Tolosa-Hunt Syndrome- Causes, Symptoms, Diagnosis, and Management

What Is Tolosa-Hunt Syndrome?

It is a recurrent or episodic inflammation behind the orbit which may involve the paralysis of the third, fourth, or sixth cranial nerves. These cranial nerves control the muscles that move the eyeball. Due to this condition, a person suffers from orbital pain or headache. These symptoms usually subside on their own without any treatment. It is a rare condition that is only seen in around one in a million cases. Idiopathic swelling of the cavernous sinus can also be associated with this condition which typically affects only one side. This causes a myriad of symptoms such as double vision, paralysis, and numbness. Moreover, the affected eye is usually seen abruptly bulging due to this condition. Although the condition is believed to be benign, there are higher chances of recurrence along with neurological defects. Patients with this condition are recommended immunosuppressants for longer periods. Also, the condition is known as

• Ophthalmoplegia syndrome.

• Painful ophthalmoplegia.

• Recurrent ophthalmoplegia.

What Are the Causes of Tolosa-Hunt Syndrome?

The etiological factor affecting this condition is still unknown. However, the following is the theory behind its possible association with Tolosa-Hunt syndrome:

• Autoimmune Response: Inflammation of the orbital fissure and cranial sinus occurs due to the accumulation of granulomatous cells. It is the result of the body’s weak immune system, which cannot defend against the foreign organism and causes generalized inflammation of the blood vessels and the body.

What Are the Signs and Symptoms of Tolosa-Hunt Syndrome?

The following are the signs and symptoms associated with Tolosa-Hunt syndrome:

• Severe periorbital headache.

• Dropping of the upper eyelid.

• Large pupil.

• Numbness of the face.

• Chronic fatigue.

• Pain.

• Reduced eye movements.

• Double vision.

• Paralysis.

• Fatigue.

How to Diagnose Tolosa-Hunt Syndrome?

There are the following diagnostic methods used for Tolosa-Hunt syndrome:

• Diagnostic Criteria Are Given by the International Headache Society: International headache society has given some criteria for diagnosing this condition, such as:

  • Unilateral headache.

  • Inflammation of cavernous sinus or orbital fissure.

  • Paralysis of one or more cranial nerves on the same side.

  • If the headache is localized around the eye.

• Neuroradiological Studies:

  • Computed Tomography (CT Scan): CT scan is used with a high resolution to see the abnormal soft tissue changes.

  • Magnetic Resonance Imaging (MRI): MRI is an excellent diagnostic tool used for getting a good coronal view. With the help of this tool, the thickening of soft tissue in the cavernous sinus can be evaluated. Moreover, it also shows the lateral wall convexity of the cavernous sinus and its extension into orbit.

• Blood and Cerebrospinal Fluid (CSF) Study: This evaluation method is used to test ophthalmoplegia and its possible involvement.

  • Hematological Tests:

    • Complete blood count.

    • Serum chemistry (glucose, electrolytes, liver, and renal function).

    • Erythrocyte sedimentation rate.

    • C-reactive protein.

    • Hemoglobin A1c.

    • Fluorescent treponemal antibody test.

    • Antinuclear antibody.

    • Anti-dsDNA antibody.

    • Anti-Sm antibody.

    • Serum protein electrophoresis.

    • Antinuclear cytoplasmic antibody.

    • Angiotensin-converting enzyme. 

  • Cerebrospinal Fluid:

    • Opening pressure.

    • Cell count and differential.

    • Protein.

    • Glucose.

What Are the Complications Associated With Tolosa-Hunt Syndrome?

The following are the complications associated with Tolosa-Hunt syndrome:

• Paralysis of an eyelid or face.

• Double vision.

• Severe headache.

How to Manage Tolosa-Hunt Syndrome?

Tolosa-Hunt syndrome is usually managed with short-term steroid drug therapy. Symptoms, such as pain, usually subside in 20 days without treatment. However, with medication such as steroids, the pain goes away within 2 to 24 hours. The following is the management option for Tolosa-Hunt syndrome:

• Glucocorticoids: An ophthalmologist gives glucocorticoids according to the patient's condition and physical capacity. The dose of this medication varies from person to person. Usually, with this medication, the patient might feel an immediate effect on the pain. However, cranial nerve palsies may or may not improve with it. The treatment involves high-dose steroid therapy with a gradually decreasing dose over a month or week.

• Immunosuppressant Therapy: This is the second line of treatment, and not every patient requires it. This medication can be given along with steroid therapy. Additionally, Azathioprine, Methotrexate, or Cyclosporine are the medication that can be used for this condition.

What Are the Differential Diagnosis of Tolosa-Hunt Syndrome?

The following are the differential diagnoses associated with Tolosa-Hunt syndrome:

• Ischemic Disease: Such as ischemic hemorrhage (when the blood vessel to the brain is clogged and leaks) or diabetic mononeuropathy.

• Syphilis: It is a sexually transmitted disease caused by bacterial infection.

• Basal Meningitis: This condition usually develops due to the rupture of the intracranial tuberculoma.

• Aneurysm: Aneurysm is an unusual elevation of the blood vessel caused by high blood pressure. Usually, this condition is seen in the brain, behind the knee, intestine, or spleen.

• Carotid-Cavernous Fistula: It is the abrupt joining between the internal carotid artery and the cavernous sinus.

• Cavernous Sinus Thrombosis: It is a fatal condition caused by a blood clot in the cavernous sinus.

• Sarcoidosis: Sarcoidosis is a condition caused by an overgrowth of inflammatory cells in the body. It is usually seen in the lungs, skin, or eyes.

• Varicella Zoster: It is a fatal viral infection caused by the herpes virus.

• Polyarteritis Nodosa: It is a rare condition that causes arterial inflammation and its weakening. It affects multiple systems and organs of the body.

• Neurosarcoidosis: It is the condition where any part of the nervous system has abrupt cell accumulation.

• Bechet’s Disease: It is a rare condition that causes abrupt inflammation of any part of the body.

• Myasthenia Gravis: It is an autoimmune disorder that majorly affects the skeletal muscles of the body.

• Lupus: When the body’s immune system attacks its cells, this condition arises. Lupus affects multiple organs of the body, such as skin, lungs, joints, and blood cells.

• Migraine: It is severe and unbearable throbbing pain of the head, which is usually seen as unilateral.

Conclusion

Tolosa-Hunt syndrome is a condition that can cause many severe symptoms in the patient. Due to its idiopathic cause, the diagnosis of the condition is very difficult. However, due to the severity and recurrence of the condition, it is essential to follow regular treatment without skipping any dose of the medication. Also, regular follow-up is very important for patients with this condition.