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[眼肌型重症肌无力:诊断要点与病情演变]

[Ocular myasthenia gravis: diagnostic aspects and evolution].

作者信息

de Entrambasaguas M, López-Bernabé R, López-Alemany M

机构信息

Servicio de Neurofisiología Clínica, Hospital General de Castellón, 12004 Castellón de la Plana, España.

出版信息

Rev Neurol. 2007;44(7):397-403.

Abstract

INTRODUCTION

Myasthenia gravis lacks a diagnostic gold standard, so diagnosis is supported by the findings of several tests. Conversion from ocular myasthenia gravis (OMG) to a more widespread disease is frequently early. AIMS. To describe and compare the clinical data and findings of the diagnostic tests of patients with OMG and those with a different diagnosis, and to know the conversion rate from OMG to generalized myasthenia.

PATIENTS AND METHODS

Descriptive and analytic retrospective study of 44 patients referred for neurophysiological testing through 4 years because of suspected OMG.

RESULTS

12 patients (27%) were diagnosed as having OMG. Evolution time prior to diagnosis tended to be shorter in OMG patients. Isolated ptosis or combined with diplopia was more frequent in OMG, while isolated diplopia was so in other diagnoses (p = 0.003). No thymoma was found. SFEMG jitter of facial muscles was abnormal, including blocking, in all OMG patients (8/8) and normal in the rest (30/30). Edrophonium test was positive in all OMG patients (7/7) and doubtful in another one (1/7). Anti-AChR titers were initially positive in 10/11 OMG patients and 0/17 with other diagnoses. Aponeurotic ptosis and strabismus were the most frequent non-myasthenic etiologies. OMG showed an early generalization in two patients who developed dysarthria.

CONCLUSIONS

The low ratio of diagnostic confirmation suggests that in the face of ptosis or diplopia diagnostic testing with a high sensibility for OMG is favoured. Jitter showed the best initial diagnostic performance.

摘要

引言

重症肌无力缺乏诊断金标准,因此需多项检查结果来支持诊断。眼肌型重症肌无力(OMG)较早出现向更广泛疾病的转变。目的:描述并比较OMG患者与其他诊断患者的临床资料及诊断检查结果,了解OMG向全身型重症肌无力的转化率。

患者与方法

对44例因疑似OMG在4年期间接受神经生理学检查的患者进行描述性和分析性回顾性研究。

结果

12例患者(27%)被诊断为OMG。OMG患者诊断前的病程往往较短。OMG患者中孤立性上睑下垂或合并复视更为常见,而其他诊断中孤立性复视更为常见(p=0.003)。未发现胸腺瘤。所有OMG患者(8/8)面部肌肉的单纤维肌电图(SFEMG)颤抖异常,包括阻滞,其余患者(30/30)正常。依酚氯铵试验在所有OMG患者(7/7)中呈阳性,另一例(1/7)可疑。抗乙酰胆碱受体(AChR)滴度最初在10/11例OMG患者中呈阳性,在其他诊断的患者中为0/17。腱膜性上睑下垂和斜视是最常见的非重症肌无力病因。两名出现构音障碍的OMG患者出现了早期全身化。

结论

确诊率较低表明,面对上睑下垂或复视时,倾向于采用对OMG敏感性高的诊断测试。颤抖显示出最佳的初始诊断性能。

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