Dekker Alain D, Coppus Antonia M W, Vermeiren Yannick, Aerts Tony, van Duijn Cornelia M, Kremer Berry P, Naudé Pieter J W, Van Dam Debby, De Deyn Peter P
Department of Neurology and Alzheimer Research Center, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands Laboratory of Neurochemistry and Behaviour, Institute Born-Bunge, University of Antwerp, Wilrijk, Antwerp, Belgium.
Dichterbij, Center for the Intellectually Disabled, Gennep, The Netherlands Department of Epidemiology, Erasmus University Medical Center, Rotterdam, The Netherlands Department of Primary and Community Care, Radboud University Medical Center, Nijmegen, The Netherlands.
J Alzheimers Dis. 2015;43(3):871-91. doi: 10.3233/JAD-140783.
Down syndrome (DS) is the most prevalent genetic cause of intellectual disability. Early-onset Alzheimer's disease (AD) frequently develops in DS and is characterized by progressive memory loss and behavioral and psychological signs and symptoms of dementia (BPSD). Predicting and monitoring the progression of AD in DS is necessary to enable adaptive caretaking.
Reliable blood biomarkers that aid the prediction of AD are necessary, since cerebrospinal fluid sampling is rather burdensome, particularly for people with DS. Here, we investigate serum levels of eight biogenic amines and their metabolites in relation to dementia staging and probable BPSD items.
Using RP-HPLC with electrochemical detection, (nor)adrenergic (NA/A and MHPG), serotonergic (5-HT and 5-HIAA), and dopaminergic (DA, HVA, and DOPAC) compounds were quantified in the serum of DS subjects with established AD at baseline (n = 51), DS subjects without AD (n = 50), non-demented DS individuals that converted to AD over time (n = 50), and, finally, healthy non-DS controls (n = 22).
Serum MHPG levels were significantly lower in demented and converted DS subjects (p < 0.0001) compared to non-demented DS individuals and healthy controls. Those subjects with MHPG levels below median had a more than tenfold increased risk of developing dementia. Furthermore, significant correlations were observed between monoaminergic serum values and various probable BPSD items within each DS group.
Decreased serum MHPG levels show great potential as biomarker to monitor and predict conversion to AD in DS. Moreover, significant monoaminergic alterations related to probable BPSD items, suggesting that monoaminergic dysregulation is an underlying biological mechanism, and demonstrating the need to develop a validated rating scale for BPSD in DS.
唐氏综合征(DS)是智力残疾最常见的遗传病因。早发性阿尔茨海默病(AD)在DS患者中经常发生,其特征为进行性记忆丧失以及痴呆的行为和心理体征及症状(BPSD)。预测和监测DS患者中AD的进展对于实现适应性护理很有必要。
由于脑脊液采样相当繁琐,尤其是对DS患者而言,因此需要可靠的血液生物标志物来辅助AD的预测。在此,我们研究了八种生物胺及其代谢物的血清水平与痴呆分期和可能的BPSD项目之间的关系。
采用反相高效液相色谱法结合电化学检测,对基线时已确诊为AD的DS患者(n = 51)、无AD的DS患者(n = 50)、随时间推移转化为AD的非痴呆DS个体(n = 50)以及健康非DS对照(n = 22)的血清中的(去甲)肾上腺素能(NA/A和MHPG)、血清素能(5-HT和5-HIAA)和多巴胺能(DA、HVA和DOPAC)化合物进行定量。
与非痴呆DS个体和健康对照相比,痴呆和转化型DS患者的血清MHPG水平显著降低(p < 0.0001)。MHPG水平低于中位数的患者患痴呆的风险增加了十多倍。此外,在每个DS组中,单胺能血清值与各种可能的BPSD项目之间观察到显著相关性。
血清MHPG水平降低作为监测和预测DS患者转化为AD的生物标志物具有巨大潜力。此外,与可能的BPSD项目相关的显著单胺能改变,表明单胺能失调是一种潜在的生物学机制,并证明有必要为DS患者的BPSD制定经过验证的评定量表。