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

, : Electrophysiological findings in patients with kearns sayre syndrome report of 2 cases. Zeitschrift fuer Kardiologie 74(5): 281-286

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.

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Related references

Gonska, B.D.; Bethge, K.P.; Holzgraefe, M.; Rahlf, G.; Kreuzer, H., 1985: Electrophysiologic findings in patients with Kearns-Sayre syndrome--report on 2 cases. The Kearns-Sayre syndrome is identified by the triad of progressive external ophthalmoplegia, atypical pigmentary retinopathy, and conduction disturbances. In addition, clinical manifestations may include mental retardation, sensorineural deafness...

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

Schwartzkopff B.; Frenzel H.; Loesse B.; Borggrefe M.; Toyka K.V.; Hammerstein W.; Setiz R.; Deckert M.; Breithardt G., 1986: Cardiac manifestation in progressive external ophthalmoplegia kearns sayre syndrome electrophysiological hemodynamic and morphological findings. The Kearns-Sayre syndrome is combined with a progressive external opthalmoplegia (PEO), retinal pigmentary degeneration and heart block. In some patients, progression of incomplete forms has been described and potentially fatal conduction disturba...

Chu, B.C.; Terae, S.; Takahashi, C.; Kikuchi, Y.; Miyasaka, K.; Abe, S.; Minowa, K.; Sawamura, T., 1999: MRI of the brain in the Kearns-Sayre syndrome: report of four cases and a review. We report brain MRI findings in four patients with typical Kearns-Sayre syndrome (KSS) and correlate them with clinical manifestations. MRI was interpreted as normal in two patients; cerebral and cerebellar atrophy was seen in the other two. On T2...

Walter G.F.; Brucher J.M.; Martin J.J.; Ceuterick C., 1984: Leighs disease or kearns sayre syndrome a report of 7 familial cases. Zentralblatt fuer Allgemeine Pathologie und Pathologische Anatomie 129(3): 265

Schwartzkopff, B.; Frenzel, H.; Breithardt, G.; Deckert, M.; Lösse, B.; Toyka, K.V.; Borggrefe, M.; Hort, W., 1988: Ultrastructural findings in endomyocardial biopsy of patients with Kearns-Sayre syndrome. Kearns-Sayre syndrome is clinically defined by progressive external ophthalmoplegia, atypical retinitis pigmentosa and the potential occurrence of complete atrioventricular (AV) block. Right septal endomyocardial biopsy specimens from nine patient...

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

Roberts, N.K.; Perloff, J.K.; Kark, R.A., 1979: 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

Ho, J.; Pacaud, Dèle.; Rakic, M.; Khan, A., 2015: Diabetes in pediatric patients with Kearns-Sayre syndrome: clinical presentation of 2 cases and a review of pathophysiology. Kearns-Sayre syndrome (KSS), resulting from a mitochondrial DNA deletion, is a rare cause of diabetes in children. We report 2 pediatric cases of KSS associated with diabetes that presented with hyperosmolar hyperglycemia with minimal ketosis. Bot...

Sacher, M.; Fatterpekar, G.M.; Edelstein, S.; Sansaricq, C.; Naidich, T.P., 2005: MRI findings in an atypical case of Kearns-Sayre syndrome: a case report. MR imaging features of mitochondrial encephalomyopathies, lactic acidosis, and stroke-like episodes, Kearns-Sayre/Pearson syndrome have been described in the literature. We describe extensive white matter changes with abnormal signal intensity les...