Childhood dementia is an umbrella group consisting of over 100 rare neurodegenerative conditions. These are typically genetic, that cause progressive cognitive decline and the loss of previously acquired skills (e.g: talking, walking, reading/writing, etc).
| Childhood dementia | |
|---|---|
| Other names | Pediatric dementia |
| Specialty | Neurology, Psychiatry, Pediatrics |
| Symptoms | Loss of previously acquired developmental skills, seizures, cognitive decline |
| Usual onset | Childhood or adolescence |
| Duration | Progressive |
| Causes | Genetic disorders, neurodegenerative diseases |
| Diagnostic method | Biochemical testing, genetic testing |
| Treatment | Symptomatic treatment (anticonvulsants, physiotherapy, etc.) |
| Prognosis | Poor (Median life expectancy of 9 years; Average life expectancy of 16.3 years) |
The group of conditions has a very poor prognosis on average, as the progression of cognitive decline and other symptoms/complications have a very significant impact on the life expectancy and quality of those affected.
Neuronal ceroid lipofuscinoses, a group of lysosomal storage disorders, are thought to be the most common cause of childhood dementia.[1]
Signs and symptoms
editAccording to multiple definitions, childhood dementia is characterized by a global, progressive decline of cognitive function and developmental skills[2][3].[4] The decline typically begins after a period of seemingly normal development[5], which likely lasts anywhere from a few years to adolescence, depending on multiple factors.[2]
This progressive decline causes difficulty concentrating, memory loss, confusion, and learning difficulties[6], in addition to the loss of developmental skills acquired previously, such as: walking, talking, writing, reading, and playing[6][7]. Eventually the body loses its ability to function, leading to an early death.[5][6][7]
Meanwhile, other symptoms and complications can occur depending on the subtype.[2]
These symptoms can include:
- Behavioral changes: Personality shifts[citation needed], aggression, and hyperactivity.[8]
- Motor decline: Loss of coordination[citation needed], balance[citation needed], and movement[6] abilities.
- Seizures: Frequent in many forms of childhood dementia.[2][6]
Whereas potential complications include:
- Loss of vision or hearing.[6]
- Cardiovascular, respiratory, and/or digestive problems[6]
- Bone or joint problems.[6]
Causes
editThe 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.[1] Other causes include mitochondrial diseases, peroxisomal disorders, and other genetic disorders affecting brain function.[9]
Diagnosis
editDiagnosis 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.[2] In most cases, childhood dementia is diagnosed after developmental regression is observed.
Management
editThere 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.[2]
- Therapies: Physiotherapy, occupational therapy, and speech therapy are used to maintain physical function for as long as possible.[2]
- Supportive care: This includes comprehensive care to address complications related to mobility, feeding, breathing, and communication.[8] Supportive care measures are customized for each patient and may include:
- Respiratory support: To manage breathing difficulties in advanced stages.
- Nutritional support: For example, feeding tubes when swallowing becomes difficult.
- Palliative care: Ensuring comfort and quality of life as the disease progresses.[10]
Prognosis
editThe 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.[2]
The median life expectancy is around 9 years, whereas the average life expectancy is 16.3 years.[2]
Epidemiology
editCurrent estimates place the incidence of childhood dementias at 1 in 1186 births.[2] This is higher than the incidence of some diseases with more widespread awareness, such as cystic fibrosis[11] (affecting around 1 in 3000-4000 births)[12] and spinal muscular atrophy (around 1 in 11000 births)[2][13].
Meanwhile, the estimates for the prevalence are lower, at 1 in 3484 people in the general population[2] and 1 in 1715 among children.[2]
History
editThe 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.[14]
See also
editReferences
edit- ^ a b Schulz, Angela; Kohlschütter, Alfried (2013). "NCL Disorders: Frequent Causes of Childhood Dementia". Iranian Journal of Child Neurology. 7 (1): 1–8. PMC 3943077. PMID 24665282.
- ^ a b c d e f g h i j k l m Elvidge, Kristina L; Christodoulou, John; Farrar, Michelle A (2023-07-20). "Collective burden of childhood dementia: a scoping review". Brain. 146 (11): 4446–4464. doi:10.1093/brain/awad208. PMC 10545495. PMID 37365902. Retrieved 2024-09-24.
- ^ Atee, Mustafa; Whiteman, Ineka; Lloyd, Rebecca; Morris, Thomas (2024). "Behaviours and psychological symptoms of childhood dementia: two cases of psychosocial interventions". Palliative Care and Social Practice. 18: 26323524241273492. doi:10.1177/26323524241273492. ISSN 2632-3524. PMID 39247715.
- ^ Djafar, Jason V.; Johnson, Alexandra M.; Elvidge, Kristina L.; Farrar, Michelle A. (2023-02-01). "Childhood Dementia: A Collective Clinical Approach to Advance Therapeutic Development and Care". Pediatric Neurology. 139: 76–85. doi:10.1016/j.pediatrneurol.2022.11.015. ISSN 0887-8994.
- ^ a b Oeffentlichkeitsarbeit, DZNE Abt fuer Presse-und. "Childhood dementia". DZNE (in German). Retrieved 2024-10-01.
- ^ a b c d e f g h "Childhood Dementia". Dementia Australia. Retrieved 2024-09-24.
- ^ a b "What is childhood dementia?". Childhood Dementia Initiative. Retrieved 2024-10-01.
- ^ a b Hemsley, Kim; Smith, Nicholas; Mubarokah, Siti (2024-04-26). "What is childhood dementia? And how could new research help?". The Conversation. Retrieved 2024-09-24.
- ^ Di Rocco, Giorgio; Tagliavini, Fabio; Di Fede, Giovanni; Pinessi, Lorenzo (2001). "Childhood Dementia Due to Neurodegenerative Diseases". Journal of Child Neurology. 16 (9): 679–685. doi:10.1177/088307380101600909.
- ^ "Childhood Dementia Support". Dementia Support Australia. Retrieved 2024-09-24.
- ^ Nevin, Suzanne M.; McGill, Brittany C.; Kelada, Lauren; Hilton, Gail; Maack, Megan; Elvidge, Kristina L.; Farrar, Michelle A.; Baynam, Gareth; Katz, Naomi T.; Donovan, Leigh; Grattan, Sarah; Signorelli, Christina; Bhattacharya, Kaustuv; Nunn, Kenneth; Wakefield, Claire E. (2023-09-07). "The psychosocial impact of childhood dementia on children and their parents: a systematic review". Orphanet Journal of Rare Diseases. 18: 277. doi:10.1186/s13023-023-02859-3. ISSN 1750-1172. PMC 10486052. PMID 37679855.
- ^ Sanders, Don B.; Fink, Aliza (August 2016). "Background and Epidemiology". Pediatric Clinics of North America. 63 (4): 567–584. doi:10.1016/j.pcl.2016.04.001. ISSN 0031-3955. PMC 4967225. PMID 27469176.
- ^ Mercuri, Eugenio; Finkel, Richard S.; Muntoni, Francesco; Wirth, Brunhilde; Montes, Jacqueline; Main, Marion; Mazzone, Elena S.; Vitale, Michael; Snyder, Brian; Quijano-Roy, Susana; Bertini, Enrico; Davis, Rebecca Hurst; Meyer, Oscar H.; Simonds, Anita K.; Schroth, Mary K. (February 2018). "Diagnosis and management of spinal muscular atrophy: Part 1: Recommendations for diagnosis, rehabilitation, orthopedic and nutritional care". Neuromuscular Disorders: NMD. 28 (2): 103–115. doi:10.1016/j.nmd.2017.11.005. hdl:2434/702816. ISSN 1873-2364. PMID 29290580.
- ^ "A Comprehensive Guide to Childhood Dementia: What You Need to Know". Discover Magazine. Retrieved 2024-09-24.
Further reading
edit- Nevin, SM et al. (2023). "The psychosocial impact of childhood dementia on children and their parents: a systematic review." Orphanet Journal of Rare Diseases. doi:10.1186/s13023-023-02859-3.
- Kohlschütter, A. (2013). "NCL Disorders: Frequent Causes of Childhood Dementia." Iranian Journal of Child Neurology.