Progressive hemiparesis (Mills syndrome) with aphasia in amyotrophic lateral sclerosis.
Progressive hemiparesis (Mills syndrome) with aphasia in amyotrophic lateral sclerosis.
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DOI：
10.1212/wnl.0000000000000090
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发表时间：
2014-02-04
期刊：
影响因子：
9.9
通讯作者：
Turner MR
中科院分区：
文献类型：
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作者：
Bäumer D;Butterworth R;Menke RA;Talbot K;Hofer M;Turner MR
肌萎缩侧索硬化症（ALS）的运动症状的发作是显著的局灶性的。在四分之三的病例中，一侧肢体出现无力，通常在数月内连续蔓延，变成双侧。1一种极其罕见的上运动神经元为主的进行性轻偏瘫临床综合征首先由美国神经学家Charles Karsner米尔斯描述（1845-1930）。2更典型的ALS与额颞叶痴呆（FTD）有共同的组织病理学特征，由含有DNA和RNA结合蛋白TDP-43的泛素化神经元和神经胶质内含物组成。认知障碍可在至少三分之一的ALS病例中检测到，主要涉及语言、执行功能和流畅性缺陷，伴有不同程度的行为障碍，这些障碍都与更纯粹的FTD综合征重叠。Frank FTD见于高达15%的ALS患者，通常发生在运动症状出现之前或之后不久，并与更快的疾病进展相关。
The onset of motor symptoms in amyotrophic lateral sclerosis (ALS) is strikingly focal. In three-quarters of cases, weakness emerges unilaterally in one limb, typically spreading contiguously over months to become bilateral.1 An extremely rare clinical syndrome of upper motor neuron–predominant, progressive hemiparesis was first described by American neurologist Charles Karsner Mills (1845–1930).2 More typical ALS shares a common histopathologic signature with frontotemporal dementia (FTD), consisting of ubiquitinated neuronal and glial inclusions containing the DNA and RNA binding protein, TDP-43. Cognitive impairment may be detected in at least one-third of ALS cases and involves mainly deficits in language, executive function, and fluency, with variable levels of behavioral impairments that all have overlap with the purer FTD syndromes. Frank FTD is seen in up to 15% of patients with ALS, in whom it typically occurs before or soon after the development of motor symptoms, and is associated with a more rapid disease progression.3