Young-onset dementia

Semin Neurol. 2013 Sep;33(4):365-85. doi: 10.1055/s-0033-1359320. Epub 2013 Nov 14.


Young-onset dementia is a neurologic syndrome that affects behavior and cognition of patients younger than 65 years of age. Although frequently misdiagnosed, a systematic approach, reliant upon attainment of a detailed medical history, a collateral history, neuropsychological testing, laboratory studies, and neuroimaging, may facilitate earlier and more accurate diagnosis with subsequent intervention. The differential diagnosis of young-onset dementia is extensive and includes early-onset forms of adult neurodegenerative conditions including Alzheimer's disease, vascular dementia, frontotemporal dementia, Lewy body dementias, Huntington's disease, and prion disease. Late-onset forms of childhood neurodegenerative conditions may also present as young-onset dementia and include mitochondrial disorders, lysosomal storage disorders, and leukodystrophies. Potentially reversible etiologies including inflammatory disorders, infectious diseases, toxic/metabolic abnormalities, transient epileptic amnesia, obstructive sleep apnea, and normal pressure hydrocephalus also represent important differential diagnostic considerations in young-onset dementia. This review will present etiologies, diagnostic strategies, and options for management of young-onset dementia with comprehensive summary tables for clinical reference.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Age of Onset*
  • Dementia* / diagnosis
  • Dementia* / etiology
  • Dementia* / physiopathology
  • Dementia* / therapy
  • Humans
  • Neurodegenerative Diseases* / diagnosis
  • Neurodegenerative Diseases* / etiology
  • Neurodegenerative Diseases* / physiopathology
  • Neurodegenerative Diseases* / therapy