DePaul R, Brooks B R
University of Wisconsin-Whitewater.
J Speech Hear Res. 1993 Dec;36(6):1158-67. doi: 10.1044/jshr.3606.1158.
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by significant speech and swallowing problems resulting from upper and lower motor neuron loss. Weakness is the primary ALS disease-related sign, and measures of muscle strength have revealed nonuniform patterns of muscle weakness in orofacial muscles. To a large extent, muscle strength measures in these studies have not been evaluated in terms of functional significance, and few researchers have addressed the relation between weakness and motor neuron loss. This study addressed whether multiple measures, including static isometric maximum voluntary contraction (MVC), a dynamic measure of the peak rate of change of force (PRCF), an upper motor neuron (UMN) index, and a functional disability score (FDS) might enhance understanding of speech dysfunction in ALS. Ten males diagnosed with sporadic ALS showing mild speech impairment and an equal number of matched controls were studied. Tongue MVC and PRCF were more impaired than those of the lip and jaw, irrespective of the time post onset and site of initial symptoms. Results also suggested that disproportionate tongue impairment may be related to UMN deficits. However, impairments in the rate of contraction did not appear to be related to UMN deficits. Tongue weakness and tongue and lower lip PRCF were related to the degree of speech severity, but none of the measures was related to speech intelligibility. The value of a functional outcome measure like speech intelligibility and its role in characterizing orofacial involvement in the early stages of ALS bulbar impairment are discussed.
肌萎缩侧索硬化症（ALS）是一种进行性神经退行性疾病，其特征是上下运动神经元丧失导致严重的言语和吞咽问题。肌无力是ALS主要的疾病相关体征，肌肉力量测量显示口面部肌肉的肌无力模式并不一致。在很大程度上，这些研究中的肌肉力量测量尚未从功能意义方面进行评估，很少有研究人员探讨肌无力与运动神经元丧失之间的关系。本研究探讨了包括静态等长最大自主收缩（MVC）、力的峰值变化率动态测量（PRCF）、上运动神经元（UMN）指数和功能残疾评分（FDS）在内的多种测量方法是否能增强对ALS言语功能障碍的理解。对10名被诊断为散发性ALS且有轻度言语障碍的男性以及数量相等的匹配对照组进行了研究。无论发病时间和初始症状部位如何，舌部的MVC和PRCF比唇部和下颌的受损更严重。结果还表明，舌部不成比例的损伤可能与UMN缺陷有关。然而，收缩率的损伤似乎与UMN缺陷无关。舌肌无力以及舌部和下唇的PRCF与言语严重程度有关，但这些测量方法均与言语清晰度无关。本文讨论了像言语清晰度这样的功能结局测量的价值及其在表征ALS延髓损伤早期口面部受累情况中的作用。
