Worster-Drought syndrome with progressive symptomatic improvement in early infancy.

IF 0.6 Q3 MEDICINE, GENERAL & INTERNAL
Kota Inoue, Shinji Nakamura, Kosuke Koyano, Takashi Kusaka
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引用次数: 0

Abstract

Worster-Drought syndrome (WDS), or congenital suprabulbar paresis, is characterised by congenital dysarthria, dysphagia and other pseudobulbar paresis without structural abnormalities around the Sylvian fissure on imaging. This rare syndrome is challenging to diagnose, particularly in preterm infants. This report describes a low-birth-weight female infant with WDS who had no sucking reflex from birth, airway obstruction due to saliva retention and muscle rigidity, who was diagnosed with the syndrome at a postconceptional age (PCA) of 1 month. She was discharged with only home oxygen therapy as respiratory support at a PCA of 3 months after gradual improvement in her clinical symptoms. Diagnosis of WDS is difficult in the early postnatal period in preterm cases owing to prematurity but should be suspected when bulbar palsy, including absence of the sucking reflex, persistent dysphagia and obstructed breathing, persists beyond a PCA of 40 weeks and when muscle stiffness is present.

婴儿早期症状进行性改善的严重-干旱综合征。
Worster-Drought综合征(WDS),又称先天性球上轻瘫,以先天性构音障碍、吞咽困难和其他假性球上轻瘫为特征,影像学上无Sylvian裂周围结构异常。这种罕见的综合征很难诊断,尤其是早产儿。本报告描述了一例低出生体重的WDS女婴,她出生时没有吮吸反射,由于唾液潴留和肌肉僵硬导致气道阻塞,在1个月的孕后年龄(PCA)被诊断为WDS综合征。在临床症状逐渐改善3个月后,她出院时仅接受家庭氧气治疗作为呼吸支持。由于早产,在产后早期诊断WDS是困难的,但当球麻痹,包括没有吸吮反射,持续吞咽困难和呼吸困难,持续超过40周,当肌肉僵硬存在时,应该怀疑。
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来源期刊
BMJ Case Reports
BMJ Case Reports Medicine-Medicine (all)
CiteScore
1.40
自引率
0.00%
发文量
1588
期刊介绍: BMJ Case Reports is an important educational resource offering a high volume of cases in all disciplines so that healthcare professionals, researchers and others can easily find clinically important information on common and rare conditions. All articles are peer reviewed and copy edited before publication. BMJ Case Reports is not an edition or supplement of the BMJ.
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