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重症肌无力的临床谱:印度东北部一家机构8年的经验

Clinical spectrum of myasthenia gravis: An institute experience over 8 years from North East India.

作者信息

Synmon Baiakmenlang, Rawat Sanjib, Kynta Reuben, Hussain Masaraf, Sharma Shri R, Lyngdoh Bifica S, Wankhar Baphira, Hynniewta Yasmeen

机构信息

Department of Neurology, Neigrihms, Shillong, India.

Department of CTVS, Neigrihms, Shillong, India.

出版信息

J Family Med Prim Care. 2022 Nov;11(11):7158-7162. doi: 10.4103/jfmpc.jfmpc_617_22. Epub 2022 Dec 16.

DOI:10.4103/jfmpc.jfmpc_617_22
PMID:36993138
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10041265/
Abstract

BACKGROUND

Myasthenia gravis (MG) is a neuromuscular junction disorder usually associated with a thymic lesion.

AIMS AND OBJECTIVE

To study the clinical, serological, and thymic pathology in patient of MG from this corner of the country.

MATERIAL AND METHOD

A retrospective study involving all myasthenia patients presenting to neurology and cardio-thoracic department from the year 2013 to 2020. The clinical findings, Osserman grade of severity, antibodies profile, computed scanning thorax findings and histopathology of the thymic lesion were noted and collected as data.

RESULTS

Thirty patients of MG were included with mean age of onset being 39.10 ± 15.77 years which included 22 females and eight males. Four patients had only ocular findings while 26 patients had generalized myasthenia with three patients of respiratory failure. Ach receptor antibodies were positive in 27 patients and negative in two patients. Anti-MUSK was positive in one out of five patients. Abnormal findings in CT thorax seen in 20 patients which included enlarged thymic gland in 11 patients, thymic hyperplasia in two patients, thymoma in four patients, and anterior mediastinal mass in three patients. Thymectomy was done in eighteen patients with thymoma as the most common histopathological findings seen in eight patients, follicular hyperplasia in five patients; other was thymic hyperplasia, thymic cyst, normal thymus gland, and features of sarcoidosis in one patient.

CONCLUSION

MG is a treatable autoimmune disorder with a variety of clinical, radiological, and histopathological findings.

摘要

背景

重症肌无力(MG)是一种神经肌肉接头疾病,通常与胸腺病变有关。

目的

研究该国这个地区MG患者的临床、血清学和胸腺病理学特征。

材料与方法

一项回顾性研究,纳入了2013年至2020年期间到神经内科和心胸外科就诊的所有重症肌无力患者。记录并收集临床症状、Osserman严重程度分级、抗体谱、胸部计算机扫描结果以及胸腺病变的组织病理学结果作为数据。

结果

纳入30例MG患者,平均发病年龄为39.10±15.77岁,其中女性22例,男性8例。4例仅有眼部症状,26例有全身型重症肌无力,3例出现呼吸衰竭。27例患者乙酰胆碱受体抗体呈阳性,2例呈阴性。5例患者中有1例抗肌肉特异性激酶抗体呈阳性。20例患者胸部CT检查有异常发现,其中11例胸腺增大,2例胸腺增生,4例胸腺瘤,3例前纵隔肿块。18例患者接受了胸腺切除术,最常见的组织病理学发现是8例胸腺瘤,5例滤泡增生;其他包括胸腺增生、胸腺囊肿、正常胸腺以及1例结节病特征。

结论

MG是一种可治疗的自身免疫性疾病,具有多种临床、放射学和组织病理学表现。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d04d/10041265/7f70961eca48/JFMPC-11-7158-g005.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d04d/10041265/e0b95e0a88d0/JFMPC-11-7158-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d04d/10041265/fad902b7ae66/JFMPC-11-7158-g002.jpg
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