Caspr1抗体自身免疫性副病伴严重四全:抗体滴度监测治疗反应的潜在相关性

IF 3.9 3区 医学 Q1 CLINICAL NEUROLOGY Journal of the Peripheral Nervous System Pub Date : 2023-05-29 DOI:10.1111/jns.12565
Lorenzo Bresciani, Alessandro Salvalaggio, Elisa Vegezzi, Andrea Visentin, Andrea Fortuna, Mariagiulia Anglani, Mario Cacciavillani, Stefano Masciocchi, Silvia Scaranzin, Miryam Carecchio, Andrea Martinuzzi, Matteo Gastaldi, Chiara Briani
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引用次数: 0

摘要

目的淋巴结病变和副淋巴结病变是与淋巴结-副淋巴结抗原(神经束蛋白140/186和155,接触蛋白1,接触蛋白相关蛋白1 [Caspr1])抗体相关的自身免疫性神经病变,其临床特征特殊,对标准免疫疗法(如静脉注射免疫球蛋白,IVIg)反应差。抗cd20单克隆抗体治疗后改善已有报道。关于Caspr1抗体致病性的数据仍然是初步的,纵向滴度的描述也很差。方法:我们报告了一位患有Caspr1/contactin-1复合物抗体的年轻女性,她在接受美罗华治疗后出现了显著的改善,抗体滴度下降。结果1例26岁女性患者表现为步态共济失调,四肢严重运动无力,伴有低频体位性震颤。由于脱髓鞘神经病变的神经生理学证据,她被诊断为慢性炎症性脱髓鞘性多根神经病变,并接受IVIg治疗,但没有效果。MRI显示臂丛、腰骶丛对称性肥大,信号明显增高。脑脊液显示蛋白710 mg/dL。尽管静脉注射甲基强的松龙,患者病情仍逐渐恶化,并成为轮椅。采用酶联免疫吸附试验(ELISA)和细胞法寻找结旁抗原抗体。抗接触蛋白/Caspr1 IgG4抗体阳性。患者接受了利妥昔单抗治疗,进展缓慢,反映了整个疾病过程中测量的抗体滴度。结论该患者病程严重,早期残疾,轴突损伤,抗体消耗治疗数月后恢复缓慢。滴度、致残性和治疗之间的密切相关性支持了Caspr1抗体的致病性,并提示其纵向评估可能为评估治疗反应提供潜在的生物标志物。
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Caspr1 antibodies autoimmune paranodopathy with severe tetraparesis: Potential relevance of antibody titers in monitoring treatment response

Aim

Nodopathies and paranodopathies are autoimmune neuropathies associated with antibodies to nodal–paranodal antigens (neurofascin 140/186 and 155, contactin-1, contactin-associated protein 1 [Caspr1]) characterized by peculiar clinical features, poor response to standard immunotherapies (e.g., intravenous immunoglobulins, IVIg). Improvement after anti-CD20 monoclonal antibody therapy has been reported. Data on Caspr1 antibodies pathogenicity are still preliminary, and longitudinal titers have been poorly described.

Methods

We report on a young woman who developed a disabling neuropathy with antibodies to the Caspr1/contactin-1 complex showing a dramatic improvement after rituximab therapy, mirrored by the decrease of antibody titers.

Results

A 26-year-old woman presented with ataxic-stepping gait, severe motor weakness at four limbs, and low frequency postural tremor. For neurophysiological evidence of demyelinating neuropathy, she was diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy and treated with IVIg without benefit. MRI showed symmetrical hypertrophy and marked signal hyperintensity of brachial and lumbosacral plexi. Cerebrospinal fluid showed 710 mg/dL protein. Despite intravenous methylprednisolone, the patient progressively worsened, and became wheelchair-bound. Antibodies to nodal–paranodal antigens were searched for by ELISA and cell-based assay. Anticontactin/Caspr1 IgG4 antibodies resulted positive. The patient underwent rituximab therapy with slow progressive improvement that mirrored the antibodies titer, measured throughout the disease course.

Conclusions

Our patient had a severe progressive course with early disability and axonal damage, and slow recovery starting only a few months after antibody-depleting therapy. The close correlation between titer, disability, and treatment, supports the pathogenicity of Caspr1 antibodies, and suggest that their longitudinal evaluation might provide a potential biomarker to evaluate treatment response.

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来源期刊
CiteScore
6.10
自引率
7.90%
发文量
45
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.
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