背景技术Morvan原纤维性舞蹈症(Morvan综合征)是一种罕见的疾病,其特征是神经症状的集合,例如肌强直症。周围神经兴奋性,神经肌强直,自主神经不稳定,记忆障碍,和谵妄.怀疑Morvan综合征是通过针对电压门控钾通道(VGKC)的抗体发生的,并与多种自身免疫性疾病和血液系统恶性肿瘤有关。我们介绍了一例与单克隆B细胞淋巴细胞增多相关的Morvan综合征。在我们的文献综述中,我们认为这是第一例与单克隆B细胞淋巴细胞增多相关的Morvan综合征病例.病例报告本病例报告描述了一名患有Morvan综合征的75岁男子。病人有不同的神经系统表现,有脑病,进行性神经性疼痛,肌肉震颤,Myokymia,感觉缺陷,和贝尔的麻痹。最终,一个副肿瘤抗体组显示contactin相关蛋白样IgG(CASPR)和VGKC抗体滴度呈阳性.流式细胞术显示少量异常λ限制的B细胞。鉴于他的症状,CASPR抗体阳性,和流式细胞术的发现,他被诊断为与单克隆B细胞淋巴细胞增多相关的Morvan综合征。他接受了静脉注射甲基强的松龙和IVIG治疗,神经症状立即得到改善.结论Morvan综合征表现出一系列神经系统症状,并通过抗CASPR2抗体与抗VGKC自身抗体相关。经典的,Morvan综合征表现为继发于胸腺瘤的副肿瘤疾病。我们的病例表明B细胞淋巴增生性疾病与Morvan综合征之间存在关联。
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.