PDF(Pubmed)
Abstract：
BACKGROUND: Facial-onset sensory and motor neuronopathy (FOSMN) is a greatly rare disease, so far, autopsy evidence that is associated with neurodegenerative. Myasthenia gravis (MG) is an antibody-mediated and complement-involved acquired autoimmune disorder of the post-synaptic neuromuscular junction. There have been few reports about if there is related between the 2. In this study, we present the case of a man who was diagnosed as FOSMN with MG in continuity.
METHODS: The patient chief complaints were right-side facial numbness and right-eyelid incomplete closure, followed by slurred speech and dysphagia, and the symptoms gradually progressed. The patient serum was positive for anti-AchR and anti-Titin antibodies.
METHODS: The patient was diagnosed FOSMN with MG.
METHODS: The patient symptoms were relieved after pyridostigmine bromide and prednisolone treatment.
RESULTS: Symptoms have improved.
CONCLUSIONS: Facial-onset sensory and motor neuronopathy and MG have disparate clinical features. Therefore, we reported a rare case in which the 2 conditions concurrently existed. Immune dysfunction might be the pathogenesis of this association, while there is no definite evidence to support it, further studies are needed.
METHODS: The patient chief complaints were right-side facial numbness and right-eyelid incomplete closure, followed by slurred speech and dysphagia, and the symptoms gradually progressed. The patient serum was positive for anti-AchR and anti-Titin antibodies.
METHODS: The patient was diagnosed FOSMN with MG.
METHODS: The patient symptoms were relieved after pyridostigmine bromide and prednisolone treatment.
RESULTS: Symptoms have improved.
CONCLUSIONS: Facial-onset sensory and motor neuronopathy and MG have disparate clinical features. Therefore, we reported a rare case in which the 2 conditions concurrently existed. Immune dysfunction might be the pathogenesis of this association, while there is no definite evidence to support it, further studies are needed.
摘要：
背景：面部发作的感觉和运动神经元病（FOSMN）是一种非常罕见的疾病，到目前为止,尸检证据与神经退行性变有关。重症肌无力（MG）是突触后神经肌肉接头的抗体介导和补体参与的获得性自身免疫性疾病。关于两者之间是否有关联的报道很少。在这项研究中,我们介绍了一个被诊断为FOSMN并连续性MG的病例.
方法：患者主诉是右侧面部麻木和右眼睑不完全闭合，其次是言语不清和吞咽困难，症状逐渐进展。患者血清抗AchR和抗Titin抗体呈阳性。
方法：患者诊断为FOSMN合并MG。
方法：患者经溴吡斯的明和泼尼松龙治疗后症状缓解。
结果：症状有所改善。
结论：面部发作的感觉和运动神经元病和MG具有不同的临床特征。因此,我们报道了一个罕见的病例，其中两种情况同时存在。免疫功能紊乱可能是这种关联的发病机制,虽然没有确切的证据支持，需要进一步的研究。
方法：患者主诉是右侧面部麻木和右眼睑不完全闭合，其次是言语不清和吞咽困难，症状逐渐进展。患者血清抗AchR和抗Titin抗体呈阳性。
方法：患者诊断为FOSMN合并MG。
方法：患者经溴吡斯的明和泼尼松龙治疗后症状缓解。
结果：症状有所改善。
结论：面部发作的感觉和运动神经元病和MG具有不同的临床特征。因此,我们报道了一个罕见的病例，其中两种情况同时存在。免疫功能紊乱可能是这种关联的发病机制,虽然没有确切的证据支持，需要进一步的研究。
