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Psych TopicsOld age psychiatry — neurocognitive disorders

Psych · Old age psychiatry — neurocognitive disorders

Young-onset dementia

Also known as Early-onset dementia · YOD · EOD · Presenile dementia (historical) · Early-onset Alzheimer disease · Working-age dementia

Exam-exhaustive fellowship reference on young-onset dementia (onset before 65): definitions, Hendriks/Harvey epidemiology, aetiologic spectrum (EOAD, FTD, vascular, alcohol, rapidly progressive, genetic-metabolic), dementia-plus signs, psychiatric misdiagnosis, structured work-up, APP/PSEN1/PSEN2 and FTD genetics, aetiology-directed treatment including donepezil when AD, antipsychotic caution, capacity, employment, and dependent children. FRANZCP-primary, globally tagged.

medium16 referencesUpdated 26 July 202615 min readVerification in progress

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FRANZCPMRCPsychABPNMD-DNBNEET-SS

Red flags

  • Progressive cognitive or behavioural decline before age 65 — do not close as depression, burnout, or 'midlife crisis' without structured neurocognitive work-up
  • Rapidly progressive course (weeks–months) — prioritise treatable and prion/autoimmune pathways urgently
  • Dementia-plus signs: myoclonus, MND features, recurrent young strokes, Kayser–Fleischer rings, chorea, ophthalmoplegia — expand genetics and treatable differentials
  • Strong family history of early dementia, ALS, or young stroke — genetic counselling before casual reassurance
  • Workplace, driving, or child-care safety failures — capacity and safeguarding, not only a cognitive score
  • New behavioural disinhibition or late-onset psychosis phenotype in midlife — consider FTD/C9orf72 spectrum before primary psychiatric closure
  • Alcohol use with confusion — parenteral thiamine before glucose when Wernicke risk present
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Your progress

Saved on this device.

Practise this topic10 MCQs with explanations

Target exams

FRANZCPMRCPsychABPNMD-DNBNEET-SS

Red flags

  • Progressive cognitive or behavioural decline before age 65 — do not close as depression, burnout, or 'midlife crisis' without structured neurocognitive work-up
  • Rapidly progressive course (weeks–months) — prioritise treatable and prion/autoimmune pathways urgently
  • Dementia-plus signs: myoclonus, MND features, recurrent young strokes, Kayser–Fleischer rings, chorea, ophthalmoplegia — expand genetics and treatable differentials
  • Strong family history of early dementia, ALS, or young stroke — genetic counselling before casual reassurance
  • Workplace, driving, or child-care safety failures — capacity and safeguarding, not only a cognitive score
  • New behavioural disinhibition or late-onset psychosis phenotype in midlife — consider FTD/C9orf72 spectrum before primary psychiatric closure
  • Alcohol use with confusion — parenteral thiamine before glucose when Wernicke risk present
The one-line answer

Young-onset dementia is dementia with symptom onset before 65 — an age-of-onset construct that does not invent a new disease but rewrites the prior probabilities: frontotemporal dementia, atypical and monogenic Alzheimer disease, alcohol-related injury and treatable rarities climb the list while typical late-onset amnestic AD slides down. Take the pedigree every time, hunt the dementia-plus signs that demand a wider work-up, image with MRI first, and treat the person in their working life — job, mortgage, dependent children, driving — not as a misplaced aged-care patient.[1][2][4][12]

Meet the patient

A 56-year-old primary-school principal is referred to psychiatry by her GP as "burnout, won't engage with CBT". For eighteen months she has made impulsive, out-of-character purchases, told crude jokes in front of parents, and put on a stone eating biscuits by the packet. Her husband says the woman he married "went somewhere" two years ago.[1][11]

She scores 29 out of 30 on a cognitive screen. The GP took that as reassurance. She is still driving, still doing the school banking, and last week left the gas on.[1][7]

Two exam questions are now live. Why is 29 out of 30 not reassurance here? And why is the behavioural change the finding, not the score? Everything below answers both at consultant depth.[1][7][11]

History nugget, and it scores marks: the old word was presenile dementia, a label that did real harm by implying these were just younger cases of "ordinary" senile decline. Rossor's 2010 framing replaced it — under-65 onset is a different mix, not the same disease at a younger age.[1]

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References16ShowHide
  1. [1]Rossor MN, Fox NC, Mummery CJ, Schott JM, Warren JD The diagnosis of young-onset dementia Lancet Neurol, 2010.PMID 20650401
  2. [2]Hendriks S, Peetoom K, Bakker C, et al. Global Prevalence of Young-Onset Dementia: A Systematic Review and Meta-analysis JAMA Neurol, 2021.PMID 34279544
  3. [3]Harvey RJ, Skelton-Robinson M, Rossor MN The prevalence and causes of dementia in people under the age of 65 years J Neurol Neurosurg Psychiatry, 2003.PMID 12933919
  4. [4]Kuruppu DK, Matthews BR Young-onset dementia Semin Neurol, 2013.PMID 24234358
  5. [5]Masellis M, Sherborn K, Neto P, et al. Early-onset dementias: diagnostic and etiological considerations Alzheimers Res Ther, 2013.PMID 24565469
  6. [6]McKhann GM, Knopman DS, Chertkow H, et al. The diagnosis of dementia due to Alzheimer's disease: recommendations from the National Institute on Aging-Alzheimer's Association workgroups on diagnostic guidelines for Alzheimer's disease Alzheimers Dement, 2011.PMID 21514250
  7. [7]Rascovsky K, Hodges JR, Knopman D, et al. Sensitivity of revised diagnostic criteria for the behavioural variant of frontotemporal dementia Brain, 2011.PMID 21810890
  8. [8]McKeith IG, Boeve BF, Dickson DW, et al. Diagnosis and management of dementia with Lewy bodies: Fourth consensus report of the DLB Consortium Neurology, 2017.PMID 28592453
  9. [9]Sherrington R, Rogaev EI, Liang Y, et al. Cloning of a gene bearing missense mutations in early-onset familial Alzheimer's disease Nature, 1995.PMID 7596406
  10. [10]Goate A, Chartier-Harlin MC, Mullan M, et al. Segregation of a missense mutation in the amyloid precursor protein gene with familial Alzheimer's disease Nature, 1991.PMID 1671712
  11. [11]Woolley JD, Khan BK, Murthy NK, et al. The diagnostic challenge of psychiatric symptoms in neurodegenerative disease: rates of and risk factors for prior psychiatric diagnosis in patients with frontotemporal dementia J Clin Psychiatry, 2011.PMID 21382304
  12. [12]Arvanitakis Z, Shah RC, Bennett DA Diagnosis and Management of Dementia: Review JAMA, 2019.PMID 31638686
  13. [13]Birks JS, Harvey RJ Donepezil for dementia due to Alzheimer's disease Cochrane Database Syst Rev, 2018.PMID 29923184
  14. [14]Schneider LS, Dagerman KS, Insel P Risk of death with atypical antipsychotic drug treatment for dementia: meta-analysis of randomized placebo-controlled trials JAMA, 2005.PMID 16234500
  15. [15]Appelbaum PS Clinical practice. Assessment of patients' competence to consent to treatment N Engl J Med, 2007.PMID 17978292
  16. [16]Apostolova LG, Aisen P, Eloyan A, et al. The Longitudinal Early-onset Alzheimer's Disease Study (LEADS): Framework and methodology Alzheimers Dement, 2021.PMID 34018654

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