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一名波斯尼亚男性同时患有重症肌无力和肌萎缩侧索硬化症:一种不常见的临床表现。

Coexistence of myasthenia gravis and amyotrophic lateral sclerosis in a Bosnian male: an unusual clinical presentation.

机构信息

Department of Neurology, University Clinical Centre Tuzla, Bosnia and Herzegovina.

Department of Physical Medicine and Rehabilitation, University Clinical Centre Tuzla, Bosnia and Herzegovina.

出版信息

Acta Myol. 2021 Mar 31;40(1):66-68. doi: 10.36185/2532-1900-044. eCollection 2021 Mar.

Abstract

PURPOSE

Myasthenia gravis (MG) and amyotrophic lateral sclerosis (ALS) are two different diseases. The coexistence of both of them is extremely rare and represents a diagnostic challenge which requires thoughtful interpretation of clinical characteristics.

CASE REPORT

We present the case of a 46-year-old Bosnian male who developed ALS five months after MG. Diagnosis of MG was based on elevated titers of anti-AchR antibodies, positive edrophonium test, and decremental responses on a repetitive nerve stimulation test while the diagnosis of ALS was based on clinical and neurophysiological findings: upper motor neuron signs in the lumbar region, lower motor neuron signs in the bulbar and cervical regions, generalized fasciculations and muscle atrophy and progressive asymetric muscle weakness together with active and chronic denervation in the cervical and lumbosacral region determined by electromyoneurography.

CONCLUSIONS

The coexistence of MG and ALS is rare and request an adequate interpretation of clinical symptoms. The relationship between these two diseases in as interesting phenomen to present.

摘要

目的

重症肌无力(MG)和肌萎缩侧索硬化症(ALS)是两种不同的疾病。这两种疾病同时存在极为罕见,是一种诊断挑战,需要对临床特征进行深思熟虑的解读。

病例报告

我们报告了一例 46 岁的波斯尼亚男性,他在 MG 后五个月发展为 ALS。MG 的诊断基于抗 AChR 抗体滴度升高、阳性依酚氯铵试验和重复神经刺激试验的递减反应,而 ALS 的诊断则基于临床和神经生理学发现:腰部的上运动神经元体征、球部和颈部的下运动神经元体征、广泛性肌束震颤和肌肉萎缩,以及电肌电图检查显示颈部和腰骶部的进行性非对称肌肉无力,伴有活动和慢性去神经支配。

结论

MG 和 ALS 同时存在较为罕见,需要对临床症状进行充分解读。这两种疾病之间的关系很有趣。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/63a9/8033427/7f4c1afe1042/am-2021-01-66-g001.jpg

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