improving the quality of life for those affected. In most cases, childhood dementia is diagnosed after developmental regression is observed.
Management
There is no treatment for most forms of childhood dementia. For these untreatable conditions, treatment focuses on managing symptoms and improving quality of life.
This can include:
Medications: Anti-seizure medications, behaviour-modifying drugs, and muscle relaxants.
Therapies: Physiotherapy, occupational therapy, and speech therapy are used to maintain physical function for as long as possible.
Supportive care: This includes comprehensive care to address complications related to mobility, feeding, breathing, and communication. Supportive care measures are customized for each patient and may include:
Respiratory support, to manage breathing difficulties in advanced stages.
Nutritional support, including the use of feeding tubes when swallowing becomes difficult.
Palliative care to ensure comfort and quality of life as the disease progresses.
Psychological impact
Childhood dementia can significantly affect both parents and the affected child by causing anxiety, feelings of helplessness, profound grief, and a sense of loss as the child conditions
continues to progress over time. Children with childhood dementias suffer severe sleep disturbances, movement disorders (e.g. muscle spasms, tremors), deterioration of communication
skills, loss of vision and hearing, mood disorders, psychosis (including hallucinations and delusions) and incontinence. This situation can cause many emotional changes for parents and children. The psychological impacts that it has on children are confusion/frustration, loss of independence, social isolation, and fear, while parents often experience self-blame, stress, financial problems, and a loss of identity. For example, sleep disturbances and behavioral difficulties can exacerbate parent distress, anxiety, sleep quality and subsequent capacity to care for their child healthcare needs.
Epidemiology
Current estimates place the incidence of childhood dementias at 1 in 1186 births. This is higher than the incidence of some diseases with more widespread awareness, such as cystic fibrosis (affecting around 1 in 3000-4000 births) and spinal muscular atrophy (around 1 in 11000 births).
Meanwhile, the estimates for the prevalence are lower, at 1 in 3484 people in the general population and 1 in 1715 among children.
History
The concept of childhood dementia gained recognition in the early 20th century with the identification of Batten disease, one of the first known forms of childhood dementia, by British neurologist Frederick Batten in 1903.
See also
Neuronal ceroid lipofuscinosis
Batten disease
Lysosomal storage disease
Neurodegenerative disease
References
External links
Dementia Australia: Childhood Dementia
Childhood Dementia Initiative
Kata Kunci Pencarian:
- Kesehatan jiwa
- Diabetes melitus
- Karl Abraham
- Kegemukan
- Sean Connery
- Stres psikologis
- IFC Films
- Childhood dementia
- Frontotemporal dementia
- Dementia
- Childhood disintegrative disorder
- Vascular dementia
- Sanfilippo syndrome
- Alzheimer's disease
- Huntington's disease
- Early onset dementia
- Sundowning
Cemetery Junction (2010)
Bioskop Online, bioskop21, BioskopKeren, Cinemaindo, Comedy, Dewanonton, Drakor ID, DrakorIndo, Drama, DramaQu, Dunia21, DutaFilm, Ganool, gudangmovie, gudangmovie21, IndoXX1, Indoxxi, KorDramas, LayarKaca21, Layarkaca21 INDOXXI, LK21, LK21 XXI, Nonton drama, Nonton Movie, Pahe.in, PusatFilm21, United Kingdom
Ricky Gervais
Bad Education (2004)
Bioskop Online, BioskopKeren, Cinemaindo, Crime, Dewanonton, Drakor ID, DrakorIndo, Drama, DramaQu, Dunia21, DutaFilm, Ganool, gudangmovie, IndoXX1, Indoxxi, KorDramas, LayarKaca21, Layarkaca21 INDOXXI, LK21, LK21 XXI, Nonton drama, Nonton Movie, Pahe.in, PusatFilm21, Belgium, Spain
Pedro Almodóvar
Miss Violence (2013)
Bioskop Online, BioskopKeren, Cinemaindo, Dewanonton, Drakor ID, DrakorIndo, Drama, DramaQu, Dunia21, DutaFilm, Ganool, gudangmovie, IndoXX1, Indoxxi, KorDramas, LayarKaca21, Layarkaca21 INDOXXI, LK21, LK21 XXI, Nonton drama, Nonton Movie, Pahe.in, PusatFilm21, Greece
Alexandros Avranas
No More Posts Available.
No more pages to load.
