Department of Neurology, National Center Hospital, National Center of Neurology and Psychiatry, 4-1-1 Ogawahigashi, Kodaira, Tokyo, 187-8551, Japan.
Department of Neurology, Dysphagia Research Center, National Center Hospital, National Center of Neurology and Psychiatry, 4-1-1 Ogawahigashi, Kodaira, Tokyo, 187-8551, Japan.
J Neurol. 2021 Mar;268(3):1016-1024. doi: 10.1007/s00415-020-10241-7. Epub 2020 Sep 26.
To determine the prevalence and characteristics of the cricopharyngeal bar (CPB), defined as marked protrusion with lacking relaxation and stricture of the upper esophageal sphincter on videofluoroscopy, in patients with inclusion body myositis (IBM).
We conducted a case-control study of comprehensive series of adult healthy individuals and consecutive patients with neuropsychiatric disorders aged over 45 (52 versus 2486). A standard videofluoroscopy was performed.
Overall, 47 individuals with CPB were identified. Of the individuals with CPB, 36% were IBM followed by neurodegenerative disorders, muscular disorders, neuromuscular disorders, and others (32%, 21%, 2.1%, and 8.5%, respectively), indicating the heterogeneity of the etiologies. Against muscular disorders, the sensitivity and specificity of the CPB for IBM were 33% (= 17/52; 95% confidence interval [CI], 20-45%) and 96% (= 264/274; 95% CI, 94-99%), respectively. IBM with CPB showed a higher frequency of obstruction-related dysphagia (88% versus 22%, p < 0.001) and severe CPB (76% versus 23%, p < 0.001) than the control with one. The ratio of the upper esophageal distance at the maximum distension at the level of C6 to that of C4 was lower in IBM with CPB than in the controls with one (0.50 versus 0.77, p < 0.001), which suggests the insufficient opening of the upper esophageal sphincter.
A CPB could be indicative of IBM. The upper esophagus in IBM with CPB became narrow, like a bottleneck. We provide new perspectives of dysphagia diagnosis by videofluoroscopy, especially for IBM-associated dysphagia, to expand the knowledge on the CPB.
确定在包涵体肌炎（IBM）患者中，通过视频荧光透视检查发现的会厌襞（CPB）的患病率和特征，其定义为上食管括约肌明显突出、缺乏松弛和狭窄。
我们对一系列成年健康个体和年龄超过 45 岁的连续神经精神障碍患者（52 例与 2486 例）进行了一项病例对照研究。进行了标准的视频荧光透视检查。
总体上，确定了 47 例 CPB 患者。在 CPB 患者中，36%为 IBM，其次为神经退行性疾病、肌肉疾病、神经肌肉疾病和其他疾病（分别为 32%、21%、2.1%和 8.5%），表明病因具有异质性。与肌肉疾病相比，CPB 对 IBM 的敏感性和特异性分别为 33%（=52 例中的 17 例；95%置信区间[CI]，20-45%）和 96%（=274 例中的 264 例；95%CI，94-99%）。CPB 伴 IBM 的梗阻相关吞咽困难发生率（88%与 22%，p<0.001）和严重 CPB 发生率（76%与 23%，p<0.001）均高于无 CPB 的对照组。CPB 伴 IBM 的上食管距离在 C6 水平最大扩张时与 C4 时的比值低于对照组（0.50 与 0.77，p<0.001），这表明上食管括约肌的开放不足。
CPB 可能提示 IBM。CPB 伴 IBM 的上食管变窄，呈瓶颈状。我们通过视频荧光透视检查为吞咽困难的诊断提供了新的视角，特别是对于 IBM 相关的吞咽困难，扩展了对 CPB 的认识。
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