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Dement Geriatr Cogn Disord 2009;27:564–571

Cognitive and Noncognitive Neurological Features of Young-Onset Dementia

Kelley B.J. · Boeve B.F. · Josephs K.A.
Department of Neurology, Mayo Clinic, Rochester, Minn., USA
email Corresponding Author

 goto top of outline Key Words

  • Young-onset dementia
  • Cognitive decline, age of onset
  • Presenile dementia, clinical features

 goto top of outline Abstract

Background: The rarity of young-onset dementia (YOD), the broad differential diagnosis and unusual clinical presentations present unique challenges to correctly recognize the condition and establish an accurate diagnosis. Limited data exist regarding clinical features associated with dementia prior to the age of 45. Methods: We retrospectively assessed cognitive and noncognitive neurological characteristics of 235 patients who presented for evaluation of YOD to investigate the clinical characteristics of YOD compared to later-onset dementias and to identify clinical features associated with specific etiologies that may aid in the evaluation of YOD. Results: Multiple cognitive domains were affected in most patients, and no significant differences in affected domains existed between groups. Early psychiatric and behavioral features occurred at very high frequencies. Nearly 80% of this YOD cohort had additional noncognitive symptoms or signs as a feature of their disease. Chorea was strongly associated with Huntington disease. Parkinsonism was not seen in patients having an autoimmune/inflammatory etiology. Conclusions: The rarity of YOD and the high frequency of early psychiatric features led to frequent misdiagnosis early in the clinical course. The high frequency of noncognitive symptoms and signs may aid clinicians in distinguishing patients requiring a more extensive evaluation for YOD.

Copyright © 2009 S. Karger AG, Basel

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 goto top of outline Author Contacts

Brendan J. Kelley, MD
Department of Neurology, University of Cincinnati
260 Stetson Street, Suite 2300
Cincinnati, OH 45267 (USA)
Tel. +1 513 558 5501, Fax +1 513 558 4305, E-Mail

 goto top of outline Article Information

Accepted: March 16, 2009
Published online: July 11, 2009
Number of Print Pages : 8
Number of Figures : 0, Number of Tables : 5, Number of References : 21

 goto top of outline Publication Details

Dementia and Geriatric Cognitive Disorders

Vol. 27, No. 6, Year 2009 (Cover Date: July 2009)

Journal Editor: Chan-Palay V. (New York, N.Y.)
ISSN: 1420-8008 (Print), eISSN: 1421-9824 (Online)

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