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An ophthalmoplegic migraine is a painful condition that causes headaches and vision trouble, but it isn’t a migraine at all. Years ago, doctors and researchers thought ophthalmoplegic migraine was a type of migraine, but new research has shown this isn’t the case.

The condition is actually a nerve problem affecting your eyes. That’s why it has been given a more accurate name in recent years: recurrent painful ophthalmoplegic neuropathy (RPON).

Recurrent painful ophthalmoplegic neuropathy (RPON), formerly ophthalmoplegic migraine, is a rare condition that causes pain around your eyes along with headaches. People with this condition often experience double vision and other visual disturbances.

Despite the painful headaches that it causes, ophthalmoplegic migraine isn’t actually a type of migraine. Instead, doctors have learned that the condition is caused by problems in the nerves that control how your eyes move and take in visual information.

In 2018, The International Classification of Headache Disorders, 3rd edition (ICHD-3) was published, and ophthalmoplegic migraine was reclassified as RPON since the condition involves the nerves around your eyes. You might still see and hear the condition referred to as ophthalmoplegic migraine. This can make it confusing and frustrating for people with RPON to find information on their condition.

Both ophthalmoplegic migraine and RPON are the same condition. Ophthalmoplegic migraine is the old name and RPON is the current name.

RPON causes episodes of pain that can come and go. Episodes vary in length. Some episodes might last a few hours, while others can linger on for weeks. In some cases, RPON symptoms can become permanent.

The headaches associated with RPON occur days or weeks before the weakness in the eye muscles occurs.

Headaches can sometimes cause throbbing pain and feel like standard migraine, but RPON headaches don’t always feel like a migraine attack. Other symptoms of RPON include:

  • pain around your eyes
  • weak muscles around the eyes
  • paralyzed muscles around the eyes
  • double vision
  • eyes that drift out of alignment
  • sensitivity to light
  • drooping upper eyelids
  • nausea
  • vomiting
  • headaches that happen on one side of the face

There is no single test for RPON. If you have RPON symptoms, your neurologist will do tests to rule out other conditions that might be causing your symptoms, such as infections, tumors, aneurysm, meningitis, sarcoidosis, or cancer.

RPON can be diagnosed if all other possible causes of your symptoms have been ruled out and if you’ve had at least two episodes. Tests will likely begin with a physical examination of your eyes. This exam will normally include the use of eye drops to dilate your eyes.

You might also have tests such as:

  • Magnetic resonance imaging (MRI). An MRI creates detailed images that can help doctors look for tumors and nerve compression.
  • Blood tests. Blood tests can be used to rule out infections and clots.
  • Angiography. This test creates images of your blood vessels and is used to check for aneurysms.
  • A spinal tap. Spinal taps are done by inserting a needle into your spine to collect tiny samples of bone marrow. This can be used to check for infections and for cancers such as lymphoma or leukemia.
  • A chest X-ray. A chest X-ray can check for inflammation caused by sarcoidosis.

Often, episodes of RPON will resolve on their own without treatment. When treatment is needed, there are options available. These treatments can help you manage an episode and prevent future ones. The exact treatment for you will depend on your overall health and how you respond to treatments.

For example, intravenous steroids such as prednisone are very helpful for some people but provide no symptom relief for others. You and your healthcare professional will work together to find the best treatment for your RPON.

Other treatment options include:

  • Botox injections
  • nerve pain medication such as Lyrica
  • blood pressure medications such as beta-blockers and calcium channel blockers
  • identifying and avoiding things that trigger RPON symptoms such as stress or alcohol

Researchers know that RPON isn’t a type of migraine, but there are still a lot of questions about this condition. Currently, the cause of RPON is unknown. Researchers theorize that RPON might be caused by nerve problems such as nerve compression, loss of protective fatty tissue around the nerves, or a lack of blood flow to the nerves.

Many people with RPON have migraine. Medical researchers don’t know the link between RPON and migraine, but a migraine history is considered a risk factor for developing RPON. Other risk factors include:

  • having a family member with migraine
  • being a child
  • being a young adult
  • being a women

There are no researched ways to prevent RPON because it’s unclear what causes people to develop the condition. If you already have migraine episodes or have been diagnosed with RPON, one of the best things you can do is to identify and avoid your triggers. This might not prevent RPON, but it can reduce the number of episodes you have.

Finding care for RPON

RPON is a painful condition that impacts your life. Since RPON is rare, it can be difficult to find the care you need, but there are options available. Here are a few ideas to help you find a medical professional to help:

  • Your primary care provider (PCP). Your PCP might not be able to diagnose RPON, but they’ll be able to order tests and refer you to a headache specialist.
  • A neurologist. A neurologist is a doctor who specializes in conditions that affect your nerves, including RPON.
  • Your insurance company. You can check with your insurance company to see if they have a list of neurologists who accept your insurance.
  • Friends and family. If you have friends or family who have migraine or other conditions that cause headaches, ask them for information about their neurologist.

There is no cure for RPON. However, many episodes of RPON resolve on their own. In some cases, RPON is a temporary condition that only records a handful of times.

When RPON is chronic, treatments help some people manage the condition with less pain. If RPON is not controlled, repeated episodes could lead to permanent eye problems such as pupil dilation, drooping eyelids, or eye misalignment.

RPON is a rare and painful nerve condition that was once called ophthalmoplegic migraine because of the painful headache it causes. However, RPON is not a type of migraine. Currently, there are no known causes or cures for RPON. Treatments such as steroids and nerve medications may help manage the condition.