Behavioural and psychological symptoms of dementia in Down syndrome: Early indicators of clinical Alzheimer's disease?

Abstract

Behavioural and Psychological Symptoms of Dementia (BPSD) are a core symptom of dementia and are associated with suffering, earlier institutionalization and accelerated cognitive decline for patients and increased caregiver burden. Despite the extremely high risk for Down syndrome (DS) individuals to develop dementia due to Alzheimer's disease (AD), BPSD have not been comprehensively assessed in the DS population. Due to the great variety of DS cohorts, diagnostic methodologies, sub-optimal scales, covariates and outcome measures, it is questionable whether BPSD have always been accurately assessed. However, accurate recognition of BPSD may increase awareness and understanding of these behavioural aberrations, thus enabling adaptive caregiving and, importantly, allowing for therapeutic interventions. Particular BPSD can be observed (long) before the clinical dementia diagnosis and could therefore serve as early indicators of those at risk, and provide a new, non-invasive way to monitor, or at least give an indication of, the complex progression to dementia in DS. Therefore, this review summarizes and evaluates the rather limited knowledge on BPSD in DS and highlights its importance and potential for daily clinical practice.

The Early Presentation of Dementia in People with Down Syndrome: a Systematic Review of Longitudinal Studies

“…Given that the majority of the studies included in this review point towards BPDS as an early sign of dementia in DS, we believe that there is an urgent need for the development of an acceptable, validated and standardised evaluation scale for BPSD in DS (Sinai et al 2016), similar to BEHAVE-AD in the general population (Dekker et al 2015). …”

Blood Beta-Amyloid and Tau in Down Syndrome: A Comparison with Alzheimer’s Disease

“…The diagnosis of BPSD was made if the patient had the clinical presentation of apathy, agitation, hyperactivity and/or general slowness, depression and psychotic symptoms (Dekker et al, 2015). Dementia was diagnosed by an ABDQ score higher than 78 or by a clinical AD diagnosis according to the degree of cognitive and functional decline; the clinical diagnosis was performed by an adult neurologist (MJC) who is an expert in the diagnosis and management of dementia.…”

“…Recently, the behavioral and psychological symptoms of dementia (BPSD) in DS have been considered as early indicators of clinical dementia (Dekker et al, 2015). However, the diagnosis of BPSD is challenging in DS because of the underlying intellectual disabilities, and the assessment of BPSD in DS has not been well studied (Dekker et al, 2015; Prasher et al, 2015).…”

“…However, the diagnosis of BPSD is challenging in DS because of the underlying intellectual disabilities, and the assessment of BPSD in DS has not been well studied (Dekker et al, 2015; Prasher et al, 2015). We have developed a new technology called immunomagnetic reduction (IMR), which is capable of assaying molecules at very low concentrations (Yang et al, 2011; Chiu et al, 2012, 2013, 2014).…”

Síndrome de Down y enfermedad de Alzheimer: factores de riesgo, evaluación e intervención

“…La limitada comprensión actual y la gran cantidad de informes inconsistentes ilustran la vital importancia de una escala de evaluación de "todo incluido", que no omita ítems específicos de estos síntomas de antemano y que se centre en los cambios individuales producidos a lo largo del tiempo, diferenciando esos cambios del comportamiento típico de la persona (Dekker et al, 2015).…”