Cognitive and noncognitive neurological features of young-onset dementia

Research output: Contribution to journalArticle

14 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)564-571
Number of pages8
JournalDementia and Geriatric Cognitive Disorders
Volume27
Issue number6
DOIs
StatePublished - Jul 2009

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Dementia
Signs and Symptoms
Psychiatry
Chorea
Huntington Disease
Parkinsonian Disorders
Diagnostic Errors
Differential Diagnosis

Keywords

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

ASJC Scopus subject areas

  • Psychiatry and Mental health
  • Geriatrics and Gerontology
  • Cognitive Neuroscience

Cite this

Cognitive and noncognitive neurological features of young-onset dementia. / Kelley, Brendan J.; Boeve, Bradley F; Josephs, Keith Anthony.

In: Dementia and Geriatric Cognitive Disorders, Vol. 27, No. 6, 07.2009, p. 564-571.

Research output: Contribution to journalArticle

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