Nasometric Scores in spinal and bulbar muscular atrophy - Effects of palatal lift prosthesis on dysarthria and dysphagia
Nasometric Scores in spinal and bulbar muscular atrophy - Effects of palatal lift prosthesis on dysarthria and dysphagia
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DOI：
10.1016/j.jns.2019.116503
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发表时间：
2019-12-15
影响因子：
4.4
通讯作者：
Katsuno, Masahisa
中科院分区：
文献类型：
--
作者：
Tanaka, Seiya;Hashizume, Atsushi;Katsuno, Masahisa
脊髓延髓肌萎缩症（SBMA）是一种遗传性神经肌肉疾病，仅影响男性，其特征是进行性肌肉萎缩和延髓和四肢肌肉无力。本研究旨在通过言语声学分析来评估SBMA受试者腭咽闭合功能障碍（VPD）的特征。23例经遗传学证实的SBMA患者入组，并通过使用Nasometer II测量nasalance评分来评估他们的言语。SBMA组的鼻侧音评分高于健康对照组（p = 0.035），并与修订的肌萎缩侧索硬化症功能评定量表的总评分显著相关（rs =-0.520，p = 0.011）。根据VPD研究的结果，在两名SBMA患者中评估了腭提升假体（PLP）治疗VPD的疗效。PLP改善了两例患者的构音障碍，尽管假体对吞咽困难的影响并不一致。本研究表明，nasalance评分是一个有用的定量测量，以评估SBMA患者的VPD。PLP可通过减少VPD改善SBMA患者的构音障碍，但鉴于其对吞咽困难可能的负面影响，应仔细考虑该手术的临床应用。
Spinal and bulbar muscular atrophy (SBMA) is a hereditary neuromuscular disease affecting only males characterized by progressive muscular atrophy and weakness in bulbar and limb muscles. The present study aimed to evaluate the features of velopharyngeal dysfunction (VPD) in SBMA subjects by an acoustic analysis of speech. Twenty-three genetically confirmed patients with SBMA were enrolled and assessed their speech by measuring the nasalance score with a Nasometer II. The nasalance scores of the SBMA group was higher than that of healthy controls (p = .035) and significantly correlated with the total scare of the revised amyotrophic lateral sclerosis functional rating scale (rs = -0.520, p = .011). On the basis of the results of the VPD study, the efficacy of a palatal lift prosthesis (PLP) was assessed in two patients with SBMA to treat their VPD. The PLP improved dysarthria in both cases, although the impact of the prosthesis on dysphagia was not consistent. The present study suggested that the nasalance score is a useful quantitative measurement to evaluate VPD in patients with SBMA. A PLP may improve dysarthria in SBMA patients by reducing VPD, but the clinical application of this procedure should be considered carefully in view of its possible negative effect on dysphagia.