A case report of Isaacs' syndrome with treatment-resistant pain responsive to rituximab.

IF 0.9 4区医学 Q4 CLINICAL NEUROLOGY

Ideggyogyaszati Szemle-Clinical Neuroscience Pub Date : 2025-05-30 DOI:10.18071/isz.78.0214

Berin Inan, Ulkuhan Duzgun, Semih Mert Kilic, Omer Karadas, Zeki Odabasi

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引用次数: 0

Abstract

Isaacs' syndrome is an immune-mediated primary peripheral nerve hyperexcitability syndrome characterized by muscle twitches, cramps, and muscle stiffness. In 30-50% of cases, antibodies against voltage-gated potassium channel (VGKC) complex proteins are present. Symptomatic treatment, intravenous immunoglobulin (IVIg), plasma exchange (PE), and intravenous methylprednisolone (IVMP) are generally effective in controlling symptoms. However, some cases remain treatment-refractory. We present a 52-year-old female patient with walking difficulty, fasciculations, and severe back and hip pain. The patient was positive for anti-VGKC antibodies with a history of thymoma. Symptomatic treatment, highdose IVMP, IVIg and PE treatments improved the patient's gait partially but failed to control the pain and fasciculations. Eventually, we treated the patient with rituximab (RTX), because of its effect on antibody-mediated autoimmunity, resulting in complete symptomatic relief without side effects. RTX is a safe and effective option for controlling symptoms in treatment-refractory cases with Isaacs' syndrome.

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对利妥昔单抗有反应的艾萨克斯综合征伴难治性疼痛1例报告。

艾萨克斯综合征是一种免疫介导的原发性周围神经兴奋性亢进综合征，以肌肉抽搐、痉挛和肌肉僵硬为特征。在30-50%的病例中，存在针对电压门控钾通道（VGKC）复合物蛋白的抗体。对症治疗、静脉注射免疫球蛋白（IVIg）、血浆置换（PE）和静脉注射甲基强的松龙（IVMP）通常能有效控制症状。然而，有些病例仍然难治性。我们报告一位52岁的女性患者，行走困难，抽搐，严重的背部和臀部疼痛。患者抗vgkc抗体阳性，既往有胸腺瘤病史。对症治疗、高剂量IVMP、IVIg和PE治疗部分改善了患者的步态，但未能控制疼痛和抽搐。最终，我们用利妥昔单抗（RTX）治疗患者，因为它对抗体介导的自身免疫有作用，导致症状完全缓解，没有副作用。RTX是治疗难治性艾萨克综合征患者控制症状的安全有效的选择。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Ideggyogyaszati Szemle-Clinical Neuroscience CLINICAL NEUROLOGY-NEUROSCIENCES

CiteScore

1.30

自引率

0.00%

发文量

审稿时长

>12 weeks

期刊介绍： The aim of Clinical Neuroscience (Ideggyógyászati Szemle) is to provide a forum for the exchange of clinical and scientific information for a multidisciplinary community. The Clinical Neuroscience will be of primary interest to neurologists, neurosurgeons, psychiatrist and clinical specialized psycholigists, neuroradiologists and clinical neurophysiologists, but original works in basic or computer science, epidemiology, pharmacology, etc., relating to the clinical practice with involvement of the central nervous system are also welcome.