Donzeau J P, Constans R, Conte D, Rochiccioli P, Bernadet P, Bounhoure J P, Calazel P
Arch Mal Coeur Vaiss. 1977 Aug;70(8):875-82.
The authors report 3 cases with an association of progressive external ophthalmoplegia (OEP) and disordered intracardiac conduction. These cases, and the twenty or so similar ones reported in the literature, show that this association is important for two reasons:--there is a therapeutic importance in that the condition affects young patients, who are at risk from sudden death due to the conduction defect; for this reason electrocardiographic follow-up must be regular, and an intracavitary pacemaker must be introduced definitively at the least indication;--there is a physiopathological importance in that the effect of the myopathies on the myocardium is well known, but most information relates to the diffuse cardiomyopathies, and in only 10% of cases are there conduction defects. By contrast, the conductive tissue appears to be involved in all cases of OEP, while cardiac failure is rare. It seems likely, therefore, that cases of OEP have a pathogenesis different from that of the diffuse myopathies, whether or not these involve the external occular muscles.
作者报告了3例伴有进行性眼外肌麻痹(OEP)和心内传导障碍的病例。这些病例,以及文献中报道的约20例类似病例,表明这种关联具有重要意义,原因有二:——具有治疗重要性,因为该病症影响年轻患者,他们因传导缺陷有猝死风险;因此必须定期进行心电图随访,且一旦有最小指征就必须最终植入心腔内起搏器;——具有生理病理学重要性,因为肌病对心肌的影响是众所周知的,但大多数信息涉及弥漫性心肌病,且仅10%的病例存在传导缺陷。相比之下,传导组织似乎在所有OEP病例中均受累,而心力衰竭很少见。因此,OEP病例的发病机制似乎与弥漫性肌病不同,无论后者是否累及眼外肌。