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

Tanaka, Seiya; Hashizume, Akira; Hijikata, Yasuki; Yamada, Shinichiro; Ito, Daisuke; Nakayama, Atsushi; Kurita, K.; Yogo, Hiroaki; Banno, Haruhiko; Suzuki, Keisuke; Yamamoto, Masahiko; Sobue, Gen; Katsuno, Masahisa

doi:10.1016/j.jns.2019.116503

Cited by 8 publications

(4 citation statements)

References 34 publications

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“…Measurement of bulbar strength may also be considered, including analysis of speech and evidence of velopharyngeal dysfunction associated with lifting of the soft palate. Nasalance score measurements using a Nasometer II device to assess nasality were abnormal in SBMA subjects, and correlated with other measures of bulbar function [41]. Additional tools to evaluate bulbar function may include the 6-K-scale assessment of V, VII, IX, X, and XII cranial nerve and ansa cervicalis function [42].…”

Section: Biomarkers For Evaluating Therapeuticsmentioning

confidence: 93%

Molecular pathogenesis of spinal bulbar muscular atrophy (Kennedy's disease) and avenues for treatment

Fischbeck

2020

Current Opinion in Neurology

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Purpose of review:To illustrate the current understanding and avenues for developing treatment in spinal and bulbar muscular atrophy (SBMA), an inherited neuromuscular disorder caused by a CAG trinucleotide repeat expansion in the androgen receptor (AR) gene.Recent findings: Important advances have been made in characterizing the molecular mechanism of the disease including the disruption of protein homeostasis, intracellular trafficking, and signaling pathways. Biomarkers such as MRI quantification of muscle volume and fat fraction have been used to track disease progression, and will be useful in future clinical studies. Therapies tested and under development have been based on diverse strategies including targeting mutant AR gene expression, stability, and activity, and pathways that mitigate disease toxicity. Summary:We provide an overview of the recent advances in understanding the SBMA disease mechanism and highlight efforts to translate these insights into safe and effective therapy.

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Section: Biomarkers For Evaluating Therapeuticsmentioning

confidence: 93%

Molecular pathogenesis of spinal bulbar muscular atrophy (Kennedy's disease) and avenues for treatment

Fischbeck

2020

Current Opinion in Neurology

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“…The combined effects of PAP and PLP, such as improved tongue pressure and prevention of nasopharyngeal reflux, led to a decrease in pharyngeal residue. It has been considered that use of a PLP may inhibit swallowing, 7 , 9 ) although evaluation of the f-PLP developed by Ohno et al, 8 ) which featured a flexible lift, showed that it reduced discomfort during swallowing. In the present case, the fPL/ACP, which is a combination of PAP and f-PLP, demonstrated the positive effects of both components on swallowing function.…”

Section: Discussionmentioning

confidence: 99%

“…Forced elevation of the soft palate by a palatal lift causes severe discomfort during swallowing. 7 ) Ohno et al fabricated a flexible-lift PLP (f-PLP) to overcome this issue. 8 ) The effects of the f-PLP on swallowing function include the elimination of nasopharyngeal reflux and increased velopharyngeal pressure, as demonstrated through high-resolution manometry.…”

Section: Introductionmentioning

confidence: 99%

A Case of Dysphagia and Dysarthria Improved by Flexible-palatal Lift/augmentation Combination Prosthesis

Hirasaki¹,

Takagi

Umeda

et al. 2023

PRM

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Background: Palatal augmentation prosthesis (PAP) and palatal lift prosthesis (PLP) have been used to improve dysphagia and dysarthria. However, to date, there are few reports on their combined use. We report a quantitative evaluation of the effectiveness of a flexible-palatal lift/augmentation combination prosthesis (fPL/ACP) based on videofluoroscopic swallowing study (VFSS) and speech intelligibility testing. Case: An 83-year-old woman was admitted to our hospital with a hip fracture. She developed aspiration pneumonia at 1 month after partial hip replacement. Oral motor function tests revealed a motor deficit of the tongue and soft palate. VFSS showed delayed oral transit, nasopharyngeal reflux, and excessive pharyngeal residue. The cause of her dysphagia was assumed to be pre-existing diffuse large B-cell lymphoma and sarcopenia. To improve the dysphagia, an fPL/ACP was fabricated and applied. It improved the patient’s oral and pharyngeal swallowing and speech intelligibility. In addition to prosthetic treatment, rehabilitation and nutritional support allowed her to be discharged. Discussion: The effects of fPL/ACP in the present case were similar to those of flexible-PLP and PAP. f-PLP assists in elevation of the soft palate and improved the nasopharyngeal reflux and hypernasal speech. PAP promotes tongue movement and results in improved oral transit and speech intelligibility. Therefore, fPL/ACP may be effective in patients with motor deficits in both the tongue and soft palate. To maximize the effect of the intraoral prosthesis, a transdisciplinary approach with concurrent swallowing rehabilitation, nutritional support, and physical and occupational therapy is necessary.

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“…Compared to the other MSD subtypes, dysarthria associated with Kennedy disease has been very little described in the literature. In previous studies, higher jitter, shimmer and HNR and hypernasality have been reported in only a subset of the patients with Kennedy disease [ 44 , 45 ]. In our data, no voice impairment was observed.…”

Section: Discussionmentioning

confidence: 99%

Differentiation of Motor Speech Disorders through the Seven Deviance Scores from MonPaGe-2.0.s

2022

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For the clinical assessment of motor speech disorders (MSDs) in French, the MonPaGe-2.0.s protocol has been shown to be sensitive enough to diagnose mild MSD based on a combination of acoustic and perceptive scores. Here, we go a step further by investigating whether these scores—which capture deviance on intelligibility, articulation, voice, speech rate, maximum phonation time, prosody, diadochokinetic rate—contribute to the differential diagnosis of MSDs. To this aim, we trained decision trees for two-class automatic classification of different pairs of MSD subtypes based on seven deviance scores that are computed in MonPaGe-2.0.s against matched normative data. We included 60 speakers with mild to moderate MSD from six neuropathologies (amyotrophic lateral sclerosis, Wilson, Parkinson and Kennedy disease, spinocerebellar ataxia, post-stroke apraxia of speech). The two-class classifications relied mainly on deviance scores from four speech dimensions and predicted with over 85% accuracy the patient’s correct clinical category for ataxic, hypokinetic and flaccid dysarthria; classification of the other groups (apraxia of speech and mixed dysarthria) was slightly lower (79% to 82%). Although not perfect and only tested on small cohorts so far, the classification with deviance scores based on clinically informed features seems promising for MSD assessment and classification.

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

Cited by 8 publications

References 34 publications

Molecular pathogenesis of spinal bulbar muscular atrophy (Kennedy's disease) and avenues for treatment

Molecular pathogenesis of spinal bulbar muscular atrophy (Kennedy's disease) and avenues for treatment

A Case of Dysphagia and Dysarthria Improved by Flexible-palatal Lift/augmentation Combination Prosthesis

Differentiation of Motor Speech Disorders through the Seven Deviance Scores from MonPaGe-2.0.s

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