ICD-11 classes
08 Diseases of the nervous system
Diseases of neuromuscular junction or muscle
Primary disorders of muscles
8C73 — Mitochondrial myopathies
9C82.0 — Progressive external ophthalmoplegia

ICD-11 9C82.0 — Progressive external ophthalmoplegia

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.).

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.

The diagnosis is coded elsewhere:

• Disorders of extraocular muscles #9929
• Mitochondrial myopathies #10052