Coomes Eric Anthony, Haghbayan Hourmazd, Spring Jenna, Mehta Sangeeta
Department of Medicine, University of Toronto, Toronto, Ontario, Canada.
Interdepartmental Division of Critical Care Medicine, University of Toronto, Toronto, Ontario, Canada.
BMJ Case Rep. 2019 Jan 14;12(1):bcr-2018-226634. doi: 10.1136/bcr-2018-226634.
A 45-year-old man with a history of systemic lupus erythematosus presented with progressive weakness and areflexia. Electromyogram revealed reduced motor and sensory amplitudes without demyelinating features. He was clinically diagnosed with the acute motor and sensory axonal neuropathy variant of Guillain-Barré syndrome. Despite intravenous immunoglobulin therapy, he deteriorated with loss of all voluntary motor function and cranial nerve reflexes. Concomitant investigations revealed class V lupus nephritis. Therapy was initiated with plasma exchange, glucocorticoids and further immunosuppression, with gradual neurological recovery. We present the first documented case of fulminant Guillain-Barré syndrome as a neuropsychiatric manifestation of systemic lupus erythematosus, highlighting how immune-mediated polyneuropathy via diffuse deafferentation may mimic the outward appearance of brain death. While glucocorticoids are not indicated in idiopathic Guillain-Barré, when this neurological disorder is a consequence of systemic lupus erythematosus, immunomodulatory treatment should be initiated to prevent neurological deterioration.
一名有系统性红斑狼疮病史的45岁男性,出现进行性肌无力和腱反射消失。肌电图显示运动和感觉波幅降低,无脱髓鞘特征。他临床诊断为吉兰-巴雷综合征急性运动和感觉轴索性神经病变异型。尽管接受了静脉注射免疫球蛋白治疗,但他仍病情恶化,所有自主运动功能和脑神经反射丧失。同时检查发现为Ⅴ型狼疮性肾炎。开始采用血浆置换、糖皮质激素及进一步免疫抑制治疗,患者神经功能逐渐恢复。我们报告了首例作为系统性红斑狼疮神经精神表现的暴发性吉兰-巴雷综合征病例,强调了通过弥漫性传入神经阻滞的免疫介导性多发性神经病如何可能模拟脑死亡的外在表现。虽然糖皮质激素在特发性吉兰-巴雷综合征中并无指征,但当这种神经系统疾病是系统性红斑狼疮的结果时,应启动免疫调节治疗以防止神经功能恶化。