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Semantic Dementia: Losing the Meaning of Everything
  1. Jonathan A. Knibb*,
  2. John R. Hodges
  1. *Research Fellow, University of Cambridge Neurology Unit;
  2. Professor in Behavioural Neurology, University of Cambridge Neurology Unit, and Addenbrooke’s Hospital and MRC Cognition and Brain Sciences Unit, Cambridge, UK; E-mail: john.hodges{at}mrc-cbu.cam.ac.uk

    Abstract

    INTRODUCTION

    Semantic dementia is one form of fronto-temporal dementia (or ‘fronto-temporal lobar degeneration’). Fronto-temporal dementia is the second most common cause of dementia in people under 65 years of age, the prevalence in a recent Cambridge series being about 15/100 000; semantic dementia accounts for a substantial minority of these cases. Over the past decade we have seen 75 patients with this condition. We will describe a typical case, focusing on the features of clinical interest; first, the initial presentation and language impairments, followed by the progression of the case and the impairments in other cognitive domains, and concluding with a summary of the neuropathology and prognosis.

    A TYPICAL CASE OF SEMANTIC DEMENTIA: FIRST PRESENTATION

    This lady was in her fifties when she presented to the Cambridge Memory Clinic complaining of ‘memory problems,’ which had been gradual in onset and progressively worsening over the previous 12 months. The first problem

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