+ Site Statistics
+ Search Articles
+ Subscribe to Site Feeds
EurekaMag Most Shared ContentMost Shared
EurekaMag PDF Full Text ContentPDF Full Text
+ PDF Full Text
Request PDF Full TextRequest PDF Full Text
+ Follow Us
Follow on FacebookFollow on Facebook
Follow on TwitterFollow on Twitter
Follow on Google+Follow on Google+
Follow on LinkedInFollow on LinkedIn

+ Translate

Electrophysiological findings in patients with kearns sayre syndrome report of 2 cases

Zeitschrift fuer Kardiologie 74(5): 281-286
Electrophysiological findings in patients with kearns sayre syndrome report of 2 cases
The Kearns-Sayre Syndrome is identified by the triad of progressive external ophthalmoplegia, atypical pigmentary retinopathy and conduction disturbances. Clinical manifestations may include mental retardation, sensorineural deafness, cerebellar ataxia and facial and peripheral muscle weakness. Morphologic alterations in skeletal muscle may be characterized by ragged-red fibers. Two patients with Kearns-Sayre Syndrome underwent electrophysiological examination. The 1st patient had a 1st and 2nd degree AV [atrioventricular] block (Mobitz type II), right bundle branch block and left axis deviation. The His-bundle electrogram showed a prolonged HV interval as a hint at an intraventricular conduction delay. The signal-averaging technique and endocardial mapping revealed ventricular after-depolarizations. The 2nd patient had an unsuspicious ECG, recurrent atrial tachycardias, normal atrial and ventricular conduction and after-depolarizations in endocardial mapping. Two mo. later he showed a 2nd degree AV block combined with clinical symptoms. Pacemakers were implanted in both patients. Beside disturbances of the conduction system in both patients signs of electrical instability of the myocardium were found. In this way the Kearns-Sayre syndrome may be seen as a form of cardiomyopathy.

Accession: 005349662

Related references

Electrophysiologic findings in patients with Kearns-Sayre syndrome--report on 2 cases. Zeitschrift für Kardiologie 74(5): 281-286, 1985

A clinical report of Kearns-Sayre syndrome with the electrophysiological evaluation. Yonago Acta Medica 19(2): 110-120, 1975

Cardiac manifestation in progressive external ophthalmoplegia kearns sayre syndrome electrophysiological hemodynamic and morphological findings. Zeitschrift fuer Kardiologie 75(3): 161-169, 1986

MRI of the brain in the Kearns-Sayre syndrome: report of four cases and a review. Neuroradiology 41(10): 759-764, 1999

Leighs disease or kearns sayre syndrome a report of 7 familial cases. Zentralblatt fuer Allgemeine Pathologie und Pathologische Anatomie 129(3): 265, 1984

Ultrastructural findings in endomyocardial biopsy of patients with Kearns-Sayre syndrome. Journal of the American College of Cardiology 12(6): 1522-1528, 1988

Electrophysiological studies on a case of Kearns-Sayre syndrome. Nippon Ganka Gakkai Zasshi 80(2): 71-75, 1976

Cardiac conduction in the Kearns-Sayre syndrome (a neuromuscular disorder associated with progressive external ophthalmoplegia and pigmentary retinopathy). Report of 2 cases and review of 17 published cases. American Journal of Cardiology 44(7): 1396-1400, 1979

Diabetes in pediatric patients with Kearns-Sayre syndrome: clinical presentation of 2 cases and a review of pathophysiology. Canadian Journal of Diabetes 38(4): 225-228, 2015

MRI findings in an atypical case of Kearns-Sayre syndrome: a case report. Neuroradiology 47(4): 241-244, 2005