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9C82.0 category

Progressive external ophthalmoplegia

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.).

Also indexed as

CPEO - [Chronic progressive external ophthalmoplegia]Chronic external ophthalmoplegia, pigmentary degeneration of retina, myopathy and cardiomyopathyChronic progressive external ophthalmoplegiaGraefe diseaseKSS - [Kearns-Sayre syndrome]Kearns-Sayre mitochondrial cytopathyKearns-Sayre syndromeMitochondrial ocular myopathyOCS - [oculocraniosomatic] syndromePEO - [progressive external ophthalmoplegia]PEOA - [Progressive external ophthalmoplegia, autosomal dominant]PEOA1 - [Progressive external ophthalmoplegia, autosomal dominant, type 1]PEOA3 - [progressive external ophthalmoplegia, autosomal dominant, type 3]PEOA5 - [progressive external ophthalmoplegia, autosomal dominant, type 5]Progressive external ophthalmoplegiaProgressive external ophthalmoplegia, autosomal dominantProgressive external ophthalmoplegia, autosomal dominant, type 1Progressive external ophthalmoplegia, autosomal dominant, type 2Progressive external ophthalmoplegia, autosomal dominant, type 3Progressive external ophthalmoplegia, autosomal dominant, type 4Progressive external ophthalmoplegia, autosomal dominant, type 5Progressive external ophthalmoplegia, autosomal recessivechronic progressive ophthalmoplegiaoculocraniosomatic dystrophyoculocraniosomatic neuromuscular disorderoculocraniosomatic syndromeophthalmoplegia plus syndrome

Nearby in Disorders of extraocular muscles