双血清阳性肌萎缩症的临床特征

IF 0.9 3区 医学 Q4 NEUROSCIENCES Neurology India Pub Date : 2024-09-01 Epub Date: 2024-10-19 DOI:10.4103/neurol-india.Neurol-India-D-24-00091
Chirag S Lalwani, Hamsini Movva, Sudheeran Kannoth, Vivek Nambiar, Siby Gopinath, Anandkumar Anandakuttan, Saraf U Umesh, Sreelakshmi Narayanan, Bijoy Jose, Annamma Mathai, Jyothi V Leelamani
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引用次数: 0

摘要

双血清阳性肌无力(DSP-MG)患者的乙酰胆碱受体(AChR)抗体和肌肉特异性酪氨酸激酶(MuSK)抗体均呈阳性。本研究旨在描述 DSP-MG 的临床表型,并评估其与胸腺瘤的组织病理学相关性。这是一项回顾性病例系列研究,于2018年2月至2021年10月期间在印度南部的一家三级甲等医院进行。我们共收治了13名DSP-MG患者(7名女性,平均年龄60.77+/-14.24)。9名患者表现为全身性,3名患者表现为球部,1名患者表现为眼部。对 11 名患者进行的多载体计算机断层扫描显示,5 名患者有胸腺瘤,1 名患者有胸腺增生。四名患者接受了胸腺切除术;组织病理学显示为 A 型、B2 型(2 例)和 AB 型胸腺瘤。所有 13 名患者在服用抗胆碱酯酶药物后病情均有好转。九名患者服用了免疫抑制剂,三名患者静脉注射了免疫球蛋白,一名患者进行了浆液吸出术。我们的研究表明,DSP-MG 与 AChR-MG 的临床表型更为相似。
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Clinical Characteristics of Double Seropositive Myasthenia Gravis.

Double seropositive myasthenia gravis (DSP-MG) has antibodies positive to both acetylcholine receptor (AChR) antibody and muscle-specific tyrosine kinase (MuSK) antibody. This study aims to delineate the clinical phenotype of DSP-MG and assess the histopathological correlation with thymoma. This is a retrospective case series conducted at a tertiary care hospital in South India between February 2018 and October 2021. We had 13 DSP-MG patients (seven females, mean age 60.77+/-14.24). The presentation was generalized in nine patients, bulbar in three, and ocular in one patient. Multi-detector computed tomography done in 11 patients showed thymoma in five and thymic hyperplasia in one. Four patients underwent thymectomy; histopathology showed Type A, B2 (n = 2), and AB thymomas. All 13 patients improved with anticholinesterases. Nine patients were administered immunosuppressants, three patients were given intravenous immunoglobulin, and a single patient underwent plasmapheresis. Our study shows that DSP-MG is more similar to the clinical phenotype of AChR-MG.

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来源期刊
Neurology India
Neurology India 医学-神经科学
CiteScore
1.60
自引率
70.40%
发文量
434
审稿时长
2 months
期刊介绍: Neurology India (ISSN 0028-3886) is Bi-monthly publication of Neurological Society of India. Neurology India, the show window of the progress of Neurological Sciences in India, has successfully completed 50 years of publication in the year 2002. ‘Neurology India’, along with the Neurological Society of India, has grown stronger with the passing of every year. The full articles of the journal are now available on internet with more than 20000 visitors in a month and the journal is indexed in MEDLINE and Index Medicus, Current Contents, Neuroscience Citation Index and EMBASE in addition to 10 other indexing avenues. This specialty journal reaches to about 2000 neurologists, neurosurgeons, neuro-psychiatrists, and others working in the fields of neurology.
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