Xue Xin, Bao Yingcun, Yu Yakun, Li Qun, Liu Mei, Li Xiaoling
Department of Rehabilitation Medicine, Second Clinical College, Lanzhou University, Lanzhou University Second Hospital, Lanzhou, China.
Department of Rheumatology and Immunology, Second Clinical College, Lanzhou University, Lanzhou University Second Hospital, Lanzhou, China.
Front Immunol. 2025 Jan 17;15:1517943. doi: 10.3389/fimmu.2024.1517943. eCollection 2024.
Guillain-Barré syndrome (GBS) is a rare, immune-mediated polyneuropathy primarily affecting the peripheral nervous system. Diagnosis is often supported by neuroconduction studies showing polyradiculoneuropathy and cerebrospinal fluid (CSF) analysis revealing albumin-cytological dissociation. However, these features may not appear in the early stages, leading to potential misdiagnosis. The central nervous system (CNS) is generally not affected due to differences in antigen expression, optic neuritis (ON), a demyelinating inflammation of the optic nerve, occasionally co-occurs with GBS as a rare variant. Although GBS can manifest with various neurological symptoms, the co-occurrence of optic neuritis (ON) is rare, especially in pediatric cases. This report documents the first known case in China of a child with severe GBS complicated by ON, which developed following an upper respiratory infection.
A 14-year-old male presented with acute progressive quadriparesis and visual impairment following a febrile illness. On admission, he displayed severe respiratory and autonomic instability requiring mechanical ventilation. Neurological examination revealed flaccid paralysis of all four limbs with absent reflexes, along with bilateral optic neuritis, confirmed by MRI showing inflammation of the optic nerve. Initial cerebrospinal fluid (CSF) analysis was normal, but subsequent testing revealed elevated protein levels typical of GBS. Neurophysiological studies indicated widespread demyelinating and axonal damage.
The patient received intravenous immunoglobulin (IVIG) therapy, high-dose corticosteroids, and, given the severe progression, rituximab. Despite initial worsening, gradual improvement in muscle strength and visual acuity was observed over several weeks. At three months, the patient was discharged with significantly restored function, with muscle strength nearing baseline and partial visual recovery.
This case highlights the clinical complexity of GBS with ON in pediatric patients, emphasizing the importance of timely immunomodulatory treatment. It also underscores the need for awareness of overlapping central and peripheral autoimmune neuropathies to improve diagnostic accuracy and patient outcomes.
吉兰 - 巴雷综合征（GBS）是一种罕见的、免疫介导的多发性神经病，主要影响周围神经系统。神经传导研究显示多神经根神经病，脑脊液（CSF）分析显示蛋白 - 细胞分离，这些通常有助于诊断。然而，这些特征在疾病早期可能不出现，从而导致潜在的误诊。由于抗原表达的差异，中枢神经系统（CNS）一般不受影响，但视神经炎（ON），一种视神经的脱髓鞘炎症，作为一种罕见的变异型偶尔与GBS同时发生。虽然GBS可表现出各种神经症状，但视神经炎（ON）的同时出现很少见，尤其是在儿科病例中。本报告记录了中国首例已知的患有严重GBS并伴有ON的儿童病例，该病例在上呼吸道感染后发病。
一名14岁男性在发热性疾病后出现急性进行性四肢瘫和视力障碍。入院时，他表现出严重的呼吸和自主神经功能不稳定，需要机械通气。神经检查发现四肢弛缓性瘫痪，反射消失，同时伴有双侧视神经炎，MRI显示视神经炎症证实了这一点。最初的脑脊液（CSF）分析正常，但随后的检测显示出GBS典型的蛋白水平升高。神经生理学研究表明存在广泛的脱髓鞘和轴突损伤。
患者接受了静脉注射免疫球蛋白（IVIG）治疗、大剂量皮质类固醇治疗，鉴于病情严重进展，还接受了利妥昔单抗治疗。尽管最初病情恶化，但在数周内观察到肌肉力量和视力逐渐改善。三个月时，患者出院，功能显著恢复，肌肉力量接近基线水平，视力部分恢复。
本病例突出了儿科患者GBS合并ON的临床复杂性，强调了及时进行免疫调节治疗的重要性。它还强调了认识中枢和周围自身免疫性神经病重叠情况对于提高诊断准确性和患者预后的必要性。
