Hsu Tai-Yi, Wang Shih-Hao, Kuo Chang-Fu, Chiu Te-Fa, Chang Yu-Che
Department of Emergency Medicine, Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Tao-Yuan, Taiwan.
Am J Emerg Med. 2009 Sep;27(7):900.e3-5. doi: 10.1016/j.ajem.2008.11.006.
A 28-year-old woman presented at the emergency department (ED) with acute bilateral leg weakness and lost the ability to walk 1 day after noticing bilateral leg numbness and intermittent pleuritic pain for 3 weeks. Physical examination revealed bilateral decreased muscle strength with score 4 symmetrically in the legs and decreased deep tendon reflex. Laboratory tests indicated lymphopenia but no other grossly remarkable findings. Cerebrospinal fluid analysis demonstrated albuminocytologic dissociation. Electrophysiologic survey also indicated sensory-motor demyelinating polyneuropathy. The clinical picture was compatible with acute inflammatory demyelinating polyneuropathy (AIDP), the most prevalent form of Guillain-Barré syndrome (GBS). Plasma exchange was immediately arranged. After serial examination, pleuritic pain, persistent lymphopenia, positive immunologic findings of anti-DNA, and antinuclear antibodies led to a diagnosis of systemic lupus erythematosus (SLE). Prednisolone was added along with plasma exchange. The patient was able to walk after 2 weeks of therapy. Acute inflammatory demyelinating polyneuropathy presenting as the initial manifestation of SLE is rather rare. The precise mechanism of SLE-related AIDP remains unclear but is probably immune related. Although steroids are not recommended in the management of AIDP or GBS, patients with SLE-related AIDP may benefit from steroid therapy. This case highlights that early initiation of evaluation for SLE by ED physicians may facilitate correct diagnosis and better outcomes in patients presenting with GBS or AIDP in the ED.
一名28岁女性因急性双侧腿部无力就诊于急诊科。在出现双侧腿部麻木和间歇性胸膜炎性疼痛3周后，她于1天前失去了行走能力。体格检查发现双侧腿部肌肉力量对称减弱，评分为4级，深部腱反射减弱。实验室检查显示淋巴细胞减少，但无其他明显异常发现。脑脊液分析显示蛋白细胞分离。电生理检查也提示感觉运动性脱髓鞘性多发性神经病。临床表现符合急性炎症性脱髓鞘性多发性神经病（AIDP），这是吉兰-巴雷综合征（GBS）最常见的形式。立即安排了血浆置换。经过一系列检查，胸膜炎性疼痛、持续性淋巴细胞减少、抗DNA和抗核抗体的阳性免疫学结果导致诊断为系统性红斑狼疮（SLE）。在进行血浆置换的同时加用了泼尼松龙。治疗2周后患者能够行走。以SLE的初始表现形式出现的急性炎症性脱髓鞘性多发性神经病相当罕见。SLE相关AIDP的确切机制尚不清楚，但可能与免疫有关。虽然在AIDP或GBS的治疗中不推荐使用类固醇，但SLE相关AIDP患者可能从类固醇治疗中获益。该病例强调，急诊科医生早期启动对SLE的评估可能有助于对急诊科出现GBS或AIDP的患者做出正确诊断并取得更好的治疗效果。
