Chiari畸形Ⅰ合并神经源性吞咽功能障碍的临床特征（英文）

Clinical characteristics of neurogenic dysphagia in adult patients with Chiari malformation typeⅠ

目的:成年Chiari畸形Ⅰ型(chiari malformation typeⅠ,CMⅠ)患者中神经源性吞咽障碍的发生率并不少见,临床上罕有关于食管高分辨率测压(high-resolution manometry,HRM)技术对CMⅠ患者吞咽功能的精确评价的报道。本研究拟通过HRM量化评价CMⅠ患者吞咽功能,初步探讨影响吞咽功能的可能相关因素及其机制。方法:共纳入北京大学第三医院2010年1月至2015年7月收治的42例经临床和MRI检查、确诊不合并寰枢椎脱位的CMⅠ患者,将患者分为吞咽障碍组20例和不合并吞咽障碍组22例。所有患者均接受HRM检查,收集所有患者的临床、影像学资料和HRM评价参数,并做统计学分析。结果:(1)合并吞咽障碍的女性CMⅠ患者比例明显高于不合并吞咽障碍组(14/20 vs.8/22,P=0.029),吞咽障碍组后组颅神经损伤的其他症状(包括声音嘶哑、咽部感觉减退、患侧面部感觉减退及汗液分泌减少等)发生率明显高于不合并吞咽障碍对照组(15/20 vs.5/22,P=0.01)。(2)HRM显示吞咽障碍组食管上括约肌(upper esophageal sphincter,UES)松弛比高于对照组(75.3%vs.63.1%,P=0.023),UES上缘亦高于对照组(17.2 cm vs.15.7 cm,P=0.005)。(3)吞咽障碍组MRI影像上延髓或上颈部的脊髓空洞比例明显高于对照组(17/20 vs.7/22,P=0.001)。结论:CMⅠ患者的吞咽障碍经常与后组颅神经损伤、共济失调和阳性锥体束征相关,HRM显示平均UES松弛比例的差异亦有可能与延髓或上颈髓的脊髓空洞症有关,CMⅠ中的吞咽障碍的机制可能是由于神经源性咽肌运动障碍所导致,吞咽障碍病因学检查应包括CMⅠ畸形鉴别诊断。

Objective：To investigate changes of swallowing function and associated symptoms in Chiari malformation typeⅠ （CMⅠ） patients with and without dysphagia by the analysis of their clinical and high-resolution manometry （HRM） parameters.Methods： A total of 42 patients diagnosed with symptomatic CMI without atlantoaxial dislocations which were confirmed by clinical manifestations and magne-tic resonance imaging（MRI） findings between January 2010 and July 2015 at Peking University Third Hospital were included in this study.Twenty patients had a history of various dysphagia symptoms,or reported symptoms of choking,coughing after eating or drinking,while the other 22 patients denied symptoms of dysphagia.The data collected from the medical records of these patients included the patient＆#39;s age,sex,date of diagnosis,duration of illness,symptoms,results of MRI and HRM,and date of sur-gery.Results： （1） Dysphagia group had 14 female patients,and no-dysphagia group had 8 female patients.Dysphagia usually occurred in female patients,and in addition to dysphagia,we recorded other symptoms and signs in the CMⅠ patients,including numbness,hypoesthesia,limb weakness,neck pain,muscle atrophy,ataxia,hoarseness,symptoms caused by posterior cranial nerve damage,pharyngeal reflex,uvula deviation,and pyramidal signs.A higher percentage of the CMⅠ patients with dysphagia （15/20） had symptoms of posterior cranial nerve damage compared with the control group （5/22;P=0.01）.（2）HRM showed a significant difference in upper esophageal sphincter （UES） relax ratio measurement （75.3% vs.63.1%,P=0.023） and UES proximal margin （17.2 cm vs.15.7 cm,P=0.005） between the two groups.（3） The percentage of syringomyelia affecting the bulbar or upper cervical region on MRI was significantly higher in the dysphagia group （17/20 vs.7/22,P=0.001）.Conclusion： CMⅠ was usually accompanied by symptoms caused by posterior cranial nerve damage,ataxia,and positive pyramidal signs.Location of the syringomyelia affecting specifically the bulbar or upper cervical region was associated with dysphagia in CMⅠ patients.These findings suggest that the mechanism of dysphagia in CMⅠ may be due to a dysfunction in the neurological pathway of pharyngeal muscle movement.Dysphagia etiology work-up should include CMⅠ in the differential diagnosis.