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Tolosa Hunt Syndrome - A Rare & Painful Eye Condition

One out of every million people gets affected by Tolosa Hunt Syndrome (THS) annually. *


But with no race, sex, or region bias, the idiopathic (of unknown cause) eye disease can get to you anytime anywhere. Even little children are not completely immune to it.


Due to the constant work pressure, sitting in front of laptops for long hours has become a norm today. And that too without breaks. To add to that, our growing obsession with cell phones.


Result? Blurry visions, headaches, eye sores, and increased light sensitivity, to name a few. However, every headache, pain, or double vision may not be an outcome of simple eye fatigue. At times, these are the warning signs of a more serious eye disorder in Tolosa Hunt Syndrome.


Thankfully, the illness is a rare occurrence and is not considered fatal. But, it does carry the potential to cause major hindrances in life if left untreated. So, come, let’s delve deep into the issue and stay better prepared in case it chooses to strike you.


What is Tolosa Hunt Syndrome?

Tolosa Hunt Syndrome is a rare eye disorder. The condition is mostly characterized by acute episodic pain in or around the orbit of your eyes. It is also accompanied by weakness or paralysis of eye muscles resulting in painful and restricted eye movements. This has led to its other name ‘Ophthalmoplegia Syndrome’. You may also know it as painful ophthalmoplegia, or recurrent ophthalmoplegia.


In most cases, THS is unilateral and concerns any one of the eyes. But for some unfortunate souls, the condition may affect both eyes.


Tolosa Hunt Syndrome Symptoms

Severe periorbital headache is the most common symptom of Tolosa Hunt Syndrome. But there are other symptoms that should be taken into consideration.



They include:

  • Drooping of the upper lids of the eye (Ptosis)

  • Abnormal protrusion of the concerned eye (Proptosis)

  • Numbness in the face

  • Double vision (Diplopia)

  • Large pupil

  • Nausea

  • Dizziness

  • Stiffness in the neck

  • Discomfort in the eye when exposed to bright light (Photophobia)


Tolosa Hunt Syndrome Causes

We are still in the dark about the exact reason behind the occurrence of Tolosa Hunt Syndrome. But some theories consider THIS to be an autoimmune issue. They believe it to be caused by the abnormal defence response of the body to inflammation in some specific parts behind the eyes. One region in question is the Cavernous Sinus (drainage path for the brain). The other is the Superior Orbital Fissure (the route for many structures from the brain to orbit). In some cases, the inflammation may be granulomatous inflammation. It happens when a certain kind of cell clumps.


THS may also occur due to a generalized form of inflammation. Or as a result of swelling or constriction in cranial blood vessels.


The list of other potential Tolosa Hunt Syndrome causes and triggers comprise of:

  • Traumatic injury

  • Tumors

  • Aneurysm (unnatural bulge in the wall of a blood vessel)


What Is The Affected Population For Tolosa Hunt Syndrome?

The onset of the Tolosa Hunt Syndrome is mostly seen at the age of 41 years.

But there is no guarantee of that. You may be diagnosed with the disorder even when you are below 30 years. In fact, some rare cases have also witnessed children under 10 years to have been affected by THS. There is no gender predisposition either.


Should A Diabetic Person Be Worried Of Tolosa Hunt Syndrome?

International Headache Society classifies Tolosa Hunt Syndrome as a painful cranial neuropathy. Cranial neuropathies are damage to the nerves arising from the brain. They can happen to aged patients who have poorly controlled diabetic conditions. But there is good news.


The simultaneous existence of THS and diabetic cranial neuropathy is quite uncommon.

That being said, one must never compromise when it comes to keeping blood sugar levels in check. Not just to avoid overcomplicating THS but for your general well-being itself.


How Is Tolosa Hunt Syndrome Treated?

To confirm the Tolosa Hunt Syndrome diagnosis, doctors get a detailed clinical examination. They also do a complete review of the medical history of the patient. Specific radiologic tests like CT scans and MRIs are equally essential. These tests help detect inflammations in the Cavernous Sinus and Superior Orbital Fissure.

Following that, they go for the below-mentioned standard therapies to treat the condition.

  1. Corticosteroids Glucocorticoids (a class of Corticosteroids) are steroid hormones. They usually provide pain relief within 24-72 hours and are most trusted as THS treatment. Many patients have shown overall significant improvement in a week with Glucocorticoids.

  2. Steroid-sparing agents (For example, methotrexate, mycophenolate mofetil, or azathioprine)

These are used in selected sections of patients. Mostly to prevent the negative impact of prolonged steroid therapy. Or to stop the disease for a longer period of time.

  1. Radiotherapy

Radiation therapy may be recommended in case steroids are not suited for the patient.

Diagnosis of exclusion is another very important aspect of Tolosa Hunt Syndrome treatment. So, let’s get on with it in our next segment.



Tolosa Hunt Syndrome Differential Diagnosis

The process of differential diagnosis is necessary for the final Tolosa Hunt Syndrome diagnosis. Wondering why? That's because the symptoms share similarities with those of many other health complications. Hence ruling out other potential causes provides further validation.

The list of diseases for Tolosa Hunt Syndrome differential diagnosis includes:

  • Anisocoria (unequal size of the pupil)

  • Cavernous Sinus Thrombosis (infection leading to blood clots in the cavernous sinuses)

  • Migraine Headache

  • Brain Metastasis (Brain tumor)

  • Meningioma (Central Nervous System tumor)

  • Cerebral Aneurysms (a bulging weak spot in the wall of a brain artery)

  • Orbital Cellulitis (bacterial infection of the soft tissues in the eye socket)


Is Tolosa Hunt Syndrome Curable?

The chances of recovering from Tolosa Hunt Syndrome are pretty decent. Corticosteroids are quite effective in this regard. For those who have not gone for any treatment, the pain can last up to 8 weeks on average.


The discomfort can go away almost spontaneously after that.

However, the condition may not get completely cured. Patients may continue to have oscular motor-related (movement of the eyeball) shortcomings.

Around 40- 50% of patients do suffer from relapses.

And it is the younger lot who are more vulnerable than their aged counterparts. Can steroids prevent relapses? Unfortunately, there’s no clear-cut answer to that yet.


Final Thoughts

Tolosa Hunt syndrome is a common cause of Orbital Apex Syndrome (impairment of cranial nerves and optic nerve) which may lead to vision loss. So as soon as you detect Tolosa Hunt Syndrome symptoms, it’s best to consult a professional.


Timely Tolosa Hunt Syndrome treatment can offer you quick relief from the pain.


Also, since this eye disorder is rare, not many people are aware of it. So, if you see anyone with similar worrying signs, direct them to seek proper medical care.

 

The Author :

Dr. Sunil Khattri

sunilkhattri@gmail.com

+91 9811618704


Dr Sunil Khattri MBBS, MS(General Surgery), LLB, is a Medical doctor and is a practicing Advocate in the Supreme Court of India and National Consumer Disputes Redressal Commission, New Delhi.

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