Chen Xi-Yuan, Ren Zhuo-Chao, Huang Xiao-Jun
Department of Respiratory Medicine, Zhejiang Provincial People's Hospital (People's Hospital of Hangzhou Medical College), Hangzhou, China.
Medicine (Baltimore). 2018 Dec;97(50):e13667. doi: 10.1097/MD.0000000000013667.
Sarcoidosis is a multisystem disorder characterized by noncaseating granulomas. The nervous system is involved in 5 to 16% of the patients. However, neurosarcoidosis in the medulla oblongata presenting as hiccough is remarkably rare.
A 55-year-old female was admitted to our hospital suffering from intractable hiccough and progressive numbness of extremities.
The MR imaging revealed a circumscribed mass lesion located on the medulla oblongata. The mass was hyperintense on T2-weighted images and enhanced homogeneously with gadolinium-diethylenetriamine penta-acetic acid. The cerebrospinal fluid analysis showed a moderately elevated protein content and a significant lymphocytosis 86.5%. Electrocardiogram (ECG) showed complete atrioventricular block. Bilateral supraclavicular, hilar, and mediastinal lymphadenopathy was diagnosed in a CT scan. Transbranchial needle aspiration biopsy revealed noncaseating granuloma consisting of epithelioid cells, lymphocytes, and rare multinucleated giant cells which was consistent with sarcoidosis. The diagnosis of multisystemic sarcoidosis was made.
The patient underwent a permanent pacemaker insertion, and was successfully treated with corticosteroids.
It is important to consider neurosarcoidosis in the differential diagnosis of intramedullary lesion, since a right recognition may lead to appropriate treatment with steroids and avoid needlessly extensive surgery.
结节病是一种以非干酪样肉芽肿为特征的多系统疾病。5%至16%的患者会累及神经系统。然而,以呃逆为表现的延髓神经结节病极为罕见。
一名55岁女性因顽固性呃逆和四肢进行性麻木入院。
磁共振成像显示延髓有一个边界清晰的肿块病变。该肿块在T2加权图像上呈高信号,注射钆喷酸葡胺后均匀强化。脑脊液分析显示蛋白含量中度升高,淋巴细胞显著增多(86.5%)。心电图显示完全性房室传导阻滞。CT扫描诊断为双侧锁骨上、肺门和纵隔淋巴结病。经支气管针吸活检显示由上皮样细胞、淋巴细胞和罕见的多核巨细胞组成的非干酪样肉芽肿,与结节病相符。诊断为多系统结节病。
患者接受了永久性起搏器植入,并成功接受了皮质类固醇治疗。
在鉴别诊断髓内病变时考虑神经结节病很重要,因为正确认识可能会导致使用类固醇进行适当治疗,并避免不必要的广泛手术。