[Kearns' syndrome (author's transl)].
Kearns' syndrome, a rare cause of chronic progressive ophthalmoplegia was observed in three patients aged 15 to 54 years. Apart from the chronic progressive external ophthalmoplegia the syndrome consists of retinal changes and cardiac conduction defects in all cases, as well as other signs indicating damage to the nervous system. High tone deafness and vestibular damage as well as an increase in CSF protein are common. Endocrine disorders and skeletal anomalies may occur. The cause is unknown. The ophthalmoplegia may be of neurogenic origin. Adams-Stokes attacks following disturbances of cardiac rhythm may be prevented by timely implantation of a cardiac pacemaker.
D000219:Adams-Stokes Syndrome / Q000150:complications; D000293:Adolescent; D000328:Adult; D001145:Arrhythmias, Cardiac / Q000175:diagnosis*; D002556:Cerebrospinal Fluid Proteins / Q000032:analysis; D002908:Chronic Disease; D003638:Deafness / Q000150:complications; D006801:Humans; D008297:Male; D008875:Middle Aged; D009886:Ophthalmoplegia / Q000175:diagnosis*; D012162:Retinal Degeneration / Q000175:diagnosis*; D013577:Syndrome
D002556:Cerebrospinal Fluid Proteins
Das Kearns-Syndrome eine Symptomkombination von chronisch progressiver externer Ophthalmoplegie, Pigmentdegeneration der Retina und Reizleitungsstörungen des Herzens