Yang Huijia, Jing Xiaozhong, Yan Jinhua, Ma Dihui
Department of Neurology, the First Hospital of Jilin University, Changchun, China.
Department of Neurology, Shanghai Tong-Ren Hospital, Shanghai Jiao-Tong University School of Medicine, Shanghai, China.
J Int Med Res. 2020 Nov;48(11):300060520974465. doi: 10.1177/0300060520974465.
Sjögren's syndrome is an autoimmune disease that can affect multiple systems. Sjögren's syndrome with motor neuron disease is rarely reported. Herein, we describe a patient with rapidly progressive motor neuron disease secondary to Sjögren's syndrome. A 42-year-old woman was admitted to our hospital with a 2-month history of progressive limb weakness. Neurological assessment revealed fasciculation in the lower limbs and amyotrophy in the bilateral supraspinatus, interosseous, and thenar muscles. Serological examination and labial gland biopsy revealed Sjögren's syndrome. In addition, electromyography demonstrated neurogenic damage to the upper and lower limbs. The patient received a short course of high-dose corticosteroids, intravenous immunoglobulins, and immunosuppressant treatment, including a weekly dose of 0.4 g cyclophosphamide and a daily dose of 0.2 g hydroxychloroquine. However, the patient's limb weakness was further aggravated and her respiratory function was compromised. Electromyography re-examination demonstrated extensive neurogenic damage, and she was diagnosed with Sjögren's syndrome with motor neuron disease. The patient died of respiratory failure after 2 months. We suggest that more effective maintenance treatments should be sought. Further investigation is required to elucidate the association between autoimmune motor neuron disease and Sjögren's syndrome.
干燥综合征是一种可累及多个系统的自身免疫性疾病。干燥综合征合并运动神经元病鲜有报道。在此，我们描述一例继发于干燥综合征的快速进展性运动神经元病患者。一名42岁女性因进行性肢体无力2个月入住我院。神经系统评估发现下肢肌束震颤以及双侧冈上肌、骨间肌和鱼际肌萎缩。血清学检查和唇腺活检确诊为干燥综合征。此外，肌电图显示上下肢神经源性损害。患者接受了短期大剂量皮质类固醇、静脉注射免疫球蛋白及免疫抑制剂治疗，包括每周一次剂量为0.4 g的环磷酰胺和每日一次剂量为0.2 g的羟氯喹。然而，患者肢体无力进一步加重，呼吸功能受损。再次肌电图检查显示广泛神经源性损害，她被诊断为干燥综合征合并运动神经元病。2个月后患者死于呼吸衰竭。我们建议应寻求更有效的维持治疗方法。需要进一步研究以阐明自身免疫性运动神经元病与干燥综合征之间的关联。
