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首页> 外文期刊>Amyotrophic lateral sclerosis eofficial publication of the World Federation of Neurology Research Group on Motor Neuron Diseases >Muscle histopathology in upper motor neuron-dominant amyotrophic lateral sclerosis
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Muscle histopathology in upper motor neuron-dominant amyotrophic lateral sclerosis

机译：上运动神经元为主的肌萎缩性侧索硬化的肌肉组织病理学

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摘要

The distinction between primary lateral sclerosis (PLS) and amyotrophic lateral sclerosis (ALS) still remains debated. Recently, PLS patients displaying lower motor neuron (LMN) signs have been defined as ‘upper motor neuron (UMN)- dominant ALS’, using ‘clinically pure PLS’ diagnosis to those with no LMN signs. To further characterize the LMN involvement in UMN-dominant ALS we investigated the presence and the extent of neurogenic abnormalities in the skeletal muscle of patients affected with a pyramidal syndrome consistent with UMN-dominant ALS. A total of nine patients affected with UMN-dominant ALS were analysed. In all cases, muscle biopsies showed the presence of scattered or clustered atrophic angulated fibres in small groups, and a mild to moderate fibre type-grouping. Target and targetoid fibres were detected in two cases only. Three patients had a second muscle biopsy which demonstrated a roughly unchanged pattern of chronic denervation with still moderate reinnervation phenomena. This study suggests that in UMN-dominant ALS muscle denervation may be characterized by an early chronic impairment of a restricted number of LMNs. The extent rather than the presence of LMN signs may allow to categorize patients with motor neuron disease involving mainly UMN into distinct entities.
机译：原发性侧索硬化症（PLS）和肌萎缩性侧索硬化症（ALS）之间的区别仍然存在争议。最近，对表现出下运动神经元（LMN）征兆的PLS患者，通过对那些没有LMN征兆的患者进行“临床纯PLS”诊断，被定义为“上运动神经元（UMN）占优势的ALS”。为了进一步表征LMN参与UMN占优势的ALS，我们调查了患有与UMN占优势的锥体综合征相关的患者的骨骼肌中神经源性异常的存在和程度。总共分析了9名受UMN主导的ALS患者。在所有情况下，肌肉活检均显示小群中存在散布或成簇的萎缩性角化纤维，以及轻度至中度纤维类型分组。仅在两种情况下检测到靶标纤维和靶标纤维。三名患者进行了第二次肌肉活检，显示慢性神经支配的模式基本没有改变，但仍存在中等程度的神经支配现象。这项研究表明，在以UMN为主导的ALS中，肌肉失神经的特征可能是有限数量的LMN的早期慢性损伤。 LMN征兆的程度而不是其存在可能允许将主要涉及UMN的运动神经元疾病患者分类为不同的实体。

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