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Semin Neurol 2013; 33(04): 365-385
DOI: 10.1055/s-0033-1359320
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Young-Onset Dementia

Dulanji K. Kuruppu
1   Indiana University School of Medicine, Indianapolis, Indiana
,
Brandy R. Matthews
2   Department of Neurology, Indiana University School of Medicine, Indianapolis, Indiana
› Author Affiliations
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Publication History

Publication Date:
14 November 2013 (online)

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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