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Occasional essay: Upper motor neuron syndrome in amyotrophic lateral sclerosis.
Journal of Neurology, Neurosurgery, and Psychiatry ( IF 11.0 ) Pub Date : 2020-03-01 , DOI: 10.1136/jnnp-2019-321938
Michael Swash 1 , David Burke 2 , Martin R Turner 3 , Julian Grosskreutz 4 , P Nigel Leigh 5 , Mamede deCarvalho 6 , Matthew C Kiernan 2, 7
Affiliation  

The diagnosis of amyotrophic lateral sclerosis (ALS) requires recognition of both lower motor neuron (LMN) and upper motor neuron (UMN) dysfunction.1 However, classical UMN signs are frequently difficult to identify in ALS.2 LMN involvement is sensitively detected by electromyography (EMG),3 but, as yet, there are no generally accepted markers for monitoring UMN abnormalities,4 the neurobiology of ALS itself and disease spread through the brain and the spinal cord.5 Full clinical assessment is therefore necessary to exclude other diagnoses and to monitor disease progression. In part, this difficulty regarding detection of UMN involvement in ALS derives from the definition of ‘the UMN syndrome’. Abnormalities of motor control in ALS require reformulation within an expanded concept of the UMN, together with the neuropathological, neuroimaging and neurophysiological abnormalities in ALS. We review these issues here. Sir Charles Sherrington (1857–1952) defined the LMN6 7 as the anterior horn cell and its motor axon, constituting the final common pathway for reflex action.8 In 1906, Sherrington,7 following Hughlings Jackson’s insights, concluded that motor acts were initiated in the brain by sensory input, thus building on activation of this simple reflex pathway, a view further developed by Sir Francis Walshe (1885–1973).9 Merton10 likened the effect of reflex action to a follow-up length servo, an influential hypothesis that was subsequently modified as servo assistance to emphasise that stretch reflexes support movement, generated centrally, rather than drive it.11 12 Despite these ideas, the UMN syndrome is not well defined.7 13–15 The clinical criteria (table 1) used by generations of neurologists to define the ‘corticospinal’ or ‘pyramidal’ syndrome, a term frequently but erroneously regarded as synonymous with ‘UMN syndrome’, rest on surprisingly uncertain pathophysiological underpinnings. The term UMN was introduced by Sir William Gowers (1845–1915) in his manual …

中文翻译:

偶发论文:肌萎缩性侧索硬化症的上运动神经元综合征。

肌萎缩性侧索硬化症(ALS)的诊断需要识别下运动神经元(LMN)和上运动神经元(UMN)的功能障碍。1但是,在ALS中通常很难识别出经典的UMN征象。2通过肌电图可以灵敏地检测到LMN参与(EMG)3,但到目前为止,还没有普遍公认的监测UMN异常的标志物4。ALS本身的神经生物学以及疾病通过脑和脊髓传播的情况。5因此,有必要进行全面的临床评估以排除其他诊断和监测疾病进展。在某种程度上,这种有关检测UMN参与ALS的困难来自“ UMN综合征”的定义。ALS的运动控制异常需要在UMN的扩展概念以及神经病理学,ALS的神经影像学和神经生理异常。我们在这里回顾这些问题。Charles Sherrington爵士(1857–1952)将LMN6 7定义为前角细胞及其运动轴突,构成了反射运动的最终共同途径。81906年,Hurrlings Jackson的见识之后,Sherrington [7]得出结论,运动行为是在弗朗西斯·沃尔什爵士(Francis Walshe,1885–1973)进一步发展了这种观点,即通过感觉输入来激活大脑,从而建立了这种简单的反射途径。9默顿[10]将反射作用的影响比作后续长度的伺服,这是一个有影响力的假设,随后被修改为伺服辅助,以强调拉伸反射支持集中产生的运动,而不是驱动运动。1112尽管有这些想法,但UMN综合征尚不明确。7 13–15几代神经学家使用的临床标准(表1)定义了“皮质脊髓型”或“金字塔型”综合症(经常但被错误地视为“ UMN综合症”的同义词),其基于令人惊讶的不确定的病理生理基础。UMN一词由William Gowers爵士(1845–1915)在其手册中引入...
更新日期:2020-02-13
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