Definition
Chronic ophthalmoplegia is characterised by progressive weakness of ocular muscles and levator muscle of the upper eyelid. The condition is mainly manifested in adults. It may be totally and permanently isolated, however in a minority of cases it is associated with skeletal myopathy, which causes abnormal fatigability and even permanent muscle weakness. In this case the affection is still termed isolated progressive external ophthalmoplegia. A large proportion of chronic ophthalmoplegias presents with multisystemic pattern of signs: neurological signs (hearing loss, retinopathy, cerebellar disorders, peripheral neuropathy, etc.), endocrine (diabetes, hypogonadism, hypoparathyroidism, etc.), kidney (kidney failure, tubulopathy, etc.), and heart disorders (conduction disorders, myocardiopathy, etc.).
Index Terms
Progressive external ophthalmoplegiaGraefe diseasePEO - [progressive external ophthalmoplegia]ophthalmoplegia plus syndromeChronic progressive external ophthalmoplegiaCPEO - [Chronic progressive external ophthalmoplegia]chronic progressive ophthalmoplegiaProgressive external ophthalmoplegia, autosomal dominantPEOA - [Progressive external ophthalmoplegia, autosomal dominant]Progressive external ophthalmoplegia, autosomal dominant, type 1PEOA1 - [Progressive external ophthalmoplegia, autosomal dominant, type 1]Progressive external ophthalmoplegia, autosomal dominant, type 2Progressive external ophthalmoplegia, autosomal dominant, type 3PEOA3 - [progressive external ophthalmoplegia, autosomal dominant, type 3]Progressive external ophthalmoplegia, autosomal dominant, type 4Progressive external ophthalmoplegia, autosomal dominant, type 5PEOA5 - [progressive external ophthalmoplegia, autosomal dominant, type 5]Progressive external ophthalmoplegia, autosomal recessiveKearns-Sayre syndromeKearns-Sayre mitochondrial cytopathyMitochondrial ocular myopathyChronic external ophthalmoplegia, pigmentary degeneration of retina, myopathy and cardiomyopathyoculocraniosomatic neuromuscular disorderKSS - [Kearns-Sayre syndrome]oculocraniosomatic syndromeoculocraniosomatic dystrophyOCS - [oculocraniosomatic] syndrome