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Biomarkers of conversion to α-synucleinopathy in isolated rapid-eye-movement sleep behaviour disorder
The Lancet Neurology ( IF 48.0 ) Pub Date : 2021-07-21 , DOI: 10.1016/s1474-4422(21)00176-9
Mitchell G Miglis 1 , Charles H Adler 2 , Elena Antelmi 3 , Dario Arnaldi 4 , Luca Baldelli 5 , Bradley F Boeve 6 , Matteo Cesari 7 , Irene Dall'Antonia 8 , Nico J Diederich 9 , Kathrin Doppler 10 , Petr Dušek 8 , Raffaele Ferri 11 , Jean-François Gagnon 12 , Ziv Gan-Or 13 , Wiebke Hermann 14 , Birgit Högl 7 , Michele T Hu 15 , Alex Iranzo 16 , Annette Janzen 17 , Anastasia Kuzkina 10 , Jee-Young Lee 18 , Klaus L Leenders 19 , Simon J G Lewis 20 , Claudio Liguori 21 , Jun Liu 22 , Christine Lo 15 , Kaylena A Ehgoetz Martens 23 , Jiri Nepozitek 8 , Giuseppe Plazzi 24 , Federica Provini 25 , Monica Puligheddu 26 , Michal Rolinski 27 , Jan Rusz 28 , Ambra Stefani 7 , Rebekah L S Summers 29 , Dallah Yoo 30 , Jennifer Zitser 31 , Wolfgang H Oertel 32
Affiliation  

Patients with isolated rapid-eye-movement sleep behaviour disorder (RBD) are commonly regarded as being in the early stages of a progressive neurodegenerative disease involving α-synuclein pathology, such as Parkinson's disease, dementia with Lewy bodies, or multiple system atrophy. Abnormal α-synuclein deposition occurs early in the neurodegenerative process across the central and peripheral nervous systems and might precede the appearance of motor symptoms and cognitive decline by several decades. These findings provide the rationale to develop reliable biomarkers that can better predict conversion to clinically manifest α-synucleinopathies. In addition, biomarkers of disease progression will be essential to monitor treatment response once disease-modifying therapies become available, and biomarkers of disease subtype will be essential to enable prediction of which subtype of α-synucleinopathy patients with isolated RBD might develop.



中文翻译:

孤立性快速眼动睡眠行为障碍转化为 α-突触核蛋白病的生物标志物

患有孤立性快速眼动睡眠行为障碍 (RBD) 的患者通常被认为处于涉及 α-突触核蛋白病理学的进行性神经退行性疾病的早期阶段,例如帕金森病、路易体痴呆或多系统萎缩。异常的 α-突触核蛋白沉积发生在中枢和周围神经系统的神经退行性过程的早期,并且可能在运动症状和认知能力下降之前几十年出现。这些发现为开发可靠的生物标志物提供了依据,这些生物标志物可以更好地预测转化为临床表现的 α-突触核蛋白病。此外,一旦疾病改善疗法可用,疾病进展的生物标志物对于监测治疗反应至关重要,

更新日期:2021-07-22
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