Tetracycline delays ocular motility decline in chronic progressive external ophthalmoplegia

Abstract

Chronic progressive external ophthalmoplegia (CPEO) is a mitochondrial cytopathy characterized by bilateral ptosis during adolescence, followed later by limitation of extraocular muscle movement and diplopia.1 The biochemical defect consists of mutations or deletions of mitochondrial DNA genes that encode respiratory chain enzymes involved in adenosine triphosphate (ATP) generation and its subsequent translocation from the mitochondria.1 To date, there is no definitive treatment that reverses or halts the progression of the muscle weakness. Herein, we report improvement in ocular motility in a patient with CPEO following treatment with tetracycline. The retrospective review was approved by the institutional review board. A 47-year-old woman, with bilateral upper eyelid ptosis since childhood, underwent ptosis repair in 1987, at age 28. There was a gradual return of the ptosis over the ensuing years. By age 33, she began experiencing diplopia. She had otherwise enjoyed excellent health. Her family history was significant for six brothers and three sisters with ptosis. We initially evaluated her in October 1997, at age 38, for diplopia and ptosis. Her visual acuity was 20/20 for both eyes. There was 6- to …