Young-Onset Dementia Demographic and Etiologic Characteristics of 235 Patients

Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA.
Archives of neurology (Impact Factor: 7.01). 11/2008; 65(11):1502-8. DOI: 10.1001/archneur.65.11.1502
Source: PubMed

ABSTRACT Onset of dementia before age 45 years presents a difficult clinical circumstance, having a broad differential diagnosis and numerous psychosocial implications for the patient and their family. Few data exist regarding the demographics characterizing this population or the etiologic diagnoses among those affected.
To characterize the demographic characteristics and the etiologic causes of dementia with age at onset younger than 45 years.
Observational, retrospective, single-cohort study.
Multispecialty group academic medical center.
We searched the Mayo Clinic Rochester electronic Medical Record Linkage System to identify individuals who were seen for evaluation of progressive cognitive decline between the ages of 17 and 45 years from January 1996 through December 2006. This search identified 235 individuals who met the established inclusion and exclusion criteria.
All available clinical, laboratory, magnetic resonance imaging, and pathological data were reviewed.
Causes varied, with neurodegenerative etiologies accounting for 31.1% of the cohort; Alzheimer disease was uncommon. Autoimmune or inflammatory causes accounted for 21.3%. At last follow-up, 44 patients (18.7%) had an unknown etiology, despite exhaustive evaluation. Cause varied with age, with inborn errors of metabolism being more common before age 30 years and with neurodegenerative etiologies being more common after age 35 years.
Young-onset dementia (age at onset, <45 years) includes a broad variety of etiologies, with few patients having a potentially treatable disorder. The etiologic spectrum and the relative percentages of patients within etiologic groups differed in important ways from existing reports of early-onset dementia (ie, age at onset, <65 years).

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    ABSTRACT: Background/Aims: Patients with early-onset dementia (EOD) often present atypically, making an accurate diagnosis difficult. Single-photon emission-computed tomography (SPECT) provides an indirect measure of cerebral metabolic activity and can help to differentiate between dementia subtypes. This study aims to investigate the clinical utility of SPECT imaging in the diagnosis of early-onset Alzheimer's disease. Methods: All patients attending a tertiary referral clinic specialising in EOD between April 2012 and October 2013 were included in the study. Statistical analysis of SPECT patterns with clinical diagnoses, Addenbrooke's Cognitive Examination version 3 scores, and magnetic resonance imaging (MRI) atrophy was undertaken. Results: The results demonstrated a highly significant association between SPECT hypoperfusion patterns and clinical diagnoses. SPECT changes were demonstrated more frequently than MRI atrophy. Conclusions: The results suggest that SPECT imaging may be a useful adjunct to clinical evaluation and a more sensitive biomarker than standard structural imaging. © 2015 S. Karger AG, Basel.
    Dementia and Geriatric Cognitive Disorders 01/2015; 39(3-4):186-193. DOI:10.1159/000369551 · 2.81 Impact Factor
  • International Psychogeriatrics 12/2014; 26(12):1931-3. DOI:10.1017/S1041610214001574 · 1.89 Impact Factor
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    ABSTRACT: ABSTRACT Background: Service planning for people with younger onset dementia (YOD; an onset of symptoms before the age of 65 years) relies on prevalence estimates, with existing models based upon older people. This pilot study investigated the prevalence and causes of YOD in a defined catchment area of Eastern Sydney, Australia. Methods: The study was conducted in three stages: publicity building, case finding, and case validation. A brief structured questionnaire was sent to health professionals in the catchment area asking how many patients with YOD they had seen over the previous 12 months. Memory clinics and hospital records were also searched for YOD patients. Clinicians assigned a Statistical Linkage Key to each patient to prevent double counting, and indicated the cause of dementia. The majority of patients were validated by a review of medical case notes. Prevalence data were calculated for the following age groups: 30-64, 30-44, and 45-64 years. Results: Two hundred and four potential patients were identified, of which 141 met inclusion criteria. The primary clinical subtypes were alcohol-related dementia (18.4%), Alzheimer's disease (17.7%), vascular dementia (12.8%), and frontotemporal dementia (11.3%). Eighty-eight patients were aged 30 to 64 years on census date and were therefore included in the prevalence calculations. The overall prevalence was 68.2 per 100,000 population at risk for the 30-64-year age group (95% Confidence Interval (CI): 54.9-83.4); 11.6 per 100,000 for the 30-44-year age group (95% CI: 5.3-21.7); and 132.9 per 100,000 for the 45-64 age group (95% CI: 105.8-164.2). Conclusions: Younger onset dementia affects a significant number of people in Eastern Sydney with a diverse range of clinical types. This prevalence rate is higher than previous reports from the United Kingdom and Japan, with a different distribution of etiologies, which have important implications for service planning for this group.
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Brad Boeve