Randall David P, Randall Matthew C
Neuromuscular Neurology, Advocate Health, 1850 Dempster Street, Park Ridge, IL, 60068, USA.
Norton College of Medicine, Upstate Medical University, Syracuse, NY, USA.
BMC Neurol. 2025 Jan 2;25(1):2. doi: 10.1186/s12883-024-03983-8.
This is an unusual case of voltage gated calcium channel (VGCC) antibodies leading to two distinct and chronologically separated neurologic syndromes without the presence of an underlying neoplasm. Lambert Eaton Myasthenic Syndrome (LEMS) presented five years prior to cerebellar ataxia. Both LEMS and cerebellar ataxia were responsive to treatment, but not the same therapy. He was diagnosed with LEMS through history, exam, electromyography/nerve conduction studies (EMG/NCS) with repetitive nerve stimulation (RNS) and antibody testing. He was treated with 3,4 diaminopyridine (3,4 DAP) with an excellent response. Five years later, he developed acute ataxia. The patient required months of intensive and continued immunomodulating therapy.
这是一例不寻常的病例，电压门控钙通道（VGCC）抗体导致了两种不同且在时间上相隔的神经综合征，而不存在潜在肿瘤。兰伯特-伊顿肌无力综合征（LEMS）在小脑共济失调出现前五年就已出现。LEMS和小脑共济失调对治疗均有反应，但并非对同一种疗法有反应。通过病史、体格检查、肌电图/神经传导研究（EMG/NCS）以及重复神经刺激（RNS）和抗体检测，他被诊断为LEMS。他接受了3,4 - 二氨基吡啶（3,4 DAP）治疗，反应良好。五年后，他出现了急性共济失调。该患者需要数月的强化和持续免疫调节治疗。
