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Mov Disord : 进行性核上麻痹,言语障碍的灰白质结构基础是什么?

2021-09-23 Freeman MedSci原创

神经解剖学损伤的不同模式与口腔和咽喉阶段的吞咽障碍相对应。

进行性核上性麻痹(PSP)是一种神经退行性tauopathy,以眼球运动功能障碍、姿势不稳和跌倒、运动障碍和认知功能障碍为特征。

延髓症状往往出现得很早,可以很严重,而且是一个独立的生存预测因素,可能是因为生理性吞咽障碍导致吸入性肺炎的风险增加,这是PSP最常见的死亡原因。

以前关于PSP吞咽困难的报告一直都是以口腔(相对于咽部)的障碍为主。常见的口腔阶段障碍包括来回摇动,或舌头的嵌顿运动,以推动药丸向咽部移动,舌根回缩减少,咽部吞咽的开始时间延迟。

咽相障碍的严重程度和气道侵入的程度与PSP疾病的严重程度有关。初级脑干和核上控制中心支持吞咽,口腔阶段受自愿控制,核上控制中心的影响较大,咽和食道阶段由髓质中央模式发生器驱动。纤维束,包括皮质-球状束和胼胝体,为这个网络的功能服务。然而,对PSP吞咽困难的神经解剖学基础知之甚少。

藉此,美国梅奥诊所的Heather M. Clark等人,探究了灰质体积和白质道的完整性与口腔和咽部的吞咽困难和气道入侵的关系。

并预测口腔阶段的障碍将与 高层皮质吞咽网络有关,而咽部相障碍将与脑干通路。

38名患有进行性核上性麻痹的参与者接受了视频荧光图吞咽评估( videofluorographic swallowing assessment)以及结构和弥散张量头部磁共振成像。线性回归模型评估了吞咽指标与区域灰质体积、白质各向异性和平均扩散率之间的关系。

他们发现:口腔阶段的障碍与顶上容积的减少和顶层和感觉运动白质、内囊后肢和上纵筋的异常扩散性有关。咽相损伤与内侧额叶、皮质脊髓束和脑梗的破坏有关。没有任何区域可以预测气道入侵。

这个研究的重要意义在于发现了:神经解剖学损伤的不同模式与口腔和咽喉阶段的吞咽障碍相对应。


原文出处:
Clark HM, Tosakulwong N, Weigand SD, et al. Gray and White Matter Correlates of Dysphagia in Progressive Supranuclear Palsy. Mov Disord. Published online August 23, 2021:mds.28731. doi:10.1002/mds.28731

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    2021-09-24 cmsvly
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介绍 65 岁展大爷病例,诊断为进行性核上性麻痹,阐述其症状、影像表现、分型及治疗方法,强调综合诊断及对症支持治疗的重要性。

第99届美国神经病理学家协会年会:混合性进行性核上性麻痹(PSP)和多系统萎缩(MSA)—— 4例临床表现异质性报告

考虑到PSP不同的临床和病理特征,以及不同的遗传危险因素(如MAPT遗传变异)和MSA(未知遗传风险),提示这4例患者的发现解释为代表了这些个体中两个独立的疾病过程。

2023 AAN| 进行性核上性麻痹中自主神经系统对听觉惊吓的反应减弱

面对强烈的外部刺激,心脏间跳间期减少较少,皮肤电导水平增加较少,提示交感神经系统反应减弱。研究人员怀疑这与脑桥回路中的脑干变性有关,这对于情绪挑战期间的惊吓反射至关重要。

JNNP:神经炎症预测进行性核上性麻痹的疾病进展

在进行性核上性麻痹(PSP)和其他疾病如阿尔茨海默病(AD)和额颞叶痴呆以及遗传、流行病学和影像学关联中,神经炎症被认为是一种常见的致病过程。例如,即使在疾病的早期阶段,激活的小胶质细胞也存在于神经原

Lancet Neurology:替拉奈单抗治疗进行性核上性麻痹的安全性和有效性:一项2期随机安慰剂对照试验

tau病是指tau蛋白聚集紊乱,其在生物化学、形态学和临床表现上具有异质性。以tau为靶点的治疗干预策略包括主动免疫(诱导免疫应答)和被动免疫(需要提供预先形成的抗体)。

2016 中国进行性核上性麻痹临床诊断标准

中华医学会神经病学分会帕金森病及运动障碍学组 · 2016-04-30

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