Milella Giammarco, Sciancalepore Diletta, Cavallaro Giada, Piccirilli Glauco, Nanni Alfredo Gabriele, Fraddosio Angela, D'Errico Eustachio, Paolicelli Damiano, Fiorella Maria Luisa, Simone Isabella Laura
Neurology Unit, Department of Translational Biomedicine and Neurosciences, 70121 Bari, Italy.
Otolaryngology Unit, Department of Translational Biomedicine and Neurosciences (DiBraiN), University of Bari Aldo Moro, 70121 Bari, Italy.
Biomedicines. 2023 Aug 31;11(9):2439. doi: 10.3390/biomedicines11092439.
Approximately 80-96% of people with amyotrophic lateral sclerosis (ALS) become unable to speak during the disease progression. Assessing upper and lower motor neuron impairment in bulbar regions of ALS patients remains challenging, particularly in distinguishing spastic and flaccid dysarthria. This study aimed to evaluate acoustic voice parameters as useful biomarkers to discriminate ALS clinical phenotypes. Triangular vowel space area (tVSA), alternating motion rates (AMRs), and sequential motion rates (SMRs) were analyzed in 36 ALS patients and 20 sex/age-matched healthy controls (HCs). tVSA, AMR, and SMR values significantly differed between ALS and HCs, and between ALS with prevalent upper (pUMN) and lower motor neuron (pLMN) impairment. tVSA showed higher accuracy in discriminating pUMN from pLMN patients. AMR and SMR were significantly lower in patients with bulbar onset than those with spinal onset, both with and without bulbar symptoms. Furthermore, these values were also lower in patients with spinal onset associated with bulbar symptoms than in those with spinal onset alone. Additionally, AMR and SMR values correlated with the degree of dysphagia. Acoustic voice analysis may be considered a useful prognostic tool to differentiate spastic and flaccid dysarthria and to assess the degree of bulbar involvement in ALS.
在肌萎缩侧索硬化症(ALS)患者疾病进展过程中,约80 - 96%的人会出现无法说话的情况。评估ALS患者延髓区域的上下运动神经元损伤仍然具有挑战性,尤其是区分痉挛性和弛缓性构音障碍。本研究旨在评估声学语音参数作为区分ALS临床表型的有用生物标志物。对36例ALS患者和20例年龄/性别匹配的健康对照(HCs)进行了三角元音空间面积(tVSA)、交替运动率(AMR)和连续运动率(SMR)分析。ALS患者与HCs之间,以及以延髓起病为主的上运动神经元(pUMN)和下运动神经元(pLMN)损伤的ALS患者之间,tVSA、AMR和SMR值存在显著差异。tVSA在区分pUMN和pLMN患者方面显示出更高的准确性。无论有无延髓症状,延髓起病的患者AMR和SMR均显著低于脊髓起病的患者。此外,伴有延髓症状的脊髓起病患者的这些值也低于单纯脊髓起病的患者。此外,AMR和SMR值与吞咽困难程度相关。声学语音分析可被视为一种有用的预后工具,用于区分痉挛性和弛缓性构音障碍,并评估ALS患者延髓受累程度。