Recurrent painful ophthalmoplegic neuropathy (RPON) was once classified as a type of migraine, but it’s now believed to be a distinct condition.
Migraine is a neurological condition, meaning it has to do with your brain and nervous system. It’s often associated with severe and frequent headaches, though it can also have other symptoms like nausea and sensitivity to:
Neuropathy, on the other hand, is any condition related to damage or improper nerve functioning. Neuropathies are similar to but distinct from neurological conditions.
There are different types of migraine. At one time, there was a type of migraine called ophthalmoplegic migraine. But in 2013, the International Headache Society reclassified it as recurrent painful ophthalmoplegic neuropathy (RPON), stating that the condition fit in with other neuropathies better than it did with migraine.
Let’s get into the specifics of RPON and answer common questions related to this condition.
Anyone can be diagnosed with RPON, but it most commonly affects children, often younger than 10 years old.
RPON usually begins with a headache around or behind your eyes that lasts a few days or a week. These headaches do not include the auras often associated with migraine.
The other primary symptom of RPON is ophthalmoplegia. This is a weakness or paralysis of the muscles controlling the movement of your eyes and eyelids. Ophthalmoplegia could begin at the same time as a headache or start up to 2 weeks later. It can last up to 3 months.
These episodes usually reoccur. The time between them could be as little as a week or as long as 5 or more years.
RPON is a relatively rare condition. It’s not very well understood, reflected by the fact that it was reclassified from a type of migraine to a type of neuropathy only a few years ago.
The causes for RPON are unknown, but experts have pointed to compression, ischemia, and demyelination — or a combination of the three — as potential causes.
Compression theoriesargue that the walls of the arteries in your head can become inflamed, which would, in turn, compress the nearby nerves that control your eyes.
Ischemia, or decreased blood flow, to your cranial nerves for various reasons may cause RPON.
Finally, there’s a protective coating around your neurons called myelin. If the myelin is damaged or destroyed, it can cause many problems, which might include RPON. This is called demyelination.
In the short term, the severe headaches associated with RPON can cause you to miss out on daily activities like attending school, work, or performing usual tasks around your home.
Because the causes of RPON are not fully understood, the risk factors are also not well known.
RPON appears to be more common in people who also have migraine or who have a family history of migraine. It’s also typically diagnosed in children, though it can affect people of any age.
Episodes of RPON may be infrequent, and symptoms like severe headaches and paralysis of the eye muscles can overlap with severe medical emergencies like stroke.
Consequently, if you’re experiencing signs of RPON, seek medical attention.
Recurrent RPON episodes can also lead to lasting damage. Seeking treatment may be the best way to prevent permanent effects.
A doctor typically diagnoses RPON after a physical exam and a medical history.
RPON most often occurs in children with a history of severe headaches. Different cranial nerves might be affected, each associated with different symptoms.
The oculomotor nerve is affected most often, and the symptoms include:
- pupil dilation
- drooping eyelids
- diminished ability to move your gaze upward, downward, or side to side
These symptoms might affect either eye or both.
There is currently no standard treatment for RPON. Because the condition is rare, no extensive studies have determined what treatments work best.
In limited studies, treatments reported to have had at least some level of success include:
It might be possible to prevent or reduce RPON episodes by taking medication. Medications usually take the form of:
These medications are not usually recommended unless you’re experiencing frequent RPON episodes.
If you usually have months or years between your RPON episodes, there are currently no recommended methods to prevent them.
You might still have some unanswered questions about RPON. Let’s briefly take a look at some of the most common.
How long does an ophthalmoplegic migraine episode last?
Ophthalmoplegia, or the weakness of the muscles that control eye movement, from RPON can last for weeks or even months.
What is the most common nerve involved in ophthalmoplegic migraine?
RPON affects one or more of your ocular cranial nerves, but the most common is the oculomotor nerve (CN III).
Is ophthalmoplegic migraine the same as ocular migraine?
No, RPON is currently understood to be a neuropathy, which is distinct from migraine. An ocular migraine is a type of migraine related to your eyes.
Recurrent painful ophthalmoplegic neuropathy (RPON) — formerly called ophthalmoplegic migraine — is a rare and poorly understood condition that typically involves severe headaches and temporary loss of eye functions. It commonly affects children.
Though there are treatments for RPON, the rarity of the condition means there is no standard treatment. Because the symptoms could also overlap with those of serious medical emergencies, people who think they might have RPON should seek medical attention.