Rapidly progressive young-onset dementia

H. Robert, Clarice Smith, Abigail Van Buren Alzheimer's, Brendan J. Kelley, Bradley F. Boeve, Keith A. Josephs

Research output: Contribution to journalArticle

18 Scopus citations

Abstract

Objective: To characterize a cohort of individuals who have experienced rapidly progressive dementia with onset before age 45. Background: Very little data regarding the clinical features or clinical spectrum of rapidly progressive young-onset dementia (RP-YOD) is available, primarily consisting of case reports or small series. Methods: A search of the Mayo Clinic medical record was employed to identify patients who had onset before age 45 of rapidly progressive dementia. All available medical records, laboratory data, neuroimaging studies, and pathologic data were reviewed. Results: Twenty-two patients met the predefined inclusion and exclusion criteria. Behavioral and affective disorders, cerebellar dysfunction, and visual and/or oculomotor dysfunction were common early clinical features within the cohort, as were clinical features often associated with Creutzfeldt-Jakob disease. Diagnostic testing identified an etiology in most patients. Conclusions: Presentations of RP-YOD result from a variety of etiologies and significant overlap in clinical features is observed. Clinical features often associated with Creutzfeldt-Jakob disease seem to be common within the entire cohort of RP-YOD patients. Diagnostic studies aided in establishing a diagnosis in most patients, however 5 had uncertain diagnoses despite exhaustive evaluation.

Original languageEnglish (US)
Pages (from-to)22-27
Number of pages6
JournalCognitive and Behavioral Neurology
Volume22
Issue number1
DOIs
StatePublished - Mar 1 2009

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Keywords

  • Creutzfeldt-Jakob disease
  • Presenile dementia
  • Rapidly progressive dementia
  • Young-onset
  • Young-onset dementia

ASJC Scopus subject areas

  • Neuropsychology and Physiological Psychology
  • Cognitive Neuroscience
  • Psychiatry and Mental health

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