Author/Editor		Jarc, Martina; Zidar, Janez; Drnovšek-Olup, Brigita; Hawlina, Marko; Popović, Petra
Title		Progresivna eksterna oftalmoplegija (PEO), diagnostika in zdravljenje
Translated title		Progressive external ophthalmoplegia (PEO), diagnostic and treatment
Type		članek
Source		In: Zbornik referatov 2. slovenski oftalmološki kongres; 1997 Sep; Portorož. Ljubljana: Združenje oftalmologov Slovenije,
Publication year		1997
Volume		str. 113-22
Language		slo
Abstract		Progressive external ophthalmoplegia is a rare neurological disorder clinically characterised by insidously progressive, symmetric immobility of the eyes and ptosis. Most patients with fixed, nonfluctuating, insidiously progresive ptosis and ophthalmoplegia have a mitochondrial citopathy. This disease is caused by mutations of mitochondrial DNA and subsequent lack of mitochondrial energy. The documentation of twelve patients with the diagnosis of mitochondrial citopathy who were diagnosed at the Institute for Clinical Neurophysiology and then operated for ptosis on Eye Clinic and Clinic for Plastic surgery in Ljubljana, was carefully studied. Besides ptosis and ophthalmoplegia there were pigmental degenerations of retina, cataracts, cardiac conduction defects, sensoryneural abnormalities, depressed ventilatory drive, dysphagia and dysphasia. Muscle biopsies, which are crucial for diagnosis, revealed ragged red fibers. Till now no specific treatment has emerged for these patients; for patients with severe ptosis the eyelid surgery is possible method of choice. Awareness of depressed ventilatory drive and risk of progression to complete heart block is extremely important and the patients should be operated under local anesthesia. Rare cases of improvement after supplementation with coenzyme Q - ubiquinone - were reported in the literature.
Descriptors		OPHTHALMOPLEGIA, CHRONIC PROGRESSIVE EXTERNAL MITOCHONDRIAL MYOPATHIES ELECTROMYOGRAPHY