兰伯特-伊顿肌无力综合征伴发音困难及构音障碍1例。

IF 1.3 Q3 MEDICINE, GENERAL & INTERNAL
Cureus Pub Date : 2025-06-24 eCollection Date: 2025-06-01 DOI:10.7759/cureus.86700
Hridya Harimohan, Mia Yasonova, Marah Sukkar, Katayoun Sabetian
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引用次数: 0

摘要

兰伯特-伊顿肌无力综合征(LEMS)是一种罕见的自身免疫性疾病,导致肌肉无力。LEMS典型表现为进行性近端肌肉无力,尤其是下肢,反射屈曲和自主神经功能障碍。球受累和呼吸损害往往发生在病程的后期,通常发生在严重的晚期病例中。我们报告一个独特的病例,50岁男性,已知LEMS病史,自身免疫性视网膜病变,勃起功能障碍和前驱糖尿病,表现为进行性球症状-言语不清和吞咽困难-没有伴随上肢或下肢无力。尽管入院前接受了四次静脉注射免疫球蛋白(IVIG),但没有任何改善,他对六次血浆置换表现出显著的临床反应。值得注意的是,在整个住院期间,肢体无力的消失和运动力量的保留增加了诊断的不确定性,促使对重症肌无力进行评估,通过乙酰胆碱受体抗体检测阴性和电压门控制钙通道(VGCC)抗体阳性确认排除了重症肌无力。胸部影像学未见潜在恶性肿瘤。本病例是非典型的LEMS表现,主要表现为严重的球功能障碍,没有肢体受累或呼吸衰竭,这是不常见的,可能类似于其他神经肌肉连接障碍,如重症肌无力。血浆置换的良好反应强调了早期识别和积极的免疫调节治疗在预防临床恶化中的重要性。临床医生应保持对LEMS的高度怀疑指数,即使在不寻常的表现,以确保及时和适当的管理。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A Rare Case of Lambert-Eaton Myasthenia Syndrome With Dysphasia and Dysarthria.

Lambert-Eaton Myasthenic Syndrome (LEMS) is a rare autoimmune disorder resulting in muscle weakness. LEMS typically presents with progressive proximal muscle weakness, especially in the lower extremities, areflexia, and autonomic dysfunction. Bulbar involvement and respiratory compromise tend to occur later in the disease course and usually in severe, advanced cases. We report a unique case of a 50-year-old male with a known history of LEMS, autoimmune retinopathy, erectile dysfunction, and prediabetes who presented with progressive bulbar symptoms - slurred speech and difficulty swallowing - without accompanying weakness in the upper or lower extremities. Despite receiving four doses of intravenous immunoglobulin (IVIG) prior to admission with no improvement, he showed a significant clinical response to six sessions of plasmapheresis. Notably, the absence of limb weakness and preserved motor strength throughout his hospital stay raised diagnostic uncertainty, prompting evaluation for myasthenia gravis, which was ruled out through negative acetylcholine receptor antibody testing and reaffirmed voltage-gated calcium channel (VGCC) antibody positivity. Chest imaging showed no underlying malignancy. This case illustrates an atypical LEMS presentation dominated by severe bulbar dysfunction in the absence of limb involvement or respiratory failure, which is uncommon and may mimic other neuromuscular junction disorders such as myasthenia gravis. The favorable response to plasmapheresis emphasizes the importance of early recognition and aggressive immunomodulatory treatment in preventing clinical deterioration. Clinicians should maintain a high index of suspicion for LEMS even in unusual presentations to ensure timely and appropriate management.

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