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J Neurol Sci. 2019 Dec 15;407:116503. doi: 10.1016/j.jns.2019.116503. Epub 2019 Oct 15.

Nasometric Scores in spinal and bulbar muscular atrophy - Effects of palatal lift prosthesis on dysarthria and dysphagia.

Author information

1
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. Electronic address: seiyat@ncgg.go.jp.
2
Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466-8550, Japan. Electronic address: hassy0707@med.nagoya-u.ac.jp.
3
Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466-8550, Japan. Electronic address: hijikata@med.nagoya-u.ac.jp.
4
Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466-8550, Japan. Electronic address: s.yamada@med.nagoya-u.ac.jp.
5
Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466-8550, Japan. Electronic address: daisuke.ito@med.nagoya-u.ac.jp.
6
Department of Oral and Maxillofacial Surgery, School of Dentistry, Aichi Gakuin University, 2-11Suemori-dori, Chikusa-ku, Nagoya 464-8651, Japan. Electronic address: nkym8020@dpc.agu.ac.jp.
7
Department of Oral and Maxillofacial Surgery, School of Dentistry, Aichi Gakuin University, 2-11Suemori-dori, Chikusa-ku, Nagoya 464-8651, Japan. Electronic address: ken-kuri@dpc.agu.ac.jp.
8
Department of Rehabilitation, Inokoshi Hospital, 1-1501 Inokoishihara, Meito-ku, Nagoya 465-0008, Japan. Electronic address: hy620206@yahoo.co.jp.
9
Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466-8550, Japan. Electronic address: banno@med.nagoya-u.ac.jp.
10
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. Electronic address: keisuke@ncgg.go.jp.
11
Department of Speech Pathology and Audiology, Aichi Gakuin University School of Health Science,12 Araike, Iwasaki-cho, Nisshin 470-0195, Japan. Electronic address: ymasahik@dpc.agu.ac.jp.
12
Research Division of Dementia and Neurodegenerative Disease, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466-8550, Japan. Electronic address: sobueg@med.nagoya-u.ac.jp.
13
Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466-8550, Japan. Electronic address: ka2no@med.nagoya-u.ac.jp.

Abstract

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.

KEYWORDS:

Acoustic analysis; Dysarthria; Dysphagia; Palatal lift prosthesis; Spinal and bulbar muscular atrophy; Velopharyngeal dysfunction

PMID:
31669728
DOI:
10.1016/j.jns.2019.116503

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