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MRI 证据显示重症肌无力患者眼外肌萎缩和脂肪替代。

MRI evidence of extraocular muscle atrophy and fatty replacement in myasthenia gravis.

机构信息

2nd Department of Radiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece.

1st Department of Neurology, School of Medicine, National and Kapodistrian University of Athens, Eginition Hospital, 74 Vas. Sophias Ave., 11528, Athens, Greece.

出版信息

Neuroradiology. 2021 Sep;63(9):1531-1538. doi: 10.1007/s00234-021-02753-4. Epub 2021 Jul 7.

Abstract

PURPOSE

The aim of the study was to evaluate extraocular muscle (EOM) atrophy and fatty replacement in ocular myasthenia gravis (OMG) and generalized myasthenia gravis (GMG) patients with chronic and untreated ocular symptoms or with inadequate response to immunotherapy and unprovoked ocular exacerbations despite chronic immunotherapy.

METHODS

Nineteen patients with either OMG or GMG and 19 healthy age-matched controls underwent an orbital MRI. Visually obvious muscle atrophy and muscle fatty replacement were evaluated by two raters independently. Maximum thickness of EOM was measured. Measurements of the muscles of each participant were added up, in order to calculate the total thickness.

RESULTS

Eleven patients suffered from AChR-positive GMG, and 8 patients from OMG. All patients had chronic ocular symptoms or inadequate response to corticosteroids and unprovoked ocular exacerbations in spite of immunotherapy. Fatty replacement was reported in 6/19 (31.6%) patients and 0/19 (0%) controls (p = 0.02). Obvious atrophy in at least one muscle was reported in 8/19 (42.1%) patients and 1/19 (5.3%) controls (p = 0.019). Statistically significant differences between the two groups were also found in the mean total thickness, as well as in the thickness of superior recti, levator palpebrae, inferior recti, and superior oblique muscles.

CONCLUSION

EOM atrophy and fatty replacement were seen frequently in our series of MG patients with treatment difficulties and frequent relapses of ocular involvement.

摘要

目的

本研究旨在评估慢性未经治疗的眼肌型重症肌无力(OMG)和全身型重症肌无力(GMG)患者的眼外肌(EOM)萎缩和脂肪替代,以及对免疫治疗反应不足或尽管进行慢性免疫治疗但仍出现自发性眼肌恶化的患者。

方法

19 例 OMG 或 GMG 患者和 19 名年龄匹配的健康对照者接受眼眶 MRI 检查。由 2 名评分者独立评估是否存在明显的肌肉萎缩和肌肉脂肪替代。测量 EOM 的最大厚度。将每位参与者的肌肉测量值相加,以计算总厚度。

结果

11 例患者患有 AChR 阳性 GMG,8 例患者患有 OMG。所有患者均有慢性眼部症状或对皮质类固醇治疗反应不足,以及尽管进行免疫治疗仍出现自发性眼肌恶化。19 例患者中有 6 例(31.6%)和 19 例对照者中无 0 例(0%)报告有脂肪替代(p=0.02)。19 例患者中有 8 例(42.1%)和 19 例对照者中有 1 例(5.3%)报告至少有 1 块肌肉明显萎缩(p=0.019)。两组间的总厚度平均值以及上直肌、提上睑肌、下直肌和上斜肌的厚度也存在显著差异。

结论

在我们的一系列治疗困难和频繁出现眼部受累复发的 MG 患者中,经常观察到 EOM 萎缩和脂肪替代。

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