Atypical Myasthenia Gravis Presentation with Limb-Girdle Weakness

Joshua Luster, Brent Jacdobus, J. Sladky, Timothy R Fullam
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Abstract

Myasthenia Gravis is a disorder characterized by autoantibodies targeting different proteins across the neuromuscular junction. The typical presentation of Myasthenia Gravis involves oculobulbar weakness, classically ptosis that may or may not be symmetric. Patients may also present with a more dramatic presentation of generalized weakness or even in myasthenic crisis requiring respiratory support for oxygenation. While these are the common presentations, our patient, a 63 year old male, presented with an atypical presentation of what is described as Limb-Girdle Myasthenia Gravis. This patient presented with proximal arm and leg weakness that rapidly progressed and lead to loss of reflexes, appearing to be a myelopathy prior to obtaining an electromyography/nerve conduction study which demonstrated decrement of 21% in ulnar, 22% in median, and 59% of radial nerves during 2Hz repetitive stimulation. Our patient improved with plasmapheresis and prednisone with full recovery of strength.
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非典型重症肌无力伴肢带无力
重症肌无力是一种疾病,其特征是自身抗体靶向神经肌肉交界处的不同蛋白质。重症肌无力的典型表现包括眼球无力,典型的上睑下垂,可能对称也可能不对称。患者也可能出现更严重的全身性无力,甚至出现需要呼吸辅助氧合的肌无力危象。虽然这些都是常见的表现,但我们的患者,一位63岁的男性,表现为非典型的四肢带状重症肌无力。该患者表现为手臂和腿部近端无力,并迅速发展并导致反射丧失,在获得肌电图/神经传导研究之前,似乎是脊髓病,结果显示在2Hz重复刺激下,尺神经减少21%,中神经减少22%,桡神经减少59%。患者经血浆置换和强的松治疗后好转,体力完全恢复。
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