Tolosa-Hunt syndrome is a rare disease which is considered as a benign condition. It is characterized by painful unilateral headaches around the sides and back part of the eye area usually caused by inflammation in the superior orbital fissure or cavernous sinus. When such disease is left untreated it may lead into permanent neurologic deficits.
It is estimated that one case per million of people yearly can acquire this type of disease and any person whether male or female with ages above 20 are equally affected. This disorder is uncommon to residents of the United States.
Pathophysiology
Tolosa-Hunt syndrome is characterized by a non-specific inflammation within the cavernous sinus area. The inflammation causes pressure which results to dysfunction of cavernous sinus structure. Disordered eye movements usually occur when cranial nerves are damaged caused by an inflammation. Pupillary dysfunction may develop if an injury to the sympathetic fibers occur surrounding the oculomotor nerve. Involvement of the trigeminal nerve may cause paresthesia in the forehead.
Causes of Tolosa-Hunt Syndrome
The exact cause of Tolosa-Hunt syndrome is still not known but it is presumed that the main causes are inflammation in the sides or behind the eyes.
Here are some conditions that have similar characteristics or diseases associated with Tolosa-Hunt syndrome and their corresponding causes:
• Unilateral headaches are pains felt at one part of the head of which the causes include migraine and temporal arteritis.
• Ophthalmoplegia is a disorder characterized by paralysis of the eye muscles. Causes include brain tumor, meningitis, brain injury, multiple sclerosis, trauma, diabetes, and many more.
• Extraocular palsies are dysfunctions of the extraocular motor nerve palsies where each of these nerves is responsible for each specific muscle. Causes include diabetic neuropathy, brain tumor, head trauma, multiple sclerosis, aneurysm, and cerebrovascular accident.
• Fever is an increase in temperature of the body and is usually the body’s natural self defense from infections. Fever can be caused by infection, inflammatory diseases, and other conditions that may lead into fever.
• Diplopia is a medical symptom caused by multiple sclerosis, graves’ ophthalmopathy, myasthenia gravis, botulism, tumor and blood clot behind eyes, head injury, eye muscle swelling, damage to eye controlling nerve muscles, and many more.
• Chronic fatigue is a condition wherein a severe type of fatigue is not relieved by a simple rest and main cause is still unknown but some suspects it’s caused by an infection.
• Exophthalamos is a protrusion in one or both eyes. Possible causes include eye blood clot, eye tumor, aneurysm, sinusitis, tumor, inflammatory disease, retracted eyelid, and many more.
• Arthralgia is one or more sharp and stabbing pain in the joints. Causes are arthritis, injury, infection, and other ailments.
• Vertigo is a symptom of the feeling of floating or spinning even when keeping still. Causes include infection, side effects from medications, injuries, and disorders.
Symptoms of Tolosa-Hunt Syndrome
Symptoms are usually at one part of the head with severe, sharp pain in the muscles surrounding the eyes. This pain may be mild to severe and can be constant and intense. As the inflammation increases, surrounding nerves in the face are also affected which leads to other symptoms. Patients with these other symptoms may experience various facial nerve paralyses, drooping in the upper eye lid, double vision, chronic fatigue, fever, athralgia, vertigo, may sometime feel protrusion at one or both the eye balls, eye numbness or tingling in the forehead, ophthalmoplegia, and sensitivity to light. When these eye problems are left untreated it may lead into vision loss or blindness.
Diagnosis of Tolosa-Hunt Syndrome
International Headache Society gives out certain criteria for patients to identify if they are suffering from Tolosa-Hunt syndrome, these are:
• For at least 8 weeks with no treatment, an eye pain usually on one side is felt
• Irritation in the third, fourth, and sixth cranial nerves
• Pain symptoms resolved within 48 hours after taking steroid medications
• Exclusions of other conditions that need to be diagnosed by appropriate testing method
Appropriate testing methods for specific conditions are as follows:
• Clinical presentation
• Laboratory tests
• LP results
• Neuroimaging results
• Clinical response to corticosteroids
• Complete blood count
• Thyroid function test
• Serum protein electrophoresis
• MRI scans
• Magnetic resonance angiography
• CT scan in the brain
• Direct tissue biopsy is needed to confirm diagnosis and help rule out neoplasm, plasma cell infiltrations, giant cell granulomas, and proliferation of fibroblasts
Treatment of Tolosa-Hunt Syndrome
Tolosa-Hunt syndrome is fortunately not a fatal condition. Steroid medications can help treat a person with Tolosa-hunt syndrome which provides pain relief for at least 24 to 72 hours. Though resolving eye vision and forehead numbness problems may take several weeks or even months, it doesn’t guarantee complete treatment. A possibility of re-occurring may happen.
Tolosa-Hunt syndrome mimics other types of syndrome, so patients should report new occurring symptoms and have them diagnosed to be given the right treatment. And close clinical and MRI follow-up is necessary for people diagnosed with Tolosa-hunt syndrome.
