兰伯特-伊顿肌无力综合征的识别、生理和治疗。

IF 4.3 3区 医学 Q1 MEDICINE, GENERAL & INTERNAL
David P Randall
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引用次数: 0

摘要

兰伯特-伊顿肌无力综合征(LEMS)是一种罕见的疾病影响神经肌肉交界处。由于在神经肌肉连接处乙酰胆碱传递失败,它会导致虚弱。由于乙酰胆碱释放在自主神经上的丧失,它也导致自主神经症状,通常导致口干,但也导致阳痿、出汗障碍和直立性低血压。潜在的病理几乎平均分为副肿瘤或自身免疫。诊断方面的挑战导致延误,这可能与癌症(主要是小细胞肺癌)患者较晚开始癌症治疗有关。对于LEMS的症状,有直接作用于神经肌肉连接处的有效治疗方法,如果存在癌症,也有治疗方法。各种类型的免疫抑制对副肿瘤LEMS (T LEMS)和非肿瘤LEMS (NT LEMS)都是有益的。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
The recognition, physiology, and treatment of Lambert-Eaton myasthenic syndrome.

Lambert-Eaton myasthenic syndrome (LEMS) is a rare disease affecting the neuromuscular junction. It causes weakness due to the failure of acetylcholine transmission at the neuromuscular junction. It is also responsible for autonomic symptoms due to the loss of acetylcholine release at autonomic nerves resulting in most often dry mouth, but also impotence, sweating disturbance and orthostatic hypotension. The underlying pathology is split nearly evenly as either paraneoplastic or autoimmune. The challenges in making a diagnosis result in a delay which may correlate with a later initiation of cancer therapy in those with cancers, predominantly small cell lung cancer. There are effective treatments for symptoms of LEMS that work directly at the neuromuscular junction, as well as treatments for the cancer if present. Various types of immunosuppression can be beneficial for both paraneoplastic (T LEMS) and non-tumor LEMS (NT LEMS).

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来源期刊
Dm Disease-A-Month
Dm Disease-A-Month 医学-医学:内科
CiteScore
5.70
自引率
2.50%
发文量
140
审稿时长
>12 weeks
期刊介绍: Designed for primary care physicians, each issue of Disease-a-Month presents an in-depth review of a single topic. In this way, the publication can cover all aspects of the topic - pathophysiology, clinical features of the disease or condition, diagnostic techniques, therapeutic approaches, and prognosis.
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