Department of Neurology, University of Pittsburgh Medical Center, Pittsburgh, PA.
Department of Neurology and Rehabilitation, University of Illinois at Chicago, Chicago, IL.
J Clin Neuromuscul Dis. 2021 Mar 1;22(3):164-168. doi: 10.1097/CND.0000000000000318.
Lambert-Eaton myasthenic syndrome (LEMS) is a presynaptic neuromuscular junction disorder, and dermatomyositis (DM) is an idiopathic inflammatory myopathy. LEMS and DM are uncommon conditions that can present similarly and are often associated with autoantibodies. Concomitant LEMS and DM have only been reported a few times, and most of those cases were paraneoplastic. We present the first reported case of a patient with antivoltage gated calcium channel antibody positive LEMS who subsequently developed DM with antitranscription intermediary factor 1-gamma (anti-TIF1-γ) antibodies. Interestingly, both conditions occurred without evidence of malignancy. This diagnosis of LEMS and DM with characteristic clinical, electrodiagnostic, and histopathological evidence led to a beneficial modification of the patient's therapeutic regimen. Due to the fact that overlapping concurrent neuromuscular conditions are rare, a high clinical suspicion is needed to identify, evaluate (including appropriate cancer screenings), and appropriately treat these patients.
Lambert-Eaton 肌无力综合征 (LEMS) 是一种突触前神经肌肉接头疾病,皮肌炎 (DM) 是一种特发性炎症性肌病。LEMS 和 DM 是不常见的疾病,它们的表现可能相似,并且常与自身抗体有关。同时发生的 LEMS 和 DM 仅报告过几次,而且大多数病例是副肿瘤性的。我们报告了首例抗电压门控钙通道抗体阳性 LEMS 患者随后发生 DM 伴抗转录中介因子 1-γ (抗-TIF1-γ) 抗体的病例。有趣的是,这两种情况均无恶性肿瘤证据。LEMS 和 DM 的诊断具有特征性的临床、电诊断和组织病理学证据,从而使患者的治疗方案得到了有益的调整。由于重叠的同时发生的神经肌肉疾病很少见,因此需要高度的临床怀疑来识别、评估(包括适当的癌症筛查)并适当治疗这些患者。