Article

The diagnosis of young-onset dementia. Lancet Neurol 9:793-806

Dementia Research Centre, Department of Neurodegeneration, UCL Institute of Neurology, Queen Square, London, UK.
The Lancet Neurology (Impact Factor: 21.82). 08/2010; 9(8):793-806. DOI: 10.1016/S1474-4422(10)70159-9
Source: PubMed

ABSTRACT A diagnosis of dementia is devastating at any age but diagnosis in younger patients presents a particular challenge. The differential diagnosis is broad as late presentation of metabolic disease is common and the burden of inherited dementia is higher in these patients than in patients with late-onset dementia. The presentation of the common degenerative diseases of late life, such as Alzheimer's disease, can be different when presenting in the fifth or sixth decade. Moreover, many of the young-onset dementias are treatable. The identification of causative genes for many of the inherited degenerative dementias has led to an understanding of the molecular pathology, which is also applicable to later-onset sporadic disease. This understanding offers the potential for future treatments to be tailored to a specific diagnosis of both young-onset and late-onset dementia.

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Available from: Cath Mummery, Dec 12, 2014
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    • "Although causes of EOD are diverse, the four most common causes are Alzheimer's disease (AD), vascular dementia (VAD), frontotemporal dementia (FTD), and dementia with Lewy bodies (DLB); responsible for 34, 18, 12, and 7 % of cases, respectively (Harvey et al. 2003). Dementia secondary to genetic or metabolic disease is more common in patients with early-onset disease (Rossor et al. 2010). Alzheimer's disease presents as a progressive amnestic disorder which evolves to affect other cognitive domains. "
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    ABSTRACT: Early-onset dementia (EOD) is characterized by functionally impairing deterioration in memory, language, personality or visuospatial skills emerging under the age of 65. Cerebral functioning can be assessed by visual electroencephalography (EEG) interpretation. The aim of this systematic review is to evaluate the diagnostic utility of visual EEG in EOD focusing on Alzheimer's disease (AD), vascular dementia (VAD), dementia with Lewy bodies (DLB), and frontotemporal dementia (FTD). Medline, Embase, Scopus, Web of Knowledge, and Google Scholar were systematically searched for studies where EEGs were included in the diagnostic evaluation of patients with dementia under the age of 65. Each paper was quality assessed and the results grouped according to dementia cause with a narrative summary. 4,157 papers were screened, 12 studies met the eligibility criteria with a total of 965 patients. An abnormal EEG was common to all causes of EOD. EEG abnormalities are more severe in early-onset AD patients. EEG severity grade is independent of disease duration. Slow wave activity is common to all dementias, but is most prominent in DLB. Frontal intermittent rhythmic delta activity could be considered as supportive for the diagnosis of DLB as can a Grand Total EEG score of over 9.5. EEG is usually normal in FTD. Focal changes can be seen in advanced VAD. Studies employed small patient groups, varying diagnostic criteria, and only a minority of patient diagnoses was pathologically confirmed. EEG may be useful as an adjunct in the diagnosis of DLB and AD. Further prospective well-powered studies are required to investigate diagnostic utility more robustly.
    Journal of Neural Transmission 07/2013; 121(1). DOI:10.1007/s00702-013-1070-5 · 2.87 Impact Factor
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    • "Les « syndrome démentiels plus » qui associent un syndrome démentiel et des signes de focalisation neurologiques et/ou systémiques peuvent correspondre à des pathologies neurodégénératives ou métaboliques (Tableaux I et II), le plus souvent héréditaires de type autosomique récessif (Rossor et al., 2010). Parmi les pathologies métaboliques, les anomalies de la chaîne respiratoire par mutation de l'ADN mitochondrial ou nucléaire représenteraient environ la moitié des causes de démence de l'adulte avant 45 ans (Kelley et al., 2008). "
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    ABSTRACT: In the international literature, early onset dementia are defined by the first signs before the age of 65. In France, the Alzheimer plan determined the threshold to 60 years, which is the age for access to certain welfare benefits or accommodation facilities. Today, the number of patients in France with a dementia before the age of 65 is estimated to 32,000 as reported by the Office Parlementaire d’Évaluation des Politiques de Santé (OPEPS) report in 2005. The prevalence of dementia before the age of 65 in population studies is 80 over 100 000 on average. The causes are more numerous and the clinical presentations are more atypical compared to dementia in the elderly, including Alzheimer's disease with a predominance of instrumental difficulties. More the age of onset is earlier, more genetic and metabolic causes, potentially treatable, are common. They are expressed most often by a “dementia plus syndrome”, requiring a detailed clinical evaluation. On the medico-social, aid granted to young patients are specific and include the Prestation de Compensation du Handicap (PCH), which can be obtained from the Maison Départementale des Personnes Handicapées (MDPH), a reference to the administrative procedures of young patients with dementia.
    Pratique Neurologique - FMC 09/2012; 3(3):185–195. DOI:10.1016/j.praneu.2012.07.001
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    • "Therefore, our recommendations are aimed at promoting public awareness of YOD as well as increasing knowledge among the medical profession. In addition, the development and/or enhancement of specialized diagnostic centres for the diagnosis of YOD are important , because a structured approach based on all clinical features is required to diagnose YOD (Rossor et al. 2010). These centres may in turn increase awareness of this subgroup in primary care (Rosness et al. 2008). "
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    ABSTRACT: BACKGROUND: The extent to which specific factors influence diagnostic delays in dementia is unclear. Therefore, the aim of the present study was to compare duration from symptom onset to diagnosis for young-onset dementia (YOD) and late-onset dementia (LOD) and to assess the effect of age at onset, type of dementia, gender, living situation, education and family history of dementia on this duration.Method Data on 235 YOD and 167 LOD patients collected from caregivers from two prospective cohort studies were used. Multiple linear regression analysis was performed. RESULTS: The duration between symptom onset and the diagnosis of YOD exceeded that of LOD by an average of 1.6 years (2.8 v. 4.4 years). Young age and being diagnosed with frontotemporal dementia were related to increases in the time to diagnosis. Subjects with vascular dementia experienced shorter time to diagnosis. CONCLUSIONS: There is a need to raise special awareness of YOD to facilitate a timely diagnosis.
    Psychological Medicine 05/2012; 43(2):1-10. DOI:10.1017/S0033291712001122 · 5.43 Impact Factor
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