Maldonado Marcos E, Williams Ralph C, Adair John C, Hart Blaine L, Gregg Laura, Sibbitt Wilmer L
Clinical and Magnetic Resonance Research Center, Department of Internal Medicine, University of New Mexico Health Sciences Center, Albuquerque 87131, USA.
J Rheumatol. 2002 Mar;29(3):633-5.
A 61-year-old woman with a history of photosensitive dermatitis and recurrent mouth ulcers presented with progressive weakness typical of amyotrophic lateral sclerosis (ALS), and subsequently underwent extensive neurologic and rheumatologic testing. We investigated whether ALS-like motor neuron disease associated with a positive antinuclear antibody (ANA) is really ALS or rather neuropsychiatric systemic lupus erythematosus (NPSLE). On neurologic evaluation, she had prominent bulbar involvement with dysarthria and dysphagia associated with profound lingual fasciculations and a denervating pattern on electromyogram. MRI showed no evidence of cerebral ischemia. Laboratory studies revealed a positive ANA (1:2560 titer), positive antiphospholipid antibodies (GPL and MPL), circulating lupus anticoagulant, and depressed C3 and C4. Repeat MRI studies at 4 and 11 mo revealed an evolving infarct in the paramedian pons consistent with the presence of NPSLE. Therapy was initiated with corticosteroids and intravenous cyclophosphamide, and the neurologic condition did not improve, but also did not progress inexorably as would be expected with ALS. NPSLE, presumably through the mechanism of ischemic vasculopathy, may present as motor neuron disease clinically indistinguishable from ALS.
一名61岁女性，有光敏性皮炎和复发性口腔溃疡病史，出现了典型的肌萎缩侧索硬化症（ALS）进行性肌无力症状，随后接受了广泛的神经学和风湿病学检查。我们研究了与抗核抗体（ANA）阳性相关的类似ALS的运动神经元病究竟是真正的ALS，还是更确切地说是神经精神性系统性红斑狼疮（NPSLE）。经神经学评估，她有明显的延髓受累，伴有构音障碍和吞咽困难，同时伴有明显的舌肌束颤，肌电图显示为失神经模式。MRI未显示脑缺血证据。实验室检查显示ANA阳性（滴度为1:2560）、抗磷脂抗体（GPL和MPL）阳性、循环狼疮抗凝物阳性，C3和C4降低。在4个月和11个月时重复进行的MRI研究显示，脑桥中份出现了一个不断演变的梗死灶，符合NPSLE的表现。开始使用皮质类固醇和静脉注射环磷酰胺进行治疗，神经状况没有改善，但也没有像ALS那样不可避免地进展。NPSLE可能通过缺血性血管病变机制，在临床上表现为与ALS难以区分的运动神经元病。
