Cho Jonathan J, Wymer James P
Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine, University of Florida College of Medicine, Gainesville, FL.
Department of Neurology, McKnight Brain Institute, University of Florida College of Medicine, Gainesville, FL.
J Clin Neuromuscul Dis. 2017 Dec;19(2):84-88. doi: 10.1097/CND.0000000000000192.
To characterize Lambert-Eaton myasthenic syndrome and limbic encephalitis with coexistent voltage-gated calcium channel (VGCC) antibody and γ-aminobutyric acid (GABA) B receptor antibody.
Case study.
A 57-year-old man presented with 6 months of weakness, unsteadiness, and vision difficulties. Examination revealed proximal weakness and diminished reflexes. Electrodiagnostic study revealed low-amplitude motor potentials and facilitation on high-frequency stimulation. Laboratory evaluation identified P/Q-type VGCC antibody. Positron emission tomography identified a mediastinal lesion, confirmed as small-cell lung carcinoma. The patient developed confusion and seizures. Cerebrospinal fluid analysis identified antibodies to GABAB receptor.
This case describes a patient with Lambert-Eaton myasthenic syndrome, limbic encephalitis, and autoantibodies to VGCC and GABAB receptor. Atypical presentation of paraneoplastic neurological syndromes could indicate the presence of a second antibody that may have significant impact on therapy.
对同时存在电压门控钙通道(VGCC)抗体和γ-氨基丁酸(GABA)B受体抗体的兰伯特-伊顿肌无力综合征和边缘性脑炎进行特征描述。
病例研究。
一名57岁男性出现6个月的肌无力、步态不稳和视力障碍。检查发现近端肌无力和反射减弱。电诊断研究显示运动电位幅度降低以及高频刺激时的易化现象。实验室评估发现P/Q型VGCC抗体。正电子发射断层扫描发现纵隔病变,确诊为小细胞肺癌。患者出现意识模糊和癫痫发作。脑脊液分析发现GABAB受体抗体。
本病例描述了一名患有兰伯特-伊顿肌无力综合征、边缘性脑炎以及VGCC和GABAB受体自身抗体的患者。副肿瘤性神经系统综合征的非典型表现可能提示存在第二种抗体,这可能对治疗产生重大影响。