Abstract
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.
Keywords
young-onset dementia - early-onset dementia - neurodegenerative disease - dementia/reversible
