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Early onset dementia (EOD) or young onset dementia (YOD) refers to dementia with symptom onset prior to age 65. This condition is a significant public health concern as the number of individuals with early onset dementia is increasing worldwide^[1].

Article body

Overview

Early onset dementia is a general term that describes a heterogenous group of conditions that feature progressive cognitive decline, particularly deficits in the domains of executive function, learning, language, memory, or behavior. This condition may occur due to various different causes, including degenerative, autoimmune, or infectious processes. The most common form of early onset dementia is Alzheimer's disease, followed by frontotemporal dementia (FTD), and vascular dementia, with Alzheimer's disease accounting for between 40 and 50% of cases^[2]^[3]. Less common forms of early onset dementia include Lewy body dementias (dementia with Lewy bodies and Parkinson's disease dementia), Huntington's disease, Creutzfeldt–Jakob disease, multiple sclerosis, alcohol-induced dementia, and other conditions.

Terminology

The term young onset dementia is becoming more widely used to avoid the potential confusion between early onset dementia and early stage dementia^[4]. Although used in the past, the term presenile dementia is no longer in favor.

Epidemiology

Early onset dementia is less common than late onset dementia, the former accounting for approximately 10% of dementias globally^[3]. Recent studies estimate the prevalence of early onset dementia to be approximately 3.9 million people aged 30-64 worldwide, with an incidence of 119 per 100,000 individuals^[1]. Additionally, there is approximately a 1:1 ratio in prevalence of EOD between males and females, with no significant difference between ethnic groups in gender distribution pattern^[5]^[6]. Similar to LOD, the prevalence of EOD increases exponentially with age, doubling every five years of age^[5]. The continuous increase in prevalence with age seen in Alzheimer's and FTD versions of EOD is disproportionally led by the most common variant of each cause, namely amnesic Alzheimer's and behavioral variant of FTD^[7].

Risk factors

Traditional risk factors for the development of late onset dementia, such as diabetes mellitus, hypertension, and obesity, have also been identified as risk factors for early onset dementia. Several other chronic conditions have recently been identified that are also associated with the development of early onset dementia, including cardiovascular, respiratory, or gastrointestinal disease^[8]. Significantly, the presence of one or multiple of these chronic conditions is more predictive of EOD compared to LOD^[8].

Diagnosis

Though widely accepted, the definition of early onset dementia as less than 65 years of age continues to be an artificial diagnostic criterion based on the traditional retirement age in most countries^[9]. Nevertheless, the purpose of having a numerical age cut-off is evidenced in the significant differences in the etiology and prognosis of dementia depending on the age category of the patient. Furthermore, the diagnosis of early onset dementia continues to be challenging due to the wide range of symptoms at presentation and propensity not to consider neurodegenerative causes in this population. Recent studies indicate an average of 4.4 years time to diagnosis for EOD, compared to 2.8 years for LOD^[5].

Disease course

Compared to late onset dementia, patients with early onset dementia are more likely to have dementias other than Alzheimer's disease, although Alzheimer's is the most common etiology in either case^[9]. In general, EOD has a faster progression and features more extensive neurological damage when compared to LOD. It is hypothesized that this may be due to decreased cognitive reserve seen in late onset dementias, causing greater complication relative to pathological damage^[9]. Furthermore, studies have shown differences in the areas of cognition that are likely to be affected when comparing early onset to late onset dementia. In terms of behavioral symptoms, EOD is more likely to affect attention, but less likely to cause confusion, delusions, hallucinations, agitation, or disinhibition. In terms of motor symptoms, EOD is less likely to affect verbal fluency and motor executive function compared to late onset dementia^[9].

Prognosis

Estimation of survival rate in early onset dementias is a component patient prognosis, management, and treatment. In general, a better prognosis is positively correlated with earlier age of onset^[9]. Average survival time is approximately 6-10 years following diagnosis for both men and women, with variability depending on specific type of dementia^[5]^[10]. The most common cause of immediate death in EOD is respiratory disease (e.g. pneumonia); other causes include cardiovascular events and cerebrovascular disease^[9].

Specific types of early onset dementia

Alzheimer's early onset dementia

Alzheimer's disease is the most common cause of both early and late onset dementia, and continuously increases in prevalence with age^[7]. Studies indicate that family history is a significant risk factor for specifically Alzheimer's early onset dementia^[6].

Though not a major cause of early onset dementia, TBIs are an important cause of EOD. Young individuals, especially males, are more likely to be part of this population group.

References

^ ^a ^b Hendriks, Stevie; Peetoom, Kirsten; Bakker, Christian; van der Flier, Wiesje M.; Papma, Janne M.; Koopmans, Raymond; Verhey, Frans R. J.; de Vugt, Marjolein; Köhler, Sebastian; Withall, Adrienne; Parlevliet, Juliette L.; Uysal-Bozkir, Özgül; Gibson, Roger C.; Neita, Susanne M.; Nielsen, Thomas Rune (2021-09). "Global Prevalence of Young-Onset Dementia". JAMA Neurology. 78 (9): 1–11. doi:10.1001/jamaneurol.2021.2161. ISSN 2168-6149. PMC 8290331. PMID 34279544. {{cite journal}}: Check date values in: |date= (help)
^ Quach, C.; Hommet, C.; Mondon, K.; Lauvin, M. A.; Cazals, X.; Cottier, J. P. (2014-04-01). "Early-onset dementias: Specific etiologies and contribution of MRI". Diagnostic and Interventional Imaging. 95 (4): 377–398. doi:10.1016/j.diii.2013.07.009. ISSN 2211-5684.
^ ^a ^b Krüger, Johanna; Aaltonen, Mikko; Aho, Kalle; Heikkinen, Sami; Kivisild, Ave; Lehtonen, Adolfina; Leppänen, Laura; Rinnankoski, Iina; Soppela, Helmi; Tervonen, Laura; Suhonen, Noora-Maria; Haapasalo, Annakaisa; Portaankorva, Anne M.; Mäki-Petäjä-Leinonen, Anna; Hartikainen, Päivi (2024-08-27). "Incidence and Prevalence of Early-Onset Dementia in Finland". Neurology. 103 (4): e209654. doi:10.1212/WNL.0000000000209654. ISSN 0028-3878. PMID 39047214.
^ van de Veen, Dennis; Bakker, Christian; Peetoom, Kirsten; Pijnenburg, Yolande; Papma, Janne; de Vugt, Marjolein; Koopmans, Raymond (2022-03). "Provisional consensus on the nomenclature and operational definition of dementia at a young age, a Delphi study". International Journal of Geriatric Psychiatry. 37 (3): 10.1002/gps.5691. doi:10.1002/gps.5691. ISSN 0885-6230. PMC 9305901. PMID 35156239. {{cite journal}}: Check date values in: |date= (help)
^ ^a ^b ^c ^d Hendriks, Stevie; Peetoom, Kirsten; Bakker, Christian; Koopmans, Raymond; van der Flier, Wiesje; Papma, Janne; Verhey, Frans; Young‐Onset Dementia Epidemiology Study Group; de Vugt, Marjolein; Köhler, Sebastian (2023-03). "Global incidence of young‐onset dementia: A systematic review and meta‐analysis". Alzheimer's & Dementia. 19 (3): 831–843. doi:10.1002/alz.12695. ISSN 1552-5260. {{cite journal}}: Check date values in: |date= (help)
^ ^a ^b Kelly, BJ; Boeve, BF; Josephs, KA (2008). "Young-Onset Dementia: Demographic and Etiologic Characteristics of 235 Patients". jamanetwork.com. Retrieved 2024-10-10.
^ ^a ^b Zamboni, Giovanna; Maramotti, Riccardo; Salemme, Simone; Tondelli, Manuela; Adani, Giorgia; Vinceti, Giulia; Carbone, Chiara; Filippini, Tommaso; Vinceti, Marco; Pagnoni, Giuseppe; Chiari, Annalisa (2024). "Age-specific prevalence of the different clinical presentations of AD and FTD in young-onset dementia". Journal of Neurology. 271 (7): 4326–4335. doi:10.1007/s00415-024-12364-7. ISSN 0340-5354. PMID 38643445.
^ ^a ^b Shang, Xianwen; Zhu, Zhuoting; Zhang, Xueli; Huang, Yu; Zhang, Xiayin; Liu, Jiahao; Wang, Wei; Tang, Shulin; Yu, Honghua; Ge, Zongyuan; Yang, Xiaohong; He, Mingguang (2022-03). "Association of a wide range of chronic diseases and apolipoprotein E4 genotype with subsequent risk of dementia in community-dwelling adults: A retrospective cohort study". EClinicalMedicine. 45: 101335. doi:10.1016/j.eclinm.2022.101335. ISSN 2589-5370. PMC 8921546. PMID 35299656. {{cite journal}}: Check date values in: |date= (help)
^ ^a ^b ^c ^d ^e ^f Vieira, Renata Teles; Caixeta, Leonardo; Machado, Sergio; Silva, Adriana Cardoso; Nardi, Antonio Egidio; Arias-Carrión, Oscar; Carta, Mauro Giovanni (2013-06-14). "Epidemiology of early-onset dementia: a review of the literature". Clinical Practice and Epidemiology in Mental Health : CP & EMH. 9: 88–95. doi:10.2174/1745017901309010088. ISSN 1745-0179. PMC 3715758. PMID 23878613.
^ Kay, David W. K.; Forster, Donald P.; Newens, Andrew J. (2000-08). "Long-term survival, place of death, and death certification in clinically diagnosed pre-senile dementia in northern England: Follow-up after 8–12 years". British Journal of Psychiatry. 177 (2): 156–162. doi:10.1192/bjp.177.2.156. ISSN 0007-1250. {{cite journal}}: Check date values in: |date= (help)

[:1-1] Hendriks, Stevie; Peetoom, Kirsten; Bakker, Christian; van der Flier, Wiesje M.; Papma, Janne M.; Koopmans, Raymond; Verhey, Frans R. J.; de Vugt, Marjolein; Köhler, Sebastian; Withall, Adrienne; Parlevliet, Juliette L.; Uysal-Bozkir, Özgül; Gibson, Roger C.; Neita, Susanne M.; Nielsen, Thomas Rune (2021-09). "Global Prevalence of Young-Onset Dementia". JAMA Neurology. 78 (9): 1–11. doi:10.1001/jamaneurol.2021.2161. ISSN 2168-6149. PMC 8290331. PMID 34279544. {{cite journal}}: Check date values in: |date= (help)

[2] Quach, C.; Hommet, C.; Mondon, K.; Lauvin, M. A.; Cazals, X.; Cottier, J. P. (2014-04-01). "Early-onset dementias: Specific etiologies and contribution of MRI". Diagnostic and Interventional Imaging. 95 (4): 377–398. doi:10.1016/j.diii.2013.07.009. ISSN 2211-5684.

[:3-3] Krüger, Johanna; Aaltonen, Mikko; Aho, Kalle; Heikkinen, Sami; Kivisild, Ave; Lehtonen, Adolfina; Leppänen, Laura; Rinnankoski, Iina; Soppela, Helmi; Tervonen, Laura; Suhonen, Noora-Maria; Haapasalo, Annakaisa; Portaankorva, Anne M.; Mäki-Petäjä-Leinonen, Anna; Hartikainen, Päivi (2024-08-27). "Incidence and Prevalence of Early-Onset Dementia in Finland". Neurology. 103 (4): e209654. doi:10.1212/WNL.0000000000209654. ISSN 0028-3878. PMID 39047214.

[4] van de Veen, Dennis; Bakker, Christian; Peetoom, Kirsten; Pijnenburg, Yolande; Papma, Janne; de Vugt, Marjolein; Koopmans, Raymond (2022-03). "Provisional consensus on the nomenclature and operational definition of dementia at a young age, a Delphi study". International Journal of Geriatric Psychiatry. 37 (3): 10.1002/gps.5691. doi:10.1002/gps.5691. ISSN 0885-6230. PMC 9305901. PMID 35156239. {{cite journal}}: Check date values in: |date= (help)

[:4-5] Hendriks, Stevie; Peetoom, Kirsten; Bakker, Christian; Koopmans, Raymond; van der Flier, Wiesje; Papma, Janne; Verhey, Frans; Young‐Onset Dementia Epidemiology Study Group; de Vugt, Marjolein; Köhler, Sebastian (2023-03). "Global incidence of young‐onset dementia: A systematic review and meta‐analysis". Alzheimer's & Dementia. 19 (3): 831–843. doi:10.1002/alz.12695. ISSN 1552-5260. {{cite journal}}: Check date values in: |date= (help)

[:2-6] Kelly, BJ; Boeve, BF; Josephs, KA (2008). "Young-Onset Dementia: Demographic and Etiologic Characteristics of 235 Patients". jamanetwork.com. Retrieved 2024-10-10.

[:5-7] Zamboni, Giovanna; Maramotti, Riccardo; Salemme, Simone; Tondelli, Manuela; Adani, Giorgia; Vinceti, Giulia; Carbone, Chiara; Filippini, Tommaso; Vinceti, Marco; Pagnoni, Giuseppe; Chiari, Annalisa (2024). "Age-specific prevalence of the different clinical presentations of AD and FTD in young-onset dementia". Journal of Neurology. 271 (7): 4326–4335. doi:10.1007/s00415-024-12364-7. ISSN 0340-5354. PMID 38643445.

[:6-8] Shang, Xianwen; Zhu, Zhuoting; Zhang, Xueli; Huang, Yu; Zhang, Xiayin; Liu, Jiahao; Wang, Wei; Tang, Shulin; Yu, Honghua; Ge, Zongyuan; Yang, Xiaohong; He, Mingguang (2022-03). "Association of a wide range of chronic diseases and apolipoprotein E4 genotype with subsequent risk of dementia in community-dwelling adults: A retrospective cohort study". EClinicalMedicine. 45: 101335. doi:10.1016/j.eclinm.2022.101335. ISSN 2589-5370. PMC 8921546. PMID 35299656. {{cite journal}}: Check date values in: |date= (help)

[:0-9] ^ ^a ^b ^c ^d ^e ^f Vieira, Renata Teles; Caixeta, Leonardo; Machado, Sergio; Silva, Adriana Cardoso; Nardi, Antonio Egidio; Arias-Carrión, Oscar; Carta, Mauro Giovanni (2013-06-14). "Epidemiology of early-onset dementia: a review of the literature". Clinical Practice and Epidemiology in Mental Health : CP & EMH. 9: 88–95. doi:10.2174/1745017901309010088. ISSN 1745-0179. PMC 3715758. PMID 23878613.

[10] Kay, David W. K.; Forster, Donald P.; Newens, Andrew J. (2000-08). "Long-term survival, place of death, and death certification in clinically diagnosed pre-senile dementia in northern England: Follow-up after 8–12 years". British Journal of Psychiatry. 177 (2): 156–162. doi:10.1192/bjp.177.2.156. ISSN 0007-1250. {{cite journal}}: Check date values in: |date= (help)

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 ==== Overview ====
-Early onset dementia is a general term that describes a heterogenous group of conditions that feature progressive cognitive decline, particularly deficits in executive function, learning, language, memory, or behavior. This condition may occur due to various different causes, including degenerative, autoimmune, or infectious processes. The most common form of early onset dementia is [[Early-onset Alzheimer's disease|Alzheimer's disease]], followed by [[frontotemporal dementia]] (FTD), and [[vascular dementia]], with Alzheimer's disease accounting for between 40 and 50% of cases<ref>{{Cite journal |last=Quach |first=C. |last2=Hommet |first2=C. |last3=Mondon |first3=K. |last4=Lauvin |first4=M. A. |last5=Cazals |first5=X. |last6=Cottier |first6=J. P. |date=2014-04-01 |title=Early-onset dementias: Specific etiologies and contribution of MRI |url=https://www.sciencedirect.com/science/article/pii/S2211568413002350?via=ihub |journal=Diagnostic and Interventional Imaging |volume=95 |issue=4 |pages=377–398 |doi=10.1016/j.diii.2013.07.009 |issn=2211-5684}}</ref><ref name=":3">{{Cite journal |last=Krüger |first=Johanna |last2=Aaltonen |first2=Mikko |last3=Aho |first3=Kalle |last4=Heikkinen |first4=Sami |last5=Kivisild |first5=Ave |last6=Lehtonen |first6=Adolfina |last7=Leppänen |first7=Laura |last8=Rinnankoski |first8=Iina |last9=Soppela |first9=Helmi |last10=Tervonen |first10=Laura |last11=Suhonen |first11=Noora-Maria |last12=Haapasalo |first12=Annakaisa |last13=Portaankorva |first13=Anne M. |last14=Mäki-Petäjä-Leinonen |first14=Anna |last15=Hartikainen |first15=Päivi |date=2024-08-27 |title=Incidence and Prevalence of Early-Onset Dementia in Finland |url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11314947/ |journal=Neurology |volume=103 |issue=4 |pages=e209654 |doi=10.1212/WNL.0000000000209654 |issn=0028-3878 |pmid=39047214}}</ref>. Less common forms of early onset dementia include [[Lewy body dementias]] ([[dementia with Lewy bodies]] and [[Parkinson's disease dementia]]), [[Huntington's disease]], [[Creutzfeldt–Jakob disease]], [[multiple sclerosis]], alcohol-induced dementia, and other conditions.
+Early onset dementia is a general term that describes a heterogenous group of conditions that feature progressive cognitive decline, particularly deficits in the domains of executive function, learning, language, memory, or behavior. This condition may occur due to various different causes, including degenerative, autoimmune, or infectious processes. The most common form of early onset dementia is [[Early-onset Alzheimer's disease|Alzheimer's disease]], followed by [[frontotemporal dementia]] (FTD), and [[vascular dementia]], with Alzheimer's disease accounting for between 40 and 50% of cases<ref>{{Cite journal |last=Quach |first=C. |last2=Hommet |first2=C. |last3=Mondon |first3=K. |last4=Lauvin |first4=M. A. |last5=Cazals |first5=X. |last6=Cottier |first6=J. P. |date=2014-04-01 |title=Early-onset dementias: Specific etiologies and contribution of MRI |url=https://www.sciencedirect.com/science/article/pii/S2211568413002350?via=ihub |journal=Diagnostic and Interventional Imaging |volume=95 |issue=4 |pages=377–398 |doi=10.1016/j.diii.2013.07.009 |issn=2211-5684}}</ref><ref name=":3">{{Cite journal |last=Krüger |first=Johanna |last2=Aaltonen |first2=Mikko |last3=Aho |first3=Kalle |last4=Heikkinen |first4=Sami |last5=Kivisild |first5=Ave |last6=Lehtonen |first6=Adolfina |last7=Leppänen |first7=Laura |last8=Rinnankoski |first8=Iina |last9=Soppela |first9=Helmi |last10=Tervonen |first10=Laura |last11=Suhonen |first11=Noora-Maria |last12=Haapasalo |first12=Annakaisa |last13=Portaankorva |first13=Anne M. |last14=Mäki-Petäjä-Leinonen |first14=Anna |last15=Hartikainen |first15=Päivi |date=2024-08-27 |title=Incidence and Prevalence of Early-Onset Dementia in Finland |url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11314947/ |journal=Neurology |volume=103 |issue=4 |pages=e209654 |doi=10.1212/WNL.0000000000209654 |issn=0028-3878 |pmid=39047214}}</ref>. Less common forms of early onset dementia include [[Lewy body dementias]] ([[dementia with Lewy bodies]] and [[Parkinson's disease dementia]]), [[Huntington's disease]], [[Creutzfeldt–Jakob disease]], [[multiple sclerosis]], alcohol-induced dementia, and other conditions.
+'''Terminology'''
+The term ''young onset dementia'' is becoming more widely used to avoid the potential confusion between ''early onset dementia'' and ''early stage dementia''<ref>{{Cite journal |last=van de Veen |first=Dennis |last2=Bakker |first2=Christian |last3=Peetoom |first3=Kirsten |last4=Pijnenburg |first4=Yolande |last5=Papma |first5=Janne |last6=de Vugt |first6=Marjolein |last7=Koopmans |first7=Raymond |date=2022-03 |title=Provisional consensus on the nomenclature and operational definition of dementia at a young age, a Delphi study |url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9305901/ |journal=International Journal of Geriatric Psychiatry |volume=37 |issue=3 |pages=10.1002/gps.5691 |doi=10.1002/gps.5691 |issn=0885-6230 |pmc=9305901 |pmid=35156239}}</ref>. Although used in the past, the term ''presenile dementia'' is no longer in favor.
 ==== Epidemiology ====
-Early onset dementia is less common than late onset dementia, accounting for approximately 10% of dementias globally<ref name=":3" />. Recent studies estimate the prevalence of early onset dementia to be approximately 3.9 million people aged 30-64 worldwide, with an incidence of 119 per 100,000 individuals<ref name=":1">{{Cite journal |last=Hendriks |first=Stevie |last2=Peetoom |first2=Kirsten |last3=Bakker |first3=Christian |last4=van der Flier |first4=Wiesje M. |last5=Papma |first5=Janne M. |last6=Koopmans |first6=Raymond |last7=Verhey |first7=Frans R. J. |last8=de Vugt |first8=Marjolein |last9=Köhler |first9=Sebastian |last10=Withall |first10=Adrienne |last11=Parlevliet |first11=Juliette L. |last12=Uysal-Bozkir |first12=Özgül |last13=Gibson |first13=Roger C. |last14=Neita |first14=Susanne M. |last15=Nielsen |first15=Thomas Rune |date=2021-09 |title=Global Prevalence of Young-Onset Dementia |url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8290331/ |journal=JAMA Neurology |volume=78 |issue=9 |pages=1–11 |doi=10.1001/jamaneurol.2021.2161 |issn=2168-6149 |pmc=8290331 |pmid=34279544}}</ref>. Additionally, studies indicate a 1:1 ratio in prevalence of EOD between males and females, with no significant difference between ethnic groups in gender distribution pattern<ref name=":4">{{Cite journal |last=Hendriks |first=Stevie |last2=Peetoom |first2=Kirsten |last3=Bakker |first3=Christian |last4=Koopmans |first4=Raymond |last5=van der Flier |first5=Wiesje |last6=Papma |first6=Janne |last7=Verhey |first7=Frans |last8=Young‐Onset Dementia Epidemiology Study Group |last9=de Vugt |first9=Marjolein |last10=Köhler |first10=Sebastian |date=2023-03 |title=Global incidence of young‐onset dementia: A systematic review and meta‐analysis |url=https://alz-journals.onlinelibrary.wiley.com/doi/10.1002/alz.12695 |journal=Alzheimer's & Dementia |language=en |volume=19 |issue=3 |pages=831–843 |doi=10.1002/alz.12695 |issn=1552-5260}}</ref><ref name=":2">{{Cite web |last=Kelly |first=BJ |last2=Boeve |first2=BF |last3=Josephs |first3=KA |date=2008 |title=Young-Onset Dementia: Demographic and Etiologic Characteristics of 235 Patients |url=https://jamanetwork.com/journals/jamaneurology/fullarticle/1107508#google_vignette |access-date=2024-10-10 |website=jamanetwork.com}}</ref>. Similar to LOD, the prevalence of EOD doubles every five years of age<ref name=":4" />. The continuous increase in prevalence with age seen in Alzheimer's and FTD versions of EOD is disproportionally led by the most common variant of each cause, namely amnesic Alzheimer's and behavioral variant of FTD<ref>{{Cite journal |last=Zamboni |first=Giovanna |last2=Maramotti |first2=Riccardo |last3=Salemme |first3=Simone |last4=Tondelli |first4=Manuela |last5=Adani |first5=Giorgia |last6=Vinceti |first6=Giulia |last7=Carbone |first7=Chiara |last8=Filippini |first8=Tommaso |last9=Vinceti |first9=Marco |last10=Pagnoni |first10=Giuseppe |last11=Chiari |first11=Annalisa |date=2024 |title=Age-specific prevalence of the different clinical presentations of AD and FTD in young-onset dementia |url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11233291/ |journal=Journal of Neurology |volume=271 |issue=7 |pages=4326–4335 |doi=10.1007/s00415-024-12364-7 |issn=0340-5354 |pmid=38643445}}</ref>.
+Early onset dementia is less common than late onset dementia, the former accounting for approximately 10% of dementias globally<ref name=":3" />. Recent studies estimate the prevalence of early onset dementia to be approximately 3.9 million people aged 30-64 worldwide, with an incidence of 119 per 100,000 individuals<ref name=":1">{{Cite journal |last=Hendriks |first=Stevie |last2=Peetoom |first2=Kirsten |last3=Bakker |first3=Christian |last4=van der Flier |first4=Wiesje M. |last5=Papma |first5=Janne M. |last6=Koopmans |first6=Raymond |last7=Verhey |first7=Frans R. J. |last8=de Vugt |first8=Marjolein |last9=Köhler |first9=Sebastian |last10=Withall |first10=Adrienne |last11=Parlevliet |first11=Juliette L. |last12=Uysal-Bozkir |first12=Özgül |last13=Gibson |first13=Roger C. |last14=Neita |first14=Susanne M. |last15=Nielsen |first15=Thomas Rune |date=2021-09 |title=Global Prevalence of Young-Onset Dementia |url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8290331/ |journal=JAMA Neurology |volume=78 |issue=9 |pages=1–11 |doi=10.1001/jamaneurol.2021.2161 |issn=2168-6149 |pmc=8290331 |pmid=34279544}}</ref>. Additionally, there is approximately a 1:1 ratio in prevalence of EOD between males and females, with no significant difference between ethnic groups in gender distribution pattern<ref name=":4">{{Cite journal |last=Hendriks |first=Stevie |last2=Peetoom |first2=Kirsten |last3=Bakker |first3=Christian |last4=Koopmans |first4=Raymond |last5=van der Flier |first5=Wiesje |last6=Papma |first6=Janne |last7=Verhey |first7=Frans |last8=Young‐Onset Dementia Epidemiology Study Group |last9=de Vugt |first9=Marjolein |last10=Köhler |first10=Sebastian |date=2023-03 |title=Global incidence of young‐onset dementia: A systematic review and meta‐analysis |url=https://alz-journals.onlinelibrary.wiley.com/doi/10.1002/alz.12695 |journal=Alzheimer's & Dementia |language=en |volume=19 |issue=3 |pages=831–843 |doi=10.1002/alz.12695 |issn=1552-5260}}</ref><ref name=":2">{{Cite web |last=Kelly |first=BJ |last2=Boeve |first2=BF |last3=Josephs |first3=KA |date=2008 |title=Young-Onset Dementia: Demographic and Etiologic Characteristics of 235 Patients |url=https://jamanetwork.com/journals/jamaneurology/fullarticle/1107508#google_vignette |access-date=2024-10-10 |website=jamanetwork.com}}</ref>. Similar to LOD, the prevalence of EOD increases exponentially with age, doubling every five years of age<ref name=":4" />. The continuous increase in prevalence with age seen in Alzheimer's and FTD versions of EOD is disproportionally led by the most common variant of each cause, namely amnesic Alzheimer's and behavioral variant of FTD<ref name=":5">{{Cite journal |last=Zamboni |first=Giovanna |last2=Maramotti |first2=Riccardo |last3=Salemme |first3=Simone |last4=Tondelli |first4=Manuela |last5=Adani |first5=Giorgia |last6=Vinceti |first6=Giulia |last7=Carbone |first7=Chiara |last8=Filippini |first8=Tommaso |last9=Vinceti |first9=Marco |last10=Pagnoni |first10=Giuseppe |last11=Chiari |first11=Annalisa |date=2024 |title=Age-specific prevalence of the different clinical presentations of AD and FTD in young-onset dementia |url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11233291/ |journal=Journal of Neurology |volume=271 |issue=7 |pages=4326–4335 |doi=10.1007/s00415-024-12364-7 |issn=0340-5354 |pmid=38643445}}</ref>.
+==== Risk factors ====
-<nowiki>*</nowiki>Results: the most common variants of both AD and FTD (amnesic AD and behavioral variant of FTD, respectively) increase disproportionally with age compared to other clinical presentations of each disease.
+Traditional risk factors for the development of late onset dementia, such as diabetes mellitus, hypertension, and obesity, have also been identified as risk factors for early onset dementia. Several other chronic conditions have recently been identified that are also associated with the development of early onset dementia, including cardiovascular, respiratory, or gastrointestinal disease<ref name=":6">{{Cite journal |last=Shang |first=Xianwen |last2=Zhu |first2=Zhuoting |last3=Zhang |first3=Xueli |last4=Huang |first4=Yu |last5=Zhang |first5=Xiayin |last6=Liu |first6=Jiahao |last7=Wang |first7=Wei |last8=Tang |first8=Shulin |last9=Yu |first9=Honghua |last10=Ge |first10=Zongyuan |last11=Yang |first11=Xiaohong |last12=He |first12=Mingguang |date=2022-03 |title=Association of a wide range of chronic diseases and apolipoprotein E4 genotype with subsequent risk of dementia in community-dwelling adults: A retrospective cohort study |url=https://pubmed.ncbi.nlm.nih.gov/35299656/ |journal=EClinicalMedicine |volume=45 |pages=101335 |doi=10.1016/j.eclinm.2022.101335 |issn=2589-5370 |pmc=8921546 |pmid=35299656}}</ref>. Significantly, the presence of one or multiple of these chronic conditions is more predictive of EOD compared to LOD<ref name=":6" />.
+==== Diagnosis ====
-'''Terminology'''
+Though widely accepted, the definition of early onset dementia as less than 65 years of age continues to be an artificial diagnostic criterion based on the traditional retirement age in most countries<ref name=":0" />. Nevertheless, the purpose of having a numerical age cut-off is evidenced in the significant differences in the etiology and prognosis of dementia depending on the age category of the patient. Furthermore, the diagnosis of early onset dementia continues to be challenging due to the wide range of symptoms at presentation and propensity not to consider neurodegenerative causes in this population. Recent studies indicate an average of 4.4 years time to diagnosis for EOD, compared to 2.8 years for LOD<ref name=":4" />.
+==== Disease course ====
-The term ''young onset dementia'' is becoming more widely used to avoid the potential confusion between ''early onset dementia'' and ''early stage dementia''<ref>{{Cite journal |last=van de Veen |first=Dennis |last2=Bakker |first2=Christian |last3=Peetoom |first3=Kirsten |last4=Pijnenburg |first4=Yolande |last5=Papma |first5=Janne |last6=de Vugt |first6=Marjolein |last7=Koopmans |first7=Raymond |date=2022-03 |title=Provisional consensus on the nomenclature and operational definition of dementia at a young age, a Delphi study |url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9305901/ |journal=International Journal of Geriatric Psychiatry |volume=37 |issue=3 |pages=10.1002/gps.5691 |doi=10.1002/gps.5691 |issn=0885-6230 |pmc=9305901 |pmid=35156239}}</ref>. Although used in the past, the term ''presenile dementia'' is no longer in favor.
+Compared to late onset dementia, patients with early onset dementia are more likely to have dementias other than Alzheimer's disease, although Alzheimer's is the most common etiology in either case<ref name=":0">{{Cite journal |last=Vieira |first=Renata Teles |last2=Caixeta |first2=Leonardo |last3=Machado |first3=Sergio |last4=Silva |first4=Adriana Cardoso |last5=Nardi |first5=Antonio Egidio |last6=Arias-Carrión |first6=Oscar |last7=Carta |first7=Mauro Giovanni |date=2013-06-14 |title=Epidemiology of early-onset dementia: a review of the literature |url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3715758/ |journal=Clinical Practice and Epidemiology in Mental Health : CP & EMH |volume=9 |pages=88–95 |doi=10.2174/1745017901309010088 |issn=1745-0179 |pmc=3715758 |pmid=23878613}}</ref>. In general, EOD has a faster progression and features more extensive neurological damage when compared to LOD. It is hypothesized that this may be due to decreased cognitive reserve seen in late onset dementias, causing greater complication relative to pathological damage<ref name=":0" />. Furthermore, studies have shown differences in the areas of cognition that are likely to be affected when comparing early onset to late onset dementia. In terms of behavioral symptoms, EOD is more likely to affect attention, but less likely to cause confusion, delusions, hallucinations, agitation, or disinhibition. In terms of motor symptoms, EOD is less likely to affect verbal fluency and motor executive function compared to late onset dementia<ref name=":0" />.
-==== Early v. late-onset dementia ====
+==== Prognosis ====
+Estimation of survival rate in early onset dementias is a component patient prognosis, management, and treatment. In general, a better prognosis is positively correlated with earlier age of onset<ref name=":0" />. Average survival time is approximately 6-10 years following diagnosis for both men and women, with variability depending on specific type of dementia<ref name=":4" /><ref>{{Cite journal |last=Kay |first=David W. K. |last2=Forster |first2=Donald P. |last3=Newens |first3=Andrew J. |date=2000-08 |title=Long-term survival, place of death, and death certification in clinically diagnosed pre-senile dementia in northern England: Follow-up after 8–12 years |url=https://www.cambridge.org/core/product/identifier/S0007125000155394/type/journal_article |journal=British Journal of Psychiatry |language=en |volume=177 |issue=2 |pages=156–162 |doi=10.1192/bjp.177.2.156 |issn=0007-1250}}</ref>. The most common cause of immediate death in EOD is respiratory disease (e.g. pneumonia); other causes include cardiovascular events and cerebrovascular disease<ref name=":0" />.
-Compared to late onset dementia, patients with early onset dementia are more likely to have dementias other than Alzheimer's disease, though Alzheimer's is the most common etiology in either case<ref name=":0">{{Cite journal |last=Vieira |first=Renata Teles |last2=Caixeta |first2=Leonardo |last3=Machado |first3=Sergio |last4=Silva |first4=Adriana Cardoso |last5=Nardi |first5=Antonio Egidio |last6=Arias-Carrión |first6=Oscar |last7=Carta |first7=Mauro Giovanni |date=2013-06-14 |title=Epidemiology of early-onset dementia: a review of the literature |url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3715758/ |journal=Clinical Practice and Epidemiology in Mental Health : CP & EMH |volume=9 |pages=88–95 |doi=10.2174/1745017901309010088 |issn=1745-0179 |pmc=3715758 |pmid=23878613}}</ref>. In general, EOD has a faster progression and features more extensive neurological damage when compared to LOD. It is hypothesized that this may be due to decreased cognitive reserve seen in late onset dementias, causing greater complication relative to pathological damage<ref name=":0" />. Furthermore, studies have shown differences in the areas of cognition that are likely to be affected when comparing early onset to late onset dementia. In terms of behavioral symptoms, EOD is more likely to affect attention, but less likely to cause confusion, delusions, hallucinations, agitation, or disinhibition. In terms of motor symptoms, EOD is less likely to affect verbal fluency and motor executive function compared to late onset dementia<ref name=":0" />. Additionally, recent studies indicate differences in the risk factors for development of early onset compared to late onset dementia. Specifically, diabetes mellitus and osteoporosis are risk factors for development of both EOD and LOD, whereas hypertension increases risk of EOD only<ref name=":5">{{Cite journal |last=Chun |first=Min Young |last2=Chae |first2=Wonjeong |last3=Seo |first3=Sang Won |last4=Jang |first4=Hyemin |last5=Yun |first5=Jihwan |last6=Na |first6=Duk L. |last7=Kang |first7=Dongwoo |last8=Lee |first8=Jungkuk |last9=Hammers |first9=Dustin B. |last10=Apostolova |first10=Liana G. |last11=Jang |first11=Sung-In |last12=Kim |first12=Hee Jin |date=2024-04-25 |title=Effects of risk factors on the development and mortality of early- and late-onset dementia: an 11-year longitudinal nationwide population-based cohort study in South Korea |url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11044300/ |journal=Alzheimer's Research & Therapy |volume=16 |pages=92 |doi=10.1186/s13195-024-01436-5 |issn=1758-9193 |pmid=38664771}}</ref>. The presence of diabetes mellitus increases risk of mortality in both EOD and LOD<ref name=":5" />.
 '''Specific types of early onset dementia'''
-Studies indicate that family history is a significant risk factor for specifically Alzheimer's early onset dementia<ref name=":2" />.
+===== Alzheimer's early onset dementia =====
+Alzheimer's disease is the most common cause of both early and late onset dementia, and continuously increases in prevalence with age<ref name=":5" />. Studies indicate that family history is a significant risk factor for specifically Alzheimer's early onset dementia<ref name=":2" />.
 Though not a major cause of early onset dementia, TBIs are an important cause of EOD. Young individuals, especially males, are more likely to be part of this population group.
-==== Diagnosis ====
-Though widely accepted, the definition of early onset dementia as less than 65 years of age continues to be an artificial diagnostic criterion based on the traditional retirement age in most countries<ref name=":0" />. Nevertheless, the purpose of having a numerical age cut-off is evidenced in the significant differences in the etiology and prognosis of dementia depending on the age category of the patient. Furthermore, the diagnosis of early onset dementia continues to be challenging due to the wide range of symptoms at presentation and propensity not to consider neurodegenerative causes in this population. Consequently, there is a 4.4 year time to diagnosis for EOD, compared to 2.8 years for LOD<ref name=":4" />.
-==== Prognosis ====
-Estimation of survival rate in early onset dementias is a component patient prognosis, management, and treatment. In general, a better prognosis is positively correlated with earlier age of onset<ref name=":0" />. Average survival time is approximately 6-10 years following diagnosis for both men and women, with variability depending on specific type of dementia<ref name=":4" /><ref>{{Cite journal |last=Kay |first=David W. K. |last2=Forster |first2=Donald P. |last3=Newens |first3=Andrew J. |date=2000-08 |title=Long-term survival, place of death, and death certification in clinically diagnosed pre-senile dementia in northern England: Follow-up after 8–12 years |url=https://www.cambridge.org/core/product/identifier/S0007125000155394/type/journal_article |journal=British Journal of Psychiatry |language=en |volume=177 |issue=2 |pages=156–162 |doi=10.1192/bjp.177.2.156 |issn=0007-1250}}</ref>. The most common cause of immediate death in EOD is respiratory disease (e.g. pneumonia); other causes include cardiovascular events and cerebrovascular disease<ref name=":0" />.
 === References ===