Ophthalmoplegic Migraine/RPON: Overview and More

Ophthalmoplegic migraine is now called recurrent ophthalmoplegic neuropathy, or RPON. Contrary to what the name "ophthalmoplegic migraine" implies, it is actually not a migraine. RPON causes a headache and temporary paralysis of certain nerves around the eye. It is a rare condition affecting about 0.7 out of every million people every year, or approximately 230 people in the United States annually.

This article will address symptoms, causes, and treatment for ophthalmoplegic migraine/RPON, as well as short- and long-term prognosis.

RPON Symptoms

Symptoms of ophthalmoplegic neuropathy/RPON include:

• Headache on one side that can last up to a week
• Light sensitivity
• Nausea
• Vomiting
• Weakness of the eye muscles, or ophthalmoplegia, that may occur immediately or up to two weeks after the headache starts. This weakness may last two weeks to three months. It is caused by nerve damage.

RPON can affect several ocular cranial nerves. A nerve called the third cranial nerve is the nerve most affected by RPON. This nerve controls many of the movements of the eye.

Although ophthalmoplegia typically gets better, it may not go away completely if a person has repeated incidences of RPON. This can lead to long-term weaknesses in eye movement and drooping of the upper eyelid.

Causes of RPON

Researchers are not sure what causes RPON. The recent change of its name from ophthalmoplegic migraine to RPON reflects some of the confusion surrounding the condition. Still, there are some possible causes of RPON that researchers have explored:

• Swelling of the walls of the internal carotid artery or posterior cerebral artery that may happen during a migraine headache: This may compress oculomotor nerves in the sinuses.
• Reduced blood flow, which is also called ischemia: The ischemia may occur in the areas of the body affected by RPON.
• Demyelinating neuropathy or neuritis: This refers to a condition that causes damage to the coverings around the nerve fibers in optic nerves as well as the brain and spinal cord. This can lead to nerve impulses that don't work as they should.

There appear to be some groups of people who are more likely to develop RPON:

• Children under age 10
• Males
• A person with a personal or family history of migraine

Diagnosis

Healthcare providers will use several types of tests to help make an RPON diagnosis. These include:

• Eye and neurological examinations
• Magnetic resonance imaging (MRI) can rule out other causes of symptoms, such as a tumor.
• Computed tomography (CT) imaging
• Blood and cerebrospinal fluid tests to help rule out other causes such as diabetes, infection, or systemic nervous system disorders

To make an RPON diagnosis, your healthcare provider will ask whether you have had this type of attack at least twice.

Treating RPON

Treatments for RPON require further study. Some occurrences do not require any treatment. Treatments that have had some success include:

• Corticosteroids: Also called steroids, this is a type of anti-inflammatory drug used for many health conditions, such as asthma and allergies. In a 2012 study for RPON, the use of corticosteroids helped 14 of 26 patients improve in one to two days, while there was clinical worsening or no benefit in three patients. The effect was unclear in nine patients.
• Botulinum toxin: Also known by the brand name Botox, botulinum toxin is often associated with smoothing facial wrinkles. However, it also has some medical uses. Botulinum toxin has been shown to treat the symptoms of RPON but may not improve long-term occurrences of RPON.
• Pregabalin: This is a type of medicine used for nerve and muscle pain. In a 2019 case report, pregabalin helped resolve symptoms of RPON within a week. The person continued to use pregabalin for a year and then was pain-free for two years.

RPON Prognosis

Many people find that RPON will resolve without treatment. However, if you have several attacks of RPON, the deficits caused by it may become more permanent. These could include persistent dilation of the eyes (called mydriasis) or eyelid drooping (ptosis).

Even if RPON resolves on its own, It still is a good idea to stay in touch with your healthcare provider. Chronic cases can cause certain eye issues in more than half of patients. These include:

• Permanently dilated pupils
• Permanent upper eyelid drooping, also called ptosis
• Eye misalignment

Summary

Once called ophthalmoplegic migraine, RPON is a nerve disorder that leads to the weakness of certain eye muscles and a headache. It is not clear what causes RPON in some people.

Healthcare providers use several approaches to diagnose RPON, including a neurological exam and imaging. There is no well-studied treatment for RPON, but steroids have shown to be effective in some people who have this rare condition.

A Word From Verywell

It can be frustrating to experience a condition like RPON, especially if you don't know what caused it. If you have RPON, work with your healthcare provider to track its symptoms and how often it occurs. If you have both RPON and migraines, try to avoid your migraine triggers. Talk to your healthcare provider about other questions you have regarding RPON.