两名无恶性肿瘤基础的脊髓炎患者体内一过性升高的抗CRMP-5自身抗体

Kennan Negrete , Zeinab Awada , Asaff Harel , Ilya Kister
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摘要

导言Collapsin response-mediator protein 5(CRMP-5)是神经元生长的细胞质调节因子。CRMP-5抗体与包括脊髓炎在内的多种神经系统疾病有关。70-90%的CRMP-5自身免疫患者存在恶性肿瘤。我们介绍了两名患有脊髓炎且抗 CRMP-5 一过性升高但无恶性肿瘤证据的患者,并讨论了这些病例中抗体的相关性。一名 44 岁男性患者出现亚急性胸脊髓炎症状,核磁共振检查发现其脊髓病变持续增强。血清中抗 CRMP-5 抗体水平最初升高,但在随后的检测中没有出现。在为期三年的随访中,三次全身正电子发射计算机断层扫描均未发现恶性肿瘤。服用类固醇后,神经系统状况有所改善。2.一名 65 岁女性出现颈髓炎症状,随后出现左侧面部无力。磁共振成像显示多处脑部和脊髓病变以及颅神经炎的证据,尽管服用了脉冲类固醇药物,但症状依然存在。症状出现九个月后,发现血清抗CRMP-5升高。恶性肿瘤检查未能发现肿瘤,抗CRMP-5水平随后恢复。结论CRMP-5自身抗体是副肿瘤性自身免疫性神经综合征的标志物。然而,这两个病例说明,抗CRMP-5只是短暂升高,与潜在的恶性肿瘤无关,因此其意义并不确定。本文讨论了抗CRMP-5自身抗体是偶然发现或假阳性的可能性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Transiently elevated anti-CRMP-5 autoantibodies in two patients with myelitis without underlying malignancy

Introduction

Collapsin response-mediator protein 5 (CRMP-5) is a cytoplasmic regulator of neurite outgrowth. Antibodies against CRMP-5 are associated with various neurologic diseases, including myelitis. Underlying malignancy is present in 70 – 90 % of patients with CRMP-5 autoimmunity. We present two patients with myelitis and transiently elevated anti-CRMP-5 without evidence of malignancy and discuss the relevance of the antibody in these cases.

Case Report

1. A 44-year-old male presented with symptoms of subacute thoracic myelitis and was found to have a persistently enhancing cord lesion on MRI. Serum anti-CRMP-5 antibody levels were initially elevated but absent on subsequent testing. Three whole-body PET scans during a three-year follow-up failed to uncover a malignancy. Neurologic condition improved on steroids. 2. A 65-year-old female presented with symptoms of a cervical myelitis followed by left facial weakness. MRI demonstrated multiple brain and spinal cord lesions as well as evidence of cranial neuritis, which persisted despite pulse steroid courses. Elevated serum anti-CRMP-5 was noted nine months after symptom onset. Malignancy workup failed to identify neoplasm and anti-CRMP-5 level subsequently seroreverted. Clinical and radiographic improvement occurred over several years of follow-up.

Conclusion

CRMP-5 autoantibody is a marker for paraneoplastic autoimmune neurologic syndromes. However, these two cases illustrate the uncertainty regarding its significance, as anti-CRMP-5 was only transiently elevated and not associated with an underlying malignancy. The possibilities that anti-CRMP-5 autoantibodies were an incidental or false-positive finding are discussed.
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