Childhood dementia is a group of rare neurodegenerative conditions that lead to progressive cognitive and physical decline in children. It affects mental function, causing the loss of previously acquired developmental skills such as walking, talking, and reasoning. This umbrella term includes over 100 distinct disorders, many of which are linked to genetic causes.[1] The most common cause of childhood dementia is neuronal ceroid lipofuscinoses (NCL), a group of lysosomal storage disorders.[2]
| 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 (life expectancy between 9-16 years) |
Signs and symptoms
editChildhood dementia typically manifests as a gradual loss of cognitive and motor functions. Symptoms can vary depending on the specific condition but often include:
- Cognitive decline: Difficulty concentrating, memory loss, confusion, and learning difficulties.[3]
- Behavioral changes: Personality shifts, aggression, and hyperactivity.[4]
- Motor decline: Loss of coordination, balance, and movement abilities.
- Seizures: Frequent in many forms of childhood dementia.[1]
Other complications include loss of vision or hearing, respiratory issues, and bone or joint problems.
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.[2] Other causes include mitochondrial diseases, peroxisomal disorders, and other genetic disorders affecting brain function.[5]
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.[1] In most cases, childhood dementia is diagnosed after developmental regression is observed.
Management
editThere is no cure for most forms of childhood dementia. Treatment focuses on managing symptoms and improving quality of life. Common management strategies include:
- Medications: Anti-seizure medications, behaviour-modifying drugs, and muscle relaxants.[1]
- Therapies: Physiotherapy, occupational therapy, and speech therapy are used to maintain physical function for as long as possible.[1]
- Supportive care: This includes comprehensive care to address complications related to mobility, feeding, breathing, and communication.[4] 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.[6]
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.[1] The median life expectancy is around 9 years.[1]
Epidemiology
editCurrent estimates place the incidence of childhood dementias at 1 in 1186 births. [1] This is higher than the incidence of some well-recognised diseases, such as Cystic Fibrosis[7] (~1 in 3000-4000 births[8]) and Spinal Muscular Atrophy (1 in 11000 births[1][9]).
The estimates for the prevalence are 1 in 3484 people in the general population and 1 per 1715 among children.
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. British neurologists first described batten disease Frederick Batten in 1903.[10]
See also
editReferences
edit- ^ a b c d e f g h i 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.
- ^ 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.
- ^ "Childhood Dementia". Dementia Australia. Retrieved 2024-09-24.
- ^ 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.