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Genetic and Rare Diseases Information Center (GARD)

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Chronic progressive external ophthalmoplegia

Other Names for this Disease
  • CPEO
  • Progressive external ophthalmoplegia
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Your Question

Is chronic progressive external ophthalmoplegia fatal? If so, what is the life expectancy?

Our Answer

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What is the long-term outlook (prognosis) for individuals with chronic progressive external ophthalmoplegia?

Chronic progressive external ophthalmoplegia (CPEO) can be an isolated condition, or it can occur as part of other underlying conditions, such as ataxia neuropathy spectrum and Kearns-Sayre syndrome. These conditions may involve not only CPEO, but various additional features that are not shared by most individuals with CPEO.[1] Individuals with isolated CPEO generally have a normal life expectancy. While symptoms tend to worsen over time, the specific symptoms and their severity can vary greatly from person to person. Therefore, when symptoms first appear, the course of the condition is very difficult to predict.[2][3]

For individuals with additional symptoms or another underlying condition associated with CPEO, the prognosis depends on the specific signs and symptoms present and/or the outlook associated with the underlying condition in the affected individual. For this reason, obtaining an accurate diagnosis is very important.
Last updated: 10/10/2013

What are the signs and symptoms of chronic progressive external ophthalmoplegia?

The signs and symptoms of chronic progressive external ophthalmoplegia (CPEO) typically begin in young adults between the ages of 18 and 40.[2][1] The most common symptoms in affected individuals include drooping eyelids (ptosis) and weakness or paralysis of the eye muscles (ophthalmoplegia). The condition may be unilateral (affecting one eye) or bilateral (affecting both eyes).[4] Some affected individuals also have weakness of the skeletal muscles (myopathy), specifically of the arms, legs, and/or neck. This may be especially noticeable during exercise.[2][1] Muscle weakness may also cause difficulty swallowing (dysphagia).[1]

Sometimes, CPEO may be associated with other signs and symptoms. In these cases, the condition is referred to as "progressive external ophthalmoplegia plus" (PEO+). Additional signs and symptoms can include hearing loss caused by nerve damage in the inner ear (sensorineural hearing loss), weakness and loss of sensation in the limbs due to nerve damage (neuropathy), impaired muscle coordination (ataxia), a pattern of movement abnormalities known as parkinsonism, or depression.[1]

CPEO can also occur as part of other underlying conditions such as Kearns-Sayre syndrome. These conditions may not only involve CPEO, but various additional features that are not shared by most individuals with CPEO.[1]
Last updated: 10/10/2013