Ten Brinck Michelle Fm, Verheijen Inge Wh, van de Wardt Jaron, van Dijk Gert W, Nijhuis Frouke Ap, Verrips Aad
Department of neurology, Canisius Wilhelmina Hospital, Nijmegen, The Netherlands.
BMJ Neurol Open. 2023 Aug 18;5(2):e000426. doi: 10.1136/bmjno-2023-000426. eCollection 2023.
Lambert-Eaton myasthenic syndrome (LEMS) is a neuromuscular junction disorder and the clinical triad consists of proximal muscle weakness, autonomic symptoms and reduced tendon reflexes. Sluggish pupillary reflexes are common but dilated fixed pupils are rare.
We report a patient with a rare clinical course of LEMS. The patient was hospitalised due to progressive dyspnoea. She was ambulant and independent of oxygen at hospitalisation. The following day she suffered an in-hospital cardiac arrest based on hypoxia due to sputum stasis. The neurology department was consulted since the patient did not trigger on the ventilator after cessation of sedation. On neurological examination, the patient had dilated and fixed pupils, severe muscle weakness and areflexia, but a normal consciousness. Finally, she was diagnosed with LEMS. In this case report, the clinical course and diagnostic workup including anti-VGCC antibody testing, imaging and the results of electrophysiological studies are discussed. We also emphasise the importance of malignancy screening since the conventional chest CT was negative for lung carcinoma, but PET-CT raised a high suspicion for small-cell lung carcinoma.
A rare course of LEMS, with early respiratory failure and wide, fixed pupils. Regarding repetitive nerve stimulation, it is important to stimulate long enough to see the incremental response. Furthermore, this study illustrated the importance of malignancy screening with PET-CT when there is a high suspicion of small-cell lung carcinoma with negative conventional CT.
兰伯特-伊顿肌无力综合征（LEMS）是一种神经肌肉接头疾病，临床三联征包括近端肌无力、自主神经症状和腱反射减弱。瞳孔反射迟钝常见，但瞳孔散大固定罕见。
我们报告一例具有罕见临床病程的LEMS患者。该患者因进行性呼吸困难入院。入院时她可步行且无需吸氧。次日，她因痰液淤积导致缺氧而在医院发生心脏骤停。由于患者在停用镇静剂后对呼吸机无反应，遂咨询神经科。神经系统检查发现，患者瞳孔散大固定、严重肌无力和无反射，但意识正常。最终，她被诊断为LEMS。在本病例报告中，讨论了临床病程及包括抗电压门控钙通道（VGCC）抗体检测、影像学检查和电生理研究结果在内的诊断检查。我们还强调了恶性肿瘤筛查的重要性，因为传统胸部CT未发现肺癌，但正电子发射断层扫描-计算机断层扫描（PET-CT）高度怀疑为小细胞肺癌。
LEMS的一种罕见病程，早期出现呼吸衰竭且瞳孔散大固定。关于重复神经电刺激，足够长时间的刺激以观察递增反应很重要。此外，本研究说明了当高度怀疑小细胞肺癌而传统CT为阴性时，PET-CT进行恶性肿瘤筛查的重要性。
