9C82.0

ICD-11 MMS 2026-01

Progressive external ophthalmoplegia

Description

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 ophthalmoplegia
  • Graefe disease
  • PEO - [progressive external ophthalmoplegia]
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  • ophthalmoplegia plus syndrome
  • Chronic progressive external ophthalmoplegia
  • CPEO - [Chronic progressive external ophthalmoplegia]
  • chronic progressive ophthalmoplegia
  • Progressive external ophthalmoplegia, autosomal dominant
  • PEOA - [Progressive external ophthalmoplegia, autosomal dominant]
  • Progressive external ophthalmoplegia, autosomal dominant, type 1
  • PEOA1 - [Progressive external ophthalmoplegia, autosomal dominant, type 1]
  • Progressive external ophthalmoplegia, autosomal dominant, type 2
  • Progressive external ophthalmoplegia, autosomal dominant, type 3
  • PEOA3 - [progressive external ophthalmoplegia, autosomal dominant, type 3]
  • Progressive external ophthalmoplegia, autosomal dominant, type 4
  • Progressive external ophthalmoplegia, autosomal dominant, type 5
  • PEOA5 - [progressive external ophthalmoplegia, autosomal dominant, type 5]
  • Progressive external ophthalmoplegia, autosomal recessive
  • Kearns-Sayre syndrome
  • Chronic external ophthalmoplegia, pigmentary degeneration of retina, myopathy and cardiomyopathy
  • KSS - [Kearns-Sayre syndrome]
  • Kearns-Sayre mitochondrial cytopathy
  • Mitochondrial ocular myopathy
  • OCS - [oculocraniosomatic] syndrome
  • oculocraniosomatic dystrophy
  • oculocraniosomatic neuromuscular disorder
  • oculocraniosomatic syndrome

Exclusions from above levels

  • 01 – Certain infectious or parasitic diseases
  • 05 – Endocrine, nutritional or metabolic diseases
  • 18 – Complications of pregnancy, childbirth and the puerperium
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  • 19 – Certain conditions originating in the perinatal period
  • 22 – Injury, poisoning or certain other consequences of external causes
  • 8A21.0 – Posterior cortical atrophy

Sibling codes

  • 9C82.1 – Muscular dystrophy affecting extraocular muscle
  • 9C82.2 – Congenital cranial dysinnervation syndrome
  • 9C82.3 – Restrictive ophthalmopathy
  • 9C82.4 – Oculomotor apraxia
  • 9C82.Y – Other specified disorders of extraocular muscles
  • 9C82.Z – Disorders of extraocular muscles, unspecified

Foundation entity

Progressive external ophthalmoplegia – http://id.who.int/icd/entity/1698427219

Allowed postcoordination axes

Part of: 9C82: Disorders of extraocular muscles › 09: Diseases of the visual system