Lin Yu-Kai, Yang Fu-Chi, Liu Feng-Cheng, Lee Jiunn-Tay, Sung Yueh-Feng
Department of Neurology, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan.
Rheumatology/Immunology and Allergy, Department of Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan.
Acta Neurol Taiwan. 2016 Sep 15;25(3):83-87.
Co-occurrence of Guillain-Barré syndrome (GBS) and other autoimmune diseases is rare. We present the case of a patient with co-occurrence of GBS and primary Sjögren syndrome (pSS).
An 82-year-old woman presented with acute ascending flaccid paralysis and acute respiratory failure. Nerve conduction studies and cerebrospinal fluid analysis confirmed the diagnosis of GBS of acute inflammatory demyelinating polyradiculoneuropathy subtype. The initial unresponsiveness to plasma exchange therapy raised the suspicion of other potential diseases. She was later proved to have underlying pSS. Her neurological deficits and respiratory failure improved dramatically with combination therapy of intravenous immunoglobulin (IVIg) and immunosuppressive agent.
pSS should be considered as a possible cause of refractory GBS, particularly in elderly women. Combination therapy with IVIg and immunosuppressive agent may be beneficial.
吉兰-巴雷综合征（GBS）与其他自身免疫性疾病同时出现的情况较为罕见。我们报告了一例GBS与原发性干燥综合征（pSS）同时发生的患者病例。
一名82岁女性出现急性上行性弛缓性麻痹和急性呼吸衰竭。神经传导研究和脑脊液分析确诊为急性炎症性脱髓鞘性多发性神经根神经病亚型的GBS。最初对血浆置换疗法无反应引发了对其他潜在疾病的怀疑。后来证实她患有潜在的pSS。通过静脉注射免疫球蛋白（IVIg）和免疫抑制剂联合治疗，她的神经功能缺损和呼吸衰竭得到了显著改善。
pSS应被视为难治性GBS的可能病因，尤其是在老年女性中。IVIg和免疫抑制剂联合治疗可能有益。
