Bromberg M B, Albers J W, McCune W J
Department of Neurology, University of Michigan, Ann Arbor.
Muscle Nerve. 1989 Jan;12(1):15-9. doi: 10.1002/mus.880120104.
We present a patient who developed the Lambert-Eaton myasthenic syndrome (LEMS) in association with systemic lupus erythematosus (SLE). Severe proximal weakness with electrodiagnostic evidence of LEMS developed over 2 days during an exacerbation of cutaneous manifestations (bullous pemphigoid) associated with SLE. Following an increase in the daily dose of prednisone, there was complete clinical restitution of strength within 2 weeks and a slower resolution of electrodiagnostic abnormalities over 6 months. Marked serologic abnormalities were present at the onset and showed improvement over 6-8 months. LEMS had been infrequently described in association with SLE. The immunologic features of both SLE and LEMS suggest a linkage between the two diseases in this patient. We hypothesize that increased antibodies associated with exacerbation of SLE cross reacted with the neuromuscular junction membrane to produce LEMS.
我们报告一名患有兰伯特-伊顿肌无力综合征(LEMS)并伴有系统性红斑狼疮(SLE)的患者。在与SLE相关的皮肤表现(大疱性类天疱疮)加重期间,2天内出现严重近端肌无力,并伴有LEMS的电诊断证据。在泼尼松每日剂量增加后,2周内肌力完全恢复正常,电诊断异常在6个月内逐渐缓解。发病时存在明显的血清学异常,6 - 8个月内有所改善。LEMS与SLE相关的情况此前鲜有报道。SLE和LEMS的免疫学特征表明这两种疾病在该患者中存在联系。我们推测,与SLE加重相关的抗体增加与神经肌肉接头膜发生交叉反应,从而产生LEMS。