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声带“瘫痪”:多系统萎缩的早期征象。

Vocal Fold "Paralysis": An Early Sign in Multiple System Atrophy.

机构信息

Department of Neurology, Mayo Clinic, Jacksonville, Florida.

Department of Otorhinolaryngology, Mayo Clinic, Rochester, Minnesota.

出版信息

J Voice. 2020 Nov;34(6):940-944. doi: 10.1016/j.jvoice.2019.06.008. Epub 2019 Jul 16.

Abstract

Multiple system atrophy (MSA) is a neurodegenerative disease characterized by a cerebellar syndrome, autonomic dysfunction, and extrapyramidal signs. Extrapyramidal signs may manifest as parkinsonism as well as dystonia, which is the involuntary contraction of a muscle(s) resulting in an abnormal posture. MSA belongs to a family of diseases known as α-synucleinopathies which are associated with dream enactment reflecting REM sleep behavior disorder (RBD). In patients with MSA, dystonia or paresis may involve the laryngeal muscles resulting in vocal fold hypomobility. We identified four individuals presenting with vocal complaints and subsequently diagnosed with vocal fold "paralysis." Within one year, each patient developed neurologic symptoms and upon evaluation by a movement disorders specialist was diagnosed with probable MSA. Our findings highlight the importance of screening by otolaryngologists when patients are diagnosed with vocal fold hypomobility. Specifically, patients should be assessed for RBD by questioning others if he/she acts out their dreams. The presence of RBD raises clinical suspicion for a synucleinopathy such as MSA. Untreated patients with MSA experiencing nocturnal stridor and breathing disorders have an increased risk for sudden death. Therefore, early evaluation by a movement disorders specialist to promptly diagnose MSA may have a substantial effect on morbidity and mortality in this high-risk patient population.

摘要

多系统萎缩症(MSA)是一种神经退行性疾病,其特征为小脑综合征、自主神经功能障碍和锥体外系体征。锥体外系体征可能表现为帕金森病以及肌张力障碍,即肌肉的不自主收缩导致异常姿势。MSA 属于 α-突触核蛋白病家族,与反映 REM 睡眠行为障碍(RBD)的梦境行为有关。在 MSA 患者中,肌张力障碍或瘫痪可能累及喉部肌肉,导致声带活动度降低。我们发现了 4 名出现声音问题并随后被诊断为声带“瘫痪”的患者。在一年内,每位患者均出现了神经系统症状,并经运动障碍专家评估后被诊断为可能的 MSA。我们的发现强调了耳鼻喉科医生在诊断声带活动度降低时进行筛查的重要性。具体而言,应通过询问他人患者是否表现出梦境中的行为来评估患者是否存在 RBD。存在 RBD 会引起对 MSA 等突触核蛋白病的临床怀疑。未经治疗的 MSA 患者在夜间出现喘鸣和呼吸障碍时,有发生猝死的风险增加。因此,运动障碍专家的早期评估可及时诊断 MSA,这可能对高危患者人群的发病率和死亡率产生重大影响。

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