Understanding COPE with Chronic Progressive External Ophthalmoplegia

Chronic Progressive External Ophthalmoplegia (CPEO), also known as Kearns-Sayre Syndrome, is a rare but serious mitochondrial disorder that can affect many parts of the body, including the eyes. It is often difficult to diagnose and can cause vision loss, fatigue, and muscle weakness. Those with CPEO do have options for effective treatments and ways to improve their overall health.

Symptoms of Chronic Progressive External Ophthalmoplegia

The major symptom of CPEO is drooping or paralyzing of the eyelids, known as ophthalmoplegia. Additional symptoms typically include:

• Decreased vision
• Difficulty straightening the eyes
• Double vision
• Nystagmus, or rapid eye movements
• Loss of peripheral vision
• Weakness of the eye muscles
• Fatigue
• Heart Conductions defects

Risk Factors for CPEO

CPEO occurs due to mutations in the mitochondria. Mitochondria are a type of “powerhouse” in the cells of the body and create energy needed to move muscles and other parts of the body. People can be predisposed to developing CPEO if they have a family history of the disorder or have other mitochondrial diseases.

Diagnosing CPEO

If initial symptoms of CPEO are present, a doctor may order a muscle biopsy to look at the muscle tissue and mitochondria. Additional tests such as an electrocardiogram and blood tests may also be ordered in order to check for heart conduction defects or risk of stroke, which can be associated with CPEO. A genetic test may also be requested.

Treating CPEO

Treatment for CPEO focuses on managing the symptoms and improving an individual’s quality of life. Symptoms may be managed with medications such as carbamazepine in order to reduce the frequency and intensity of muscle contractions. Physical therapy may be recommended to restore strength, improve mobility, and reduce fatigue. Eye drops may be used to reduce symptoms such as drooping of the eyelids and double vision. Surgery may also be recommended for some cases.

Living with CPEO

Work closely with your doctor to develop an effective treatment plan that works for you. Taking good care of your health is key – practice healthy eating, stay active, manage stress, and get plenty of rest. This can help you cope with the symptoms of CPEO, as well as maintain a healthy lifestyle. Additionally, don’t hesitate to seek support from family and friends, as well as support groups for those living with a mitochondrial disorder.