Update on Chronic Progressive External Ophthalmoplegia

被引:27
|
作者
Bau, Viktoria [1 ]
Zierz, Stephan [2 ]
机构
[1] Univ Halle Wittenberg, Kiln & Poliklin Augenheilkunde, Halle, Germany
[2] Univ Halle Wittenberg, Neurolog Univ klin, Halle, Germany
关键词
Chronic progressive external ophthalmoplegia; mitochondrial encephalomyopathy; Kearns-Sayre syndrome; myogenic ptosis; mitochondrial DNA; genetic disorders;
D O I
10.1080/09273970500216432
中图分类号
R77 [眼科学];
学科分类号
100212 ;
摘要
Mitochondrial encephalomyopathies are clinically and genetically heterogeneous disorders. External ophthalmoplegia is the most frequent symptom. Other frequently involved tissues and organs include the retina, heart, limb muscles, peripheral and central nervous system, inner ear and endocrine system. The diagnosis is based on the finding of elevated serum lactate, the characteristic histopathological changes in the muscle biopsy, and decreased activities of mitochondrial respiratory chain enzymes. In many cases, the underlying molecular defect in the mtDNA can be identified. The efficacy of pharmacological therapies (e.g., coenzyme Q) has not been established so far. Symptomatic ophthalmological treatment includes ptosis and strabismus surgery. Early cardiac pacemaker implantation may be life-saving.
引用
收藏
页码:133 / 142
页数:10
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