[Progressive external ophthalmoplegia and disorders of ventricular conduction. Apropos of 3 recent cases].
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.
D000328:Adult; D001145:Arrhythmias, Cardiac / Q000150:complications* / Q000175:diagnosis; D005260:Female; D006327:Heart Block / Q000150:complications / Q000175:diagnosis; D006801:Humans; D008297:Male; D009886:Ophthalmoplegia / Q000150:complications* / Q000175:diagnosis; D010138:Pacemaker, Artificial; D011379:Prognosis
Ophtalmoplégie externe progressive et troubles de la conduction ventriculaire. A propos de 3 nouvelles observations.