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Shoumitro Deb

Imperial College London, UK

Title: Dementia in down syndrome

Biography

Biography: Shoumitro Deb

Abstract

People with Down Syndrome (DS) are at a higher risk of developing Alzheimer’s Dementia at an early age compared with people without intellectual (learning) disabilities (ID). However, there are difficulties in making an early and accurate diagnosis of dementia in individuals with ID and the screening instruments such as the Mini Mental Status Examination that are used in the general population often show floor effects when used for individuals with DS because of their pre-existing cognitive impairment. Both informant-rated and direct neuropsychological tests have been used for the case detection of dementia for individuals with DS.

There are many similarities and some differences in the clinical manifestation of dementia in individuals with ID and the non-ID general population. Impaired recent memory and confusion in the context of relatively intact distant memory is likely to be an early symptom in individuals with ID who have a mild ID, whereas loss of skills and behaviour changes are likely to be an early feature for those with more severe ID. Many symptoms, including features of ‘frontal lobe dysfunction’ that tend to appear late in the dementing process in the general population, may appear early in individuals with ID and DS.

Ideally, individuals with ID should be screened for signs of dementia from before the age of 30/35. A multidisciplinary approach should be taken for diagnosing dementia in individuals with ID using a combination of informant-rated scales and neuropsychological tests in a longitudinal fashion over time. Important differential diagnoses include hypothyroidism, depression, and sensory impairment. Assessment should include physical, psychological, and social aspects including appropriate examinations and investigations. The evidence-base for the pharmacological management of dementia in ID and DS is poor, which does not allow to draw any definitive conclusion about their efficacy in this population. Therefore, non-pharmacological interventions should be considered.