Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466-8550, Japan; Innovation Center for Clinical Research, National Center for Geriatrics and Gerontology, 7-430 Morioka-cho, Obu 474-8511, Japan; Department of Speech Pathology and Audiology, Aichi Gakuin University School of Health Science,12 Araike, Iwasaki-cho, Nisshin 470-0195, Japan.
Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466-8550, Japan.
J Neurol Sci. 2019 Dec 15;407:116503. doi: 10.1016/j.jns.2019.116503. Epub 2019 Oct 15.
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 score 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.
脊髓延髓肌肉萎缩症（SBMA）是一种仅影响男性的遗传性神经肌肉疾病，其特征为球部和肢体肌肉进行性萎缩和无力。本研究旨在通过语音声学分析评估 SBMA 患者的软腭功能障碍（VPD）特征。我们招募了 23 名经基因确认的 SBMA 患者，并使用 Nasometer II 测量鼻共鸣值来评估他们的言语。SBMA 组的鼻共鸣值高于健康对照组（p=0.035），且与修订后的肌萎缩侧索硬化功能评定量表的总分呈显著负相关（rs=-0.520，p=0.011）。基于 VPD 研究的结果，我们评估了 2 名 SBMA 患者使用腭提升假体（PLP）治疗 VPD 的疗效。PLP 改善了这 2 例患者的构音障碍，但对吞咽困难的影响并不一致。本研究表明，鼻共鸣值是评估 SBMA 患者 VPD 的一种有用的定量测量方法。PLP 可通过降低 VPD 改善 SBMA 患者的构音障碍，但鉴于其对吞咽困难的潜在负面影响，该手术的临床应用应谨慎考虑。
