Paraneoplastic Leucine Zipper 4 IgG Associated Motor-Predominant Polyradiculoneuropathy

IF 3.9 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Peripheral Nervous System Pub Date : 2025-03-18 DOI:10.1111/jns.70013

Alex Panrudkevich, Duaa Jabari, Brendan Putko, Catherine Daley, Richard A. Lewis, Divyanshu Dubey, Marcus V. Pinto

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Abstract

Background and Aims

Leucine Zipper 4 (LUZP4)-immunoglobulin G (IgG) is a novel antibody implicated in germ cell tumor-related paraneoplastic neurologic syndrome. We report a case of painful, progressive tetraparesis associated with testicular seminoma and LUZP4-IgG seropositivity.

Case Report

A 51-year-old male presented with a 5-week history of lower limb predominant progressive weakness. Nerve conduction studies (NCS) and electromyography (EMG) revealed a subacute axonal polyradiculoneuropathy. MRI lumbar spine showed thickening of the cauda equina and enhancement of the lower thoracic spinal cord/conus. Cerebrospinal fluid (CSF) showed a lymphocytic pleocytosis, three oligoclonal bands, and mildly elevated protein. Initial treatment with intravenous immunoglobulin (IVIG) and prednisone produced temporary improvement. CT targeted retroperitoneal lymph node biopsy revealed a seminoma, which was treated with orchiectomy and chemotherapy. The testicular tissue was consistent with a regressed germ cell tumor. His course was subsequently refractory to IVIG, prednisone, and plasma exchange. LUZP4 antibodies were detected in serum, prompting treatment with cyclophosphamide and prednisone. At the 4-month follow-up, the patient had significant improvement in hand strength and had transitioned from walker to cane.

Interpretation

LUZP4-IgG seropositivity and identification of retroperitoneal seminoma confirmed a paraneoplastic neurologic syndrome, which is a CD8+ T-cell-mediated disorder. Aggressive immunotherapy was initiated, resulting in clinical improvement. This case underscores the importance of identifying specific serologic biomarkers that can inform therapeutic decisions and improve outcomes.

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副肿瘤亮氨酸拉链4 IgG相关运动显性多根神经病变

背景与目的Leucine Zipper 4 (LUZP4)-免疫球蛋白G （IgG）是一种与生殖细胞肿瘤相关的副肿瘤神经系统综合征有关的新型抗体。我们报告一例疼痛，进行性全萎与睾丸精原细胞瘤和LUZP4-IgG血清阳性。病例报告一名51岁男性，以5周下肢进行性无力为主。神经传导研究（NCS）和肌电图（EMG）显示亚急性轴突多神经根神经病。腰椎MRI显示马尾增厚，胸椎下段脊髓/圆锥增强。脑脊液显示淋巴细胞增多，三个寡克隆带，轻度蛋白升高。最初静脉注射免疫球蛋白（IVIG）和强的松治疗产生了暂时的改善。CT靶向腹膜后淋巴结活检显示精原细胞瘤，并接受睾丸切除术和化疗。睾丸组织符合退行性生殖细胞瘤。随后对IVIG、强的松和血浆置换治疗难治。血清中检出LUZP4抗体，提示给予环磷酰胺和强的松治疗。在4个月的随访中，患者的手部力量有了明显的改善，并且已经从助行器过渡到手杖。LUZP4-IgG血清阳性和腹膜后精原细胞瘤的鉴定证实了一种副肿瘤神经系统综合征，这是一种CD8+ t细胞介导的疾病。积极的免疫治疗开始，导致临床改善。该病例强调了确定特定血清学生物标志物的重要性，这些标志物可以为治疗决策提供信息并改善结果。

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来源期刊

Journal of the Peripheral Nervous System 医学-临床神经学

CiteScore

6.10

自引率

7.90%

发文量

审稿时长

>12 weeks

期刊介绍： The Journal of the Peripheral Nervous System is the official journal of the Peripheral Nerve Society. Founded in 1996, it is the scientific journal of choice for clinicians, clinical scientists and basic neuroscientists interested in all aspects of biology and clinical research of peripheral nervous system disorders. The Journal of the Peripheral Nervous System is a peer-reviewed journal that publishes high quality articles on cell and molecular biology, genomics, neuropathic pain, clinical research, trials, and unique case reports on inherited and acquired peripheral neuropathies. Original articles are organized according to the topic in one of four specific areas: Mechanisms of Disease, Genetics, Clinical Research, and Clinical Trials. The journal also publishes regular review papers on hot topics and Special Issues on basic, clinical, or assembled research in the field of peripheral nervous system disorders. Authors interested in contributing a review-type article or a Special Issue should contact the Editorial Office to discuss the scope of the proposed article with the Editor-in-Chief.