区分吉兰-巴雷综合征中的不良预后与治疗相关波动以决定是否重复免疫球蛋白治疗：一例说明性病例报告

文献信息

中文摘要

背景： 吉兰-巴雷综合征（GBS）是一种急性免疫介导的多发性神经根神经病，可继发于感染触发因素，并可进展为严重无力、颅神经受累、自主神经功能障碍和呼吸衰竭。巨细胞病毒（CMV）相关的GBS常与更严重的临床病程相关。尽管静脉注射免疫球蛋白（IVIG）和血浆置换是既定的一线治疗，但当患者在初始治疗后恶化时，管理变得具有挑战性，尤其是在区分不良预后与治疗相关波动（TRF）时。
病例介绍： 我们报告一例42岁女性，出现进行性感觉异常、弥漫性疼痛、步态障碍、反射消失、面部受累、吞咽困难、构音障碍、自主神经功能障碍，随后出现呼吸衰竭。脑脊液分析显示蛋白细胞分离，电诊断研究支持急性多发性神经根神经病，MRI显示面神经根和马尾神经对比增强。血清检测显示CMV IgM阳性并可检测到CMV DNA，符合CMV相关GBS。患者接受了IVIG和更昔洛韦治疗。在首次IVIG疗程后初步稳定后，她出现突发呼吸恶化，需要插管。鉴于先稳定后恶化的时间模式，该次发作被解释为发生在具有不良预后特征的患者中的TRF。给予第二个IVIG疗程，随后呼吸和神经系统逐渐改善。
讨论： 本病例说明了在严重GBS中决定重复免疫治疗是否合理的临床困难。当前证据不鼓励对治疗无反应的不良预后患者常规进行第二个IVIG疗程；然而，该建议不一定适用于TRF，在TRF中可考虑重新进行免疫治疗。在该患者中，IVIG后稳定随后急性恶化支持TRF的解释；持续性CMV病毒血症被认为是持续免疫刺激的可能标志。
结论： CMV相关GBS可能呈严重且波动的病程。仔细区分不良预后、无反应和治疗相关波动至关重要，因为这直接影响治疗决策。密切监测、个体化免疫治疗、强化支持治疗和早期康复仍然是优化严重GBS结局的核心。

英文摘要

BACKGROUND: Guillain-Barré syndrome (GBS) is an acute immune-mediated polyradiculoneuropathy that may follow infectious triggers and can progress to severe weakness, cranial nerve involvement, autonomic dysfunction, and respiratory failure. Cytomegalovirus (CMV)-associated GBS is often linked to a more severe clinical course. Although intravenous immunoglobulin (IVIG) and plasma exchange are established first-line treatments, management becomes challenging when patients deteriorate after initial therapy, particularly when distinguishing poor prognosis from treatment-related fluctuation (TRF).
CASE PRESENTATION: We report the case of a 42-year-old woman who developed progressive paresthesias, diffuse pain, gait disturbance, areflexia, facial involvement, dysphagia, dysarthria, autonomic dysfunction, and later respiratory failure. Cerebrospinal fluid analysis showed albuminocytologic dissociation, electrodiagnostic studies supported acute polyradiculoneuropathy, and MRI demonstrated contrast enhancement of the facial nerve roots and cauda equina. Serum testing revealed CMV IgM positivity with detectable CMV DNA, consistent with CMV-associated GBS. The patient received IVIG and ganciclovir. After initial stabilization following the first IVIG course, she experienced sudden respiratory deterioration requiring intubation. Given the temporal pattern of stabilization followed by worsening, the episode was interpreted as TRF occurring in a patient with otherwise poor prognostic features. A second IVIG cycle was administered, followed by gradual respiratory and neurological improvement.
DISCUSSION: This case illustrates the clinical difficulty of deciding whether repeated immunotherapy is justified in severe GBS. Current evidence discourages routine second IVIG courses in patients with poor prognosis who fail to respond; however, this recommendation does not necessarily apply to TRF, where renewed immunotherapy may be considered. In this patient, post-IVIG stabilization followed by acute deterioration supported the interpretation of TRF; persistent CMV viremia was considered a possible marker of ongoing immune stimulation.
CONCLUSION: CMV-associated GBS may follow a severe and fluctuating course. Careful distinction between poor prognosis, nonresponse, and treatment-related fluctuation is essential because it directly influences therapeutic decision-making. Close monitoring, individualized immunotherapy, intensive supportive care, and early rehabilitation remain central to optimizing outcomes in severe GBS.