Shenoy Niraj, Tesfaye Melaku, Brown Joshua, Simmons Nichelle, Weiss Deborah, Meholli Mimoza, Mabie Peter
Department of Medicine, Jacobi Medical Center, Albert Einstein College of Medicine, Bronx, New York, USA.
Pract Neurol. 2015 Aug;15(4):289-92. doi: 10.1136/practneurol-2015-001112. Epub 2015 May 2.
We report an intriguing case of corticosteroid-resistant bulbar neurosarcoidosis responding to intravenous immunoglobulin. A 37-year-old man presented with dysphagia to solids and liquids, dysphonia, fatigue and 50 lb weight loss over 2 months. We suspected sarcoidosis, based on an elevated serum angiotensin-converting enzyme concentration and hilar lymphadenopathy on chest imaging; we subsequently confirmed this after transbronchial biopsy found non-caseating granulomas. MR scan of brain was normal; barium swallow showed severe oropharyngeal dysphagia and electromyography identified bulbar muscle denervation. He took corticosteroids for 3 weeks without improvement, requiring a percutaneous endoscopic gastrostomy tube for nutrition, but then he promptly improved with a 2-day course of intravenous immunoglobulin. Although there have been a few reports of intravenous immunoglobulin helping peripheral neurosarcoidosis, this case suggests that it also helps bulbar neurosarcoidosis. This case shows that bulbar neurosarcoidosis can mimic the clinical and electrophysiological features of fatal neurological disorders such as progressive bulbar palsy. The case illustrates the diagnostic challenge particularly when imaging is inconclusive and there is no response to corticosteroids. It also suggests that intravenous immunoglobulin can be considered before cytotoxic therapy for corticosteroid-resistant neurosarcoidosis, particularly in decompensated patients, given its favourable side effect profile. We also review the literature on bulbar neurosarcoidosis.
我们报告了一例对皮质类固醇耐药的延髓型神经结节病，静脉注射免疫球蛋白后有反应。一名37岁男性出现吞咽固体和液体困难、发音障碍、疲劳，且在2个月内体重减轻了50磅。基于血清血管紧张素转换酶浓度升高及胸部影像学显示肺门淋巴结肿大，我们怀疑为结节病；经支气管活检发现非干酪样肉芽肿后，我们随后确诊了该病。脑部磁共振扫描正常；吞钡检查显示严重的口咽吞咽困难，肌电图检查发现延髓肌肉失神经支配。他服用皮质类固醇3周，病情无改善，需要经皮内镜下胃造瘘管补充营养，但随后静脉注射免疫球蛋白2天疗程后病情迅速改善。虽然已有一些关于静脉注射免疫球蛋白有助于治疗周围型神经结节病的报道，但该病例表明其对延髓型神经结节病也有帮助。该病例表明，延髓型神经结节病可模仿进行性延髓麻痹等致命性神经系统疾病的临床和电生理特征。该病例说明了诊断上的挑战，尤其是在影像学检查结果不明确且对皮质类固醇无反应时。这也表明，对于皮质类固醇耐药的神经结节病，特别是失代偿患者，鉴于其良好的副作用 profile，在进行细胞毒性治疗前可考虑使用静脉注射免疫球蛋白。我们还回顾了关于延髓型神经结节病的文献。
