Childhood Dementia: A Collective Clinical Approach to Advance Therapeutic Development and Care

Djafar, Jason V.; Johnson, Alexandra; Elvidge, Kristina L.; Farrar, Michelle A.

doi:10.1016/j.pediatrneurol.2022.11.015

Cited by 4 publications

(15 citation statements)

References 105 publications

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“…We included original, peer-reviewed articles published in English addressing the parent-reported psychosocial impacts of caring for a child with a childhood dementia. To surmount the heterogeneity between childhood dementia conditions, we adopted the clinical criteria proposed in Box 1, adapted from published case definitions [11]. When studies included sub-groups of parents of healthy children, general population norms, or parents of children with chronic diseases, we included these sub-groups as comparisons.…”

Section: Eligibility Criteriamentioning

confidence: 99%

“…Examples of conditions that consistently fit the definition of childhood dementia include Batten disease, Sanfilippo syndrome, Niemann-Pick disease types A and C, Tay-Sachs disease, metachromatic leukodystrophy, and some mitochondrial disorders, amongst others [7][8][9][10]. While global neurocognitive decline is a core deficit, each individual childhood dementia-causing disorder is clinically heterogeneous, with variable patterns of disease progression that affects every aspect of adaptive function [9][10][11]. Pathways to identify causes for this clinically severe group of conditions are complex [12].…”

Section: Introductionmentioning

confidence: 99%

“…It has been estimated that 75% of children with dementia die before the age of 18 [16]. While extensive progress has been made in understanding the underlying pathophysiology for individually rare childhood dementia conditions, with a growing number of genetic causes still being discovered, scientific advancements have yet to translate into accessible healthcare services and psychosocial supports for affected families [11,17,18].…”

Section: Introductionmentioning

confidence: 99%

“…In other cases, syndromes can be so severe that the impact on neurodevelopment and associated neurocognitive decline begins from a low baseline of development, resulting in the prevention of the attainment of developmental skills [20][21][22]. Although symptomatology can vary across disorder groups, children commonly develop a spectrum of coexisting and progressive symptoms related to the primary disease mechanism that impact cognitive, behavioral, and physical domains, including organ systems associated with their dementia condition [11,23]. Childhood dementia shares several hallmark features akin to adult dementia, including: decline in cognitive ability, memory loss, wandering and restlessness, emotional difficulties (e.g.…”

Section: Introductionmentioning

confidence: 99%

“…Moreover, 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 [11]. Childhood dementia conditions are also severely life-limiting and life-threatening; causes of death include respiratory complications (e.g.…”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

The psychosocial impact of childhood dementia on children and their parents: a systematic review

Nevin,

McGill,

Kelada

et al. 2023

Orphanet J Rare Dis

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Background Childhood dementias are a group of rare and ultra-rare paediatric conditions clinically characterised by enduring global decline in central nervous system function, associated with a progressive loss of developmentally acquired skills, quality of life and shortened life expectancy. Traditional research, service development and advocacy efforts have been fragmented due to a focus on individual disorders, or groups classified by specific mechanisms or molecular pathogenesis. There are significant knowledge and clinician skill gaps regarding the shared psychosocial impacts of childhood dementia conditions. This systematic review integrates the existing international evidence of the collective psychosocial experiences of parents of children living with dementia. Methods We used the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. We systematically searched four databases to identify original, peer-reviewed research reporting on the psychosocial impacts of childhood dementia, from the parent perspective. We synthesised the data into three thematic categories: parents’ healthcare experiences, psychosocial impacts, and information and support needs. Results Nineteen articles met review criteria, representing 1856 parents. Parents highlighted extensive difficulties connecting with an engaged clinical team and navigating their child’s rare, life-limiting, and progressive condition. Psychosocial challenges were manifold and encompassed physical, economic, social, emotional and psychological implications. Access to coordinated healthcare and community-based psychosocial supports was associated with improved parent coping, psychological resilience and reduced psychological isolation. Analysis identified a critical need to prioritize access to integrated family-centred psychosocial supports throughout distinct stages of their child’s condition trajectory. Conclusion This review will encourage and guide the development of evidence-based and integrated psychosocial resources to optimise quality of life outcomes for of children with dementia and their families.

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Section: Eligibility Criteriamentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

The psychosocial impact of childhood dementia on children and their parents: a systematic review

Nevin,

McGill,

Kelada

et al. 2023

Orphanet J Rare Dis

Self Cite

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Characterizing Common Phenotypes Across the Childhood Dementia Disorders: A Cross-sectional Study From Two Australian Centers

Djafar,

Smith,

Johnson

et al. 2023

Pediatric Neurology

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The collective burden of childhood dementia: a scoping review

Elvidge,

Christodoulou,

Farrar

et al. 2023

Brain

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Childhood dementia is a devastating and under-recognised group of disorders with a high level of unmet need. Typically monogenic in origin, this collective of individual neurodegenerative conditions are defined by a progressive impairment of neurocognitive function, presenting in childhood and adolescence. This scoping review aims to clarify definitions and conceptual boundaries of childhood dementia and quantify the collective disease burden. A literature review identified conditions that met the case definition. An expert clinical working group reviewed and ratified inclusion. Epidemiological data were extracted from published literature and collective burden modelled. One hundred and seventy genetic childhood dementia disorders were identified. Of these, 25 were analysed separately as treatable conditions. Collectively, currently untreatable childhood dementia was estimated to have an incidence of 34.5 per 100,000 (1 in 2,900 births), median life expectancy of 9 years and prevalence of 5.3 per 100,000 persons. The estimated number of premature deaths per year is similar to childhood cancer (0-14 years) and approximately 70% of those deaths will be prior to adulthood. An additional 49.8 per 100,000 births are attributable to treatable conditions that would cause childhood dementia if not diagnosed early and stringently treated. A relational database of the childhood dementia disorders has been created and will be continually updated as new disorders are identified (https://knowledgebase.childhooddementia.org/). We present the first comprehensive overview of monogenic childhood dementia conditions and their collective epidemiology. Unifying these conditions, with consistent language and definitions, reinforces motivation to advance therapeutic development and health service supports for this significantly disadvantaged group of children and their families.

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Childhood Dementia: A Collective Clinical Approach to Advance Therapeutic Development and Care

Cited by 4 publications

References 105 publications

The psychosocial impact of childhood dementia on children and their parents: a systematic review

The psychosocial impact of childhood dementia on children and their parents: a systematic review

Characterizing Common Phenotypes Across the Childhood Dementia Disorders: A Cross-sectional Study From Two Australian Centers

The collective burden of childhood dementia: a scoping review

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