Sulfatide antibody-mediated neuropathy: an analysis of clinical characteristics and immunotherapeutic responses.

IF 1.5 4区医学 Q3 CLINICAL NEUROLOGY

Neurological Research Pub Date : 2025-10-01 Epub Date: 2025-05-29 DOI:10.1080/01616412.2025.2509154

Jinglong Hu, Qi Zheng, Fanjing Zhou, Wenqian Gao, Yun Xu, Zhuo Liu, Meijuan Zhang

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引用次数: 0

Abstract

Background: Sulfatide antibodies, targeting glycosphingolipids linked to myelin phospholipids, play a key role in immune-mediated peripheral neuropathies. However, their clinical features and response to immunotherapy remain unclear.

Methods: A retrospective analysis of 19 sulfatide antibodies-mediated neuropathy cases from Nanjing Drum Tower Hospital (July 2019 to July 2024) assessed clinical scores (MRC, INCAT, HFGS) at admission, discharge, and 3-6 months post-discharge, with detailed documentation of clinical characteristics, electromyography findings, and treatment responses.

Results: A total of 19 patients were enrolled, including 10 Guillain-Barré syndrome (GBS, 53%) and 9 chronic inflammatory demyelinating polyneuropathy (CIDP, 47%) cases. All had distal limb weakness, with two patients (11%) showing central nervous system involvement. Electromyography revealed that anti-sulfatide antibody caused damage to both myelin and axons， not only in motor nerves but also in sensory nerves. Regarding treatment, 89% patients responded well to immunotherapy. However, one CIDP patient worsened with corticosteroids, and two patients died during follow-up. Compared to the sulfatide with poly-antibodies group, the sole sulfatide antibody group demonstrated more severe clinical symptoms, with lower MRC scores (p = 0.0140) and higher HFGS (p = 0.0052) and INCAT (p = 0.0057) scores. Consistently, the sole sulfatide antibody group presented lower nerve amplitudes (p = 0.0498) and conduction velocity (p < 0.05) as well. Interestingly, this group of patients showed greater improvement after immunotherapy (p < 0.05), particularly in GBS patients.

Conclusion: Sulfatide antibodies may attack peripheral nerves and the brainstem. The prognosis is relatively good for most patients by immunotherapy. Patients with sole sulfatide antibody exhibited more severe symptoms and electrophysiological issues but responded better to treatment.

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硫脂抗体介导的神经病变：临床特征和免疫治疗反应分析。

背景：针对与髓磷脂相关的鞘糖脂的硫脂抗体在免疫介导的周围神经病变中起关键作用。然而，它们的临床特征和对免疫治疗的反应尚不清楚。方法：回顾性分析南京鼓楼医院2019年7月至2024年7月收治的19例磺胺脂抗体介导的神经病患者入院、出院及出院后3-6个月的临床评分（MRC、INCAT、HFGS），详细记录患者的临床特征、肌电图表现及治疗效果。结果：共纳入19例患者，其中格林-巴勒综合征（GBS） 10例（53%），慢性炎性脱髓鞘性多神经病变（CIDP） 9例（47%）。所有患者均有远端肢体无力，其中2例（11%）显示中枢神经系统受累。肌电图显示，抗硫脂抗体不仅对运动神经，而且对感觉神经的髓磷脂和轴突均有损伤。在治疗方面，89%的患者对免疫疗法反应良好。然而，一名CIDP患者因皮质类固醇而恶化，两名患者在随访期间死亡。与磺胺脂多抗体组相比，单一磺胺脂抗体组表现出更严重的临床症状，MRC评分较低（p = 0.0140）， HFGS （p = 0.0052）和INCAT （p = 0.0057）评分较高。单抗组神经振幅较低（p = 0.0498），传导速度较慢（p = 0.0498）。结论：单抗可累及周围神经及脑干。多数患者经免疫治疗预后较好。单硫脂抗体患者表现出更严重的症状和电生理问题，但对治疗的反应更好。

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来源期刊

Neurological Research 医学-临床神经学

CiteScore

3.60

自引率

0.00%

发文量

116

审稿时长

5.3 months

期刊介绍： Neurological Research is an international, peer-reviewed journal for reporting both basic and clinical research in the fields of neurosurgery, neurology, neuroengineering and neurosciences. It provides a medium for those who recognize the wider implications of their work and who wish to be informed of the relevant experience of others in related and more distant fields. The scope of the journal includes: •Stem cell applications •Molecular neuroscience •Neuropharmacology •Neuroradiology •Neurochemistry •Biomathematical models •Endovascular neurosurgery •Innovation in neurosurgery.