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Gerstmann-Sträussler-Scheinker 病伴 Pro105Leu 患者的吞咽功能评估:病例报告。

Swallowing Function Evaluation in a Patient with Gerstmann-Sträussler-Scheinker Disease with Pro105Leu: A Case Report.

机构信息

Department of Dysphagia Rehabilitation, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo 113-8549, Japan.

出版信息

Int J Environ Res Public Health. 2021 Sep 15;18(18):9734. doi: 10.3390/ijerph18189734.

Abstract

Gerstmann-Sträussler-Scheinker disease (GSS) is a genetic prion disease. Swallowing function evaluation in patients with GSS remains unclear. Here, we describe a case of videofluoroscopic examination of swallowing (VF) to facilitate continued oral ingestion in a patient with P105L GSS. A 67-year-old woman developed gait disturbance and Parkinsonism symptoms at the age of 54 years. Since her family wanted her to continue oral ingestion, we performed VF, which revealed impairment and preservation of the oral and pharyngeal phases, respectively. Moreover, the impairment of the oral phase was improved by adjusting the patient's posture and food consistency. A swallowing function evaluation based on the condition of a patient with GSS may facilitate continued oral ingestion.

摘要

Gerstmann-Straussler-Scheinker 病(GSS)是一种遗传性朊病毒病。GSS 患者的吞咽功能评估仍不清楚。在这里,我们描述了一例使用视频透视吞咽检查(VF)来促进 P105L GSS 患者继续口服摄入的病例。一名 67 岁女性在 54 岁时出现步态障碍和帕金森病症状。由于她的家人希望她继续口服摄入,我们进行了 VF,结果分别显示口腔和咽部阶段的损害和保留。此外,通过调整患者的姿势和食物稠度,口腔阶段的损害得到改善。基于 GSS 患者的病情进行吞咽功能评估可能有助于继续口服摄入。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b5fc/8466103/5588ed29e3cf/ijerph-18-09734-g001.jpg

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