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延髓性肌萎缩侧索硬化症诊断的多学科临床方案

Multi-disciplinary clinical protocol for the diagnosis of bulbar amyotrophic lateral sclerosis.

作者信息

Chiaramonte Rita, Di Luciano Carmela, Chiaramonte Ignazio, Serra Agostino, Bonfiglio Marco

机构信息

Department of Physical Medicine and Rehabilitation, University of Catania, 95125 Catania, Italy.

Department for Health activities and Epidemiologic Observatory, Sicily Region, Italy.

出版信息

Acta Otorrinolaringol Esp (Engl Ed). 2019 Jan-Feb;70(1):25-31. doi: 10.1016/j.otorri.2017.12.002. Epub 2018 Apr 23.

Abstract

INTRODUCTION AND OBJECTIVES

The objective of this study was to examine the role of different specialists in the diagnosis of amyotrophic lateral sclerosis (ALS), to understand changes in verbal expression and phonation, respiratory dynamics and swallowing that occurred rapidly over a short period of time.

MATERIALS AND METHODS

22 patients with bulbar ALS were submitted for voice assessment, ENT evaluation, Multi-Dimensional Voice Program (MDVP), spectrogram, electroglottography, fiberoptic endoscopic evaluation of swallowing.

RESULTS

In the early stage of the disease, the oral tract and velopharyngeal port were involved. Three months after the initial symptoms, most of the patients presented hoarseness, breathy voice, dysarthria, pitch modulation problems and difficulties in pronunciation of explosive, velar and lingual consonants. Values of MDVP were altered. Spectrogram showed an additional formant, due to nasal resonance. Electroglottography showed periodic oscillation of the vocal folds only during short vocal cycle. Swallowing was characterized by weakness and incoordination of oro-pharyngeal muscles with penetration or aspiration.

CONCLUSIONS

A specific multidisciplinary clinical protocol was designed to report vocal parameters and swallowing disorders that changed more quickly in bulbar ALS patients. Furthermore, the patients were stratified according to involvement of pharyngeal structures, and severity index.

摘要

引言与目的

本研究的目的是探讨不同专科医生在肌萎缩侧索硬化症(ALS)诊断中的作用,以了解在短时间内迅速发生的言语表达与发声、呼吸动力学及吞咽方面的变化。

材料与方法

22例延髓性ALS患者接受了嗓音评估、耳鼻喉科评估、多维度嗓音程序(MDVP)、声谱图、电声门图、纤维内镜吞咽评估。

结果

在疾病早期,口腔及腭咽口受累。初始症状出现三个月后,大多数患者出现声音嘶哑、气息声、构音障碍、音高调制问题以及爆破音、软腭音和舌辅音发音困难。MDVP值发生改变。声谱图显示由于鼻腔共鸣出现额外的共振峰。电声门图显示仅在短发声周期内声带出现周期性振荡。吞咽的特点是口咽肌肉无力及不协调,伴有食物渗入或误吸。

结论

设计了一项特定的多学科临床方案,以报告延髓性ALS患者中变化更快的嗓音参数及吞咽障碍。此外,根据咽部结构受累情况及严重程度指数对患者进行分层。

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