What Is Ophthalmoplegic Migraine?: An Overview

Ophthalmoplegic migraine,  more commonly known now as recurrent painful ophthalmoplegic neuropathy (RPON), is a medical condition that impacts the nervous system. For sufferers of the condition, there can be head pain and dysfunction in the eye muscles, creating a condition where eye movement can become difficult. 

RPON affects a small part of the population, around 0.7 out of 1 million people a year. That makes it an extremely rare disease. However, it makes it no less a serious health matter. The condition typically starts with pain on one side of the head.

Some mistakenly associate migraines with RPON, but head pain and RPON are different conditions. The pain associated with RPONis more a form of headache. 

RPON is a disorder where nerves in the eyes get damaged. It involves the third cranial nerve, although there are also rare cases involving the fourth and sixth cranial nerve. 

RPON is transient, meaning symptoms can come and go. While there are adult cases (where many involve women), RPON is more common in children. 

Symptoms of RPON

The condition usually starts in childhood, but it’s not unusual for symptoms to start in adolescence. Most sufferers of the condition are female. Among other unconfirmed theories, diabetes and hypertension may be influential as the conditions restrict blood flow.

Symptoms can show up periodically, but they can last for hours or weeks. There are rare instances where the damage is permanent.

Here are common symptoms of RPON.

• Sensitivity to light

• Paralysis or weakness in muscles in or around either or both eyes

• Pain in the vicinity of the eyeballs

• Eyes moving or drifting out of alignment

• Different sized pupils 

• Double vision

• Drooping of upper eyelids

• Vomiting or nausea

There can be throbbing pain that feels like a migraine. The symptom typically surfaces on the same side of the head or face.

Diagnosing ophthalmoplegic migraine/RPON

There are no specific tests to confirm RPON. It is more of a diagnosis of exclusion, where the doctors will rule out other potential causes.

If you have had no less than two episodes and no other diagnosis applies, the doctor may diagnose RPON.

The medical community uses one or more of the following to come to potential conclusions.

Physical diagnosis

Doctors once believed the condition was a form of migraine. Recent studies led to the belief that RPON is more of a nerve-related condition. The associated pain is neuralgia, which means pain in the nerve pathway.

Usually, RPON starts with a child suffering from episodic headaches. Ophthalmoplegia (paralysis of the muscles that control the movements of the eye) is considered by determining a third cranial nerve innervation. If there is a possible involvement of the oculomotor nerve, the clinician begins to look for signs of ptosis (a condition where the upper eyelid droops) and dysfunction in the upward, medial, and downward gaze.

Associated headaches are linked to trigeminal sensory fiber irritation. That is secondary to the third nerve’s impact on post-viral inflammation. The condition activates the trigeminovascular system, particularly in sufferers predisposed to migraines. That triggers the headaches.

Still, RPON headaches are not necessarily migraines or accompanied by conventional migraine symptoms. The findings show only about one-third of patients¹ endured classic characteristics of migraine. The report further found nausea in 66% of cases, photophobia (eye discomfort in bright light) present in 65%, and regurgitation in 69%.

Ophthalmologic examination

Optometrists and ophthalmologists specialize in eye health. They will perform eye examinations or might refer you to specialists for more detailed examinations.

There are several approaches the doctor may take. Clinicians may use an ophthalmoscope or fundoscopy. It’s a common screening device with numerous lenses and a light attachment. It checks for damage or abnormalities in the retina, blood vessels, and optic disc. The ophthalmoscope can reveal a reduction in blood flow or damage to an affected eye.

Neurologic examination

A neurologic exam does many things and can lead to an RPON diagnosis. The neurological exam can check cranial nerves and muscle strength. RPON is directly linked to those bodily functions.

A detailed examination can take a considerable time, but the basics are done in less than 5 minutes, finding deficits that prompt further investigation.

However, recognizing neurological red flags is a challenge. The exam is often tailored to locate a specific disease. Indeed, there’s no true test for finding RPON, but it can find signs that warrant further investigation. 

Clinical diagnosis

Using physical examinations and health history, doctors will try to identify conditions of RPON. Doctors may conduct anything from blood tests to imaging tests. 

After an initial diagnosis, doctors may have the information they need to move forward. Speculation entails your symptoms have a link to inflammatory cranial neuropathy. The diagnosis can help narrow the scope of the prognosis.

Differential diagnosis

A differential diagnosis involves investigating a series of conditions sharing the same symptoms associated with RPON. Clinicians perform a series of tests to eliminate similar conditions with similar symptoms, streamlining the lists of possibilities. 

As RPON is difficult to determine, a differential diagnosis is a sound way to reach a decision. 

Other considerations

Here is a list of conditions that can turn up while investigating systems of RPON.

Myasthenia gravis 

Myasthenia gravis provokes muscle weakness. The condition can worsen after activity and improve following rest. The condition has an impact on eye and eyelid control, facial expression, talking, and chewing and swallowing. The disorder can be sudden, with symptoms often misdiagnosed.

Though Myasthenia gravis can mimic RPON, the former can lead to respiratory failure and demand emergency medical treatment. 

Acetylcholine receptor

Acetylcholine receptors play a part in a range of pathological and physiological functions. You can see muscle weakness, including around the eye. 

Muscles are not receiving neurotransmissions that prevent normal function. Without acetylcholine, eyes can have, among other problems, the inability to constrict.

Painful ophthalmoplegia

Painful ophthalmoplegia is clinically diagnosed as a defect in the sensory facility. It’s characterized by hermicranial or periorbital pain accompanied by oculomotor paralysis. Causes of the condition can be vascular, neoplasmic, infection-based, inflammatory, or diabetic. 

Diagnostic tests and procedures

Let’s take a look at tests and procedures the medical community turns to identify RPON.

Magnetic resonance imaging (MRI)

An MRI has shown value in diagnosing a range of complex conditions, but it does not necessarily create findings that demonstrate RPON. 

MRIs can’t narrow the field regarding many cranial conditions, including tension or cluster headaches. What they do is eliminate medical conditions akin to RPON, helping to narrow the list of conditions. 

Blood tests

Like much other medical investigation, blood tests cannot determine if someone has RPON. However, blood tests rule out clots and infections that can affect function. The tests will give clinicians more information about what needs to be done next.

Angiography

Angiography creates images of blood vessels. Doctors use the results to look for aneurysms or the results of an aneurysm. 

For instance, a brain aneurysm can impact the brain and eye function, both of which are components of RPON. Eliminating aneurysms with angiography narrows the gap in RPON.

Spinal taps

A spinal tap is a procedure involving inserting a needle into the spine. It collects bone marrow to look for infections that influence certain medical complications. 

Chest X-rays

X-rays may uncover sarcoidosis, which causes inflammatory conditions. Inflammation is a symptom of RPON and with other procedures in conjunction with a chest X-ray, clinicians can further investigate and potentially learn more about a patient having RPON.

Treatments for RPON

Frequently, RPON consists of episodes that resolve themselves with no treatment. When this is not the case, treatment options are available. Doctors can treat subsequent episodes and implement preventative measures for future episodes.

Treatments depend on medicative response and overall health. A good example is the use of intravenous steroids. A compound like prednisone is a valuable resource for some sufferers yet offers no relief for others. Medications like Lyrica, a pregabalin, demonstrated mild success when steroids did not work at all.

Other treatments for RPON include:

• Botox treatment

• Medication for management of nerve pain or nausea

• Blood pressure treatments such as calcium channel blockers and beta-blockers

• The identification and avoidance of RPON symptom triggers like stress or alcohol

• Eliminating certain foods 

• Pain relievers, including NSAIDs (an anti-inflammatory) or opioids

• Medications that stop muscle spasms 

Remember, there is no cure for RPON. You need to work closely with your clinician to find a treatment that works for you to alleviate symptoms. 

The good news is that RPON is transient and resolves on its own. Multiple attacks may turn persistent, but your medical team will do whatever it takes to manage the condition and help you maintain a healthy lifestyle.

Causes

While research has determined that RPON is not a form of migraine, they still have a number of unanswered questions about it. The medical community reasons the condition is likely the cause of nerve compression, lack of blood to the nerves, or a loss of protective fatty tissue around certain nerves.

Many sufferers of RPON have migraines while others do not. But migraines appear to be a distant symptom as researchers have yet to find a link between RPON and migraines. 

They do see the possibility of migraine history in the family as a risk factor in the development of RPON. They see other risks, such as being a child, a young adult, or a woman.

Prevention

The only way to prevent RPON is to know it is coming. Unfortunately, that’s not likely. Most conditions are not foreseen as there is no way to gauge their possibility. 

Science has yet to determine what inarguably influences the condition. Once the condition gets diagnosed, the best treatment is to identify and avoid the triggers. This will not prevent the condition but may help reduce the number of episodes.

Getting care for RPON

RPON is rare but impactful. You may not be able to easily locate the care required as even skilled ophthalmologists may have no real comprehension of the condition.

Here are a few avenues to take when you find yourself suffering from RPON.

Primary care provider

Your primary care provider is the first step. While there is no true way to diagnose the condition, your doctor will proceed with any number of the options covered here. The physician will order tests or refer you to a specialist for further exams.

Neurologist

The neurologist specializes in defective nerve conditions, of which RPON is one. They will run through family and medical history and perform tests, among others, and diagnose the issue with the knowledge the medical community has regarding RPON.

Insurance company

Check with your health insurance provider. They will likely have a resource of neurologists who operate under their umbrella.

Family and friends 

Talk with friends, family, coworkers, neighbors, or your pharmacist. If possible, you may encounter sufferers who know migraines or headaches or others who do. 

Living with RPON

RPON is rare and stressful. Steroids and nerve medications can help manage the episodes. If not managed, the condition can worsen, and the number of episodes may increase. Damage can also lead to permanent eye problems, including drooping eyelids, pupil dilation, or eye misalignment.

If episodes occur frequently, if attacks last longer than an hour (and proceed to grow longer), or if they result in complicated visual dysfunction, you should schedule a medical assessment as soon as possible.

The lowdown

Ophthalmoplegic migraine, or RPON, remains a mystery in the scientific community and is hard to diagnose. Doctors conduct varying examinations to determine a diagnosis and treat its symptoms. Treatments may include medication or behavioral adjustments. 

While RPON is difficult to diagnose and there is no cure, there are ways to deal with it. Consult with your doctor to manage its symptoms.