![]() ![]() The young onset forms of these diseases are frequently familial. While the degenerative dementias characteristically affect older patients, they are also an important cause of YOD: indeed, Alzheimer’s disease is the commonest single cause of YOD with an estimated 3000 cases in the United Kingdom, followed by vascular dementia and the frontotemporal lobar degenerations (table 1). YOD poses a diagnostic challenge and may present with a wide variety of subtle behavioural, cognitive, psychiatric, or neurological symptoms. 1 Prevalence rates of YOD have been estimated between 67 to 81 per 100 000 in the 45 to 65 year old age group 2, 3 thus there are currently approximately 10 000 patients with YOD in the United Kingdom alone. nvCJD, new variant Creutzfeldt-Jakob diseaseĭementia in younger people (young onset dementia, YOD) is increasingly recognised as an important clinical and social problem, with frequently devastating consequences for both the sufferer and those who care for them.FTLD, frontotemporal lobar degeneration.FTDP-17, frontotemporal dementia-parkinsonism linked to chromosome 17.FLAIR, fluid attenuated inversion recovery.CADASIL, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy.This review presents an approach to the diagnosis, investigation, and management of patients with young onset dementia, with particular reference to common and treatable causes. However, accurate diagnosis is often possible, and all patients should be thoroughly investigated to identify treatable processes. The differential diagnosis is wide, and includes a number of rare sporadic and hereditary diseases. Young onset dementia is a challenging clinical problem with potentially devastating medical and social consequences. ![]()
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