与单克隆 B 细胞淋巴细胞增多症相关的莫凡纤维性舞蹈症

IF 1 Q3 MEDICINE, GENERAL & INTERNAL
Daniel Park, Jeffrey Means, Andrew W Campion, Alan Kelton, Seema Nagpal, Haifaa Abdulhaq
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引用次数: 0

摘要

背景 莫凡纤维性舞蹈症(莫凡综合征)是一种罕见的疾病,主要表现为肌强直、周围神经兴奋、神经肌张力障碍、自主神经不稳定、记忆障碍和谵妄等一系列神经症状。莫文综合征疑似通过针对电压门控钾通道(VGKC)的抗体而发生,并与多种自身免疫性疾病和血液系统恶性肿瘤有关。我们报告了一例莫文综合征合并单克隆B细胞淋巴细胞增多症的病例。通过查阅文献,我们认为这是首例莫文综合征合并单克隆 B 细胞淋巴细胞增多症的病例。病例报告 本病例报告描述了一名 75 岁男性莫文氏综合征患者。患者有多种神经系统表现,包括脑病、进行性神经病理性疼痛、肌肉痉挛、肌强直、感觉障碍和贝尔氏麻痹。最终,副肿瘤抗体检测显示,接触素相关蛋白样 IgG(CASPR)和 VGKC 抗体滴度呈阳性。流式细胞术显示有少量异常的λ受限 B 细胞。鉴于他的症状、CASPR 抗体阳性和流式细胞术结果,他被诊断为莫文综合征伴单克隆 B 细胞淋巴细胞增多症。他接受了甲基强的松龙静脉注射和 IVIG 治疗,神经症状立即得到改善。结论 莫凡综合征表现为一系列神经系统症状,与通过抗 CASPR2 抗体产生的针对 VGKC 的自身抗体有关。传统上,莫凡综合征是继发于胸腺瘤的一种副肿瘤性疾病。我们的病例表明,B细胞淋巴增生性疾病与莫文综合征之间存在关联。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Morvan Fibrillary Chorea Associated with Monoclonal B Cell Lymphocytosis.

BACKGROUND Morvan fibrillary chorea (Morvan syndrome) is a rare disorder marked by a collection of neurological symptoms such as myokymia, peripheral nerve excitability, neuromyotonia, autonomic instability, memory impairment, and delirium. Morvan syndrome is suspected to occur through antibodies directed against voltage gated potassium channels (VGKC), and has been linked with several autoimmune conditions and hematologic malignancies. We present a case of Morvan syndrome in association with monoclonal B cell lymphocytosis. Upon our literature review, we believe this to be the first documented case of Morvan syndrome associated with monoclonal B cell lymphocytosis. CASE REPORT The present case report describes a 75-year-old man with Morvan's syndrome. The patient had a diverse neurologic presentation with encephalopathy, progressive neuropathic pain, muscle fasciculations, myokymia, sensory deficits, and Bell's palsy. Ultimately, a paraneoplastic antibody panel revealed a positive titer of contactin-associated protein-like IgG (CASPR) and VGKC antibody. Flow cytometry showed a small population of abnormal lambda-restricted B cells. Given his symptoms, positive CASPR antibody, and flow cytometry findings, he was diagnosed with Morvan syndrome associated with monoclonal B cell lymphocytosis. He was treated with IV methylprednisolone and IVIG, with immediate improvement in neurologic symptoms. CONCLUSIONS Morvan syndrome presents with a spectrum of neurologic symptoms and is associated with autoantibodies against VGKC through anti-CASPR2 antibodies. Classically, Morvan syndrome presents as a paraneoplastic disease secondary to thymomas. Our case demonstrates that there is an association between B cell lymphoproliferative disorders and Morvan syndrome.

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来源期刊
American Journal of Case Reports
American Journal of Case Reports Medicine-Medicine (all)
CiteScore
1.80
自引率
0.00%
发文量
599
期刊介绍: American Journal of Case Reports is an international, peer-reviewed scientific journal that publishes single and series case reports in all medical fields. American Journal of Case Reports is issued on a continuous basis as a primary electronic journal. Print copies of a single article or a set of articles can be ordered on demand.
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