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Postgraduate Medical Journal 2004;80:125-139
© 2004 Fellowship of Postgraduate Medicine


REVIEW

Young onset dementia

E L Sampson 1, J D Warren 1, M N Rossor 2

1 Dementia Research Group, Institute of Neurology, University College London, London, UK
2 Dementia Research Group, Institute of Neurology, University College London and Division of Neurosciences, Faculty of Medicine, Imperial College, London, UK

Correspondence to:
Correspondence to:
Professor Martin Rossor
Dementia Research Group, National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK; m.rossor{at}dementia.ion.ucl.ac.uk

Young onset dementia is a challenging clinical problem with potentially devastating medical and social consequences. The differential diagnosis is wide, and includes a number of rare sporadic and hereditary diseases. However, accurate diagnosis is often possible, and all patients should be thoroughly investigated to identify treatable processes. 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.


Keywords: young onset dementia; presenile dementia; Alzheimer’s disease; frontotemporal lobar degeneration

Abbreviations: BSE, bovine spongiform encephalopathy; CADASIL, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy; CJD, Creutzfeldt-Jakob disease; FLAIR, fluid attenuated inversion recovery; FTDP-17, frontotemporal dementia-parkinsonism linked to chromosome 17; FTLD, frontotemporal lobar degeneration; MRI, magnetic resonance imaging; nvCJD, new variant Creutzfeldt-Jakob disease; PrP, prion protein; PS, presenilin; YOD, young onset dementia




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