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眼肌型重症肌无力:临床病程及乙酰胆碱受体抗体检测的诊断效用

Ocular Myasthenia: Clinical Course and the Diagnostic Utility of Assaying Acetylcholine Receptor Antibodies.

作者信息

Behbehani Raed, Ali Abdullah, Al-Moosa Ashraf

机构信息

Al-Bahar Ophthalmology Center, IBN SINA Hospital, Kuwait.

出版信息

Neuroophthalmology. 2022 Mar 15;46(4):220-226. doi: 10.1080/01658107.2022.2037662. eCollection 2022.

Abstract

Myasthenia gravis (MG) is an autoimmune disease that causes neuromuscular junction transmission defect and has a predilection for the with neuromuscular junction transmission defect and predilection for extra-ocular and eyelid muscles. Most cases of ocular MG (OMG) convert later to generalised MG (GMG). Assaying acetylcholine receptor antibodies (AchRA) has been used to diagnose MG, but the reported sensitivity in OMG is lower (50%) than in GMG. We report the clinical course and the diagnostic yield of assaying AchRA in a Kuwaiti cohort of patients with OMG. We carried out a retrospective review of 47 patients diagnosed with OMG who were tested for AchRA. Ancillary tests included the ice test, single-fibre electromyography (SFMEG), and repetitive nerve stimulation electromyography (RNS). Progression to GMG occurred in 51% of OMG patients with a mean time to progression of 12.1 months (range 4 to 20 months). AchRAs were positive in 46 of 47 cases (98%), while SFEMG was positive in 31 of 34 cases (91.1%). Older age (44.25 years versus 38 years, p < .05) and higher AchRA titre (2.0 nmol/L versus 1.27 nmol/L, p < .05) were significantly associated with conversion to GMG. We have found a high rate of AchRA seropositivity in relatively younger subjects of OMG. Higher AchRA titres and older age were associated with conversion to GMG, usually within the first 2 years.

摘要

重症肌无力(MG)是一种自身免疫性疾病,可导致神经肌肉接头传递缺陷,好发于眼外肌和眼睑肌肉。大多数眼肌型重症肌无力(OMG)病例随后会转变为全身型重症肌无力(GMG)。检测乙酰胆碱受体抗体(AchRA)已被用于诊断重症肌无力,但据报道,OMG中的检测敏感性(50%)低于GMG。我们报告了科威特一组OMG患者的临床病程及检测AchRA的诊断率。我们对47例被诊断为OMG且接受了AchRA检测的患者进行了回顾性研究。辅助检查包括冰试验、单纤维肌电图(SFMEG)和重复神经刺激肌电图(RNS)。51%的OMG患者进展为GMG,平均进展时间为12.1个月(范围4至2个月)。47例中有46例(98%)AchRA呈阳性,而34例中有31例(91.1%)SFEMG呈阳性。年龄较大(44.25岁对38岁,p<0.05)和AchRA滴度较高(2.0 nmol/L对1.27 nmol/L,p<0.05)与转变为GMG显著相关。我们发现在相对年轻OMG患者中AchRA血清阳性率较高。较高的AchRA滴度和年龄较大与转变为GMG相关,通常在头2年内。 (注:原文中“范围4至2个月”可能有误,推测为“范围4至20个月”,译文按推测修改)

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