Liu Bingyou, Zhou Lei, Zheng Yongsheng, Sun Chong, Lin Jie
Department of Neurology, Huashan Hospital, Fudan University, 12 Middle Wulumuqi Road, Jingan, Shanghai 200040, China.
Brain Sci. 2023 Feb 21;13(3):374. doi: 10.3390/brainsci13030374.
The anti-CV2/CRMP5 antibody is a well-characterized biomarker of paraneoplastic neurological syndrome. The anti-NF186 antibody is a recently discovered antibody associated with central or peripheral demyelination. The co-occurrence of these two antibodies has not been reported. Herein, we report a case with anti-CV2/CRMP5 and anti-NF186 antibodies in a 57-year-old male presenting with progressive numbness and weakness in his four limbs. At first admission, the spinal cord MRI showed a cervical cord demyelinating lesion and electrophysiological examination showed a mixed demyelinating and axonal polyneuropathy. Anti-CV2/CRMP5 and anti-NF186 antibodies were both detected in his serum. Initially, the patient showed a positive response to IVIG and glucocorticoid treatment. However, the syndrome relapsed and mass lesions in lung and mediastinum were detected at second admission. This time the anti-NF186 antibody was not detected but the anti-CV2/CRMP5 antibody was still present. IVIG and glucocorticoid treatment was no longer effective. This case illustrated that paraneoplastic syndrome should be considered when diagnosing patients with central and peripheral demyelination, and that the anti-NF186 antibody may help distinguish a subset of patients who can benefit from immunomodulatory treatments.
抗CV2/CRMP5抗体是副肿瘤性神经系统综合征的一种特征明确的生物标志物。抗NF186抗体是一种最近发现的与中枢或周围脱髓鞘相关的抗体。这两种抗体同时出现的情况尚未见报道。在此,我们报告一例57岁男性患者,其体内同时存在抗CV2/CRMP5和抗NF186抗体,表现为进行性四肢麻木和无力。首次入院时,脊髓MRI显示颈髓脱髓鞘病变,电生理检查显示为混合性脱髓鞘和轴索性多发性神经病。其血清中检测到抗CV2/CRMP5和抗NF186抗体。最初,患者对静脉注射免疫球蛋白(IVIG)和糖皮质激素治疗反应良好。然而,该综合征复发,第二次入院时发现肺部和纵隔有占位性病变。此次未检测到抗NF186抗体,但抗CV2/CRMP5抗体仍存在。IVIG和糖皮质激素治疗不再有效。该病例表明,在诊断中枢和周围脱髓鞘患者时应考虑副肿瘤综合征,并且抗NF186抗体可能有助于区分一部分能从免疫调节治疗中获益的患者。