Tolosa-Hunt syndrome (THS) is a rare disorder impacting the eye and is characterized by severe headaches and decreased and excruciating eye movements (ophthalmoplegia). It affects both men and women and is rarely seen in individuals younger than 20. There are hardly any noted pediatric cases of Tolosa-Hunt Syndrome.
What is Tolosa-Hunt Syndrome?
The Tolosa-Hunt Syndrome is a condition that usually affects only one eye and in most cases, causes sharp pain and decreased eye movement. The symptoms last for a few days to weeks and often go away without intervention; they may recur randomly.
Affected individuals are likely to show signs of paralysis of certain cranial nerves, resulting in the drooping of the upper eyelid (ptosis), double vision (diplopia), enlarged pupil, and facial numbness. The affected eye often protrudes abnormally.
The condition is also known by different names including ophthalmoplegia, painful, ophthalmoplegia, recurrent, and ophthalmoplegia syndrome.
Causes of Tolosa-Hunt Syndrome
The exact cause of Tolosa-Hunt syndrome is unknown, but the disorder is thought to be associated with the inflammation of the cavernous sinus and superior orbital fissure, located behind the eye. Other causes of painful ophthalmoplegia include tumors, vasculitis, basal meningitis, sarcoidosis, diabetes mellitus, and ophthalmoplegic migraine.
Symptoms of Tolosa-Hunt Syndrome
This condition of the eye presents the following symptoms:
- Persistent hemicranial or periorbital pain
- An illusion of spinning movements referred to as vertigo, where the patient experiences disability in their normal mobility
- Cranial nerve involvement affecting any or all cranial nerves, the optic nerve, and periarterial sympathetic nerves
- Paraesthesia over the forehead
- Paralysis of eye muscles and or muscles surrounding the eyes
- Unilateral or bilateral lesions
- Normal, pale or swollen optic disc
- Blurred vision
- Nausea and vomiting
Diagnosis of Tolosa-Hunt Syndrome
Investigations are mostly aimed at eliminating other causes of symptoms. If Tolosa-Hunt syndrome is suspected, investigations may include:
- Entire blood count and other blood tests
- Functioning of thyroid-releasing glands
- Head and or eyes scan for delineating any abnormalities (benign or malignant neoplasm tumors)
- Syphilis serology
- Antinuclear antibody, anti-double-stranded DNA, and anti-smooth muscle antibodies.
- Serum protein electrophoresis
- Lumbar puncture and examination of cerebrospinal fluid (CSF)
- MRI scans or CT scan to study the cavernous sinus and orbital apex
- Biopsies
Other conditions that may mimic THS, but do not show up on MRIs include trauma, vascular lesions, tumors, infections, giant cell arteritis, eosinophilic granuloma, diabetic ophthalmoplegia and migraines.
Treatment Strategies for Tolosa-Hunt Syndrome
If left untreated, the painful episodes linked to Tolosa-Hunt syndrome typically last up to eight weeks. But the following common treatment strategies could be used for the condition:
- Corticosteroids: Most preferred treatment, usually provides substantial pain relief within 24-72 hours of the first dose. Ophthalmoparesis may take weeks to months to get cured and its healing depends on the degree of inflammation and the aggressiveness of therapy.
- Azathioprine (Imuran) and Methotrexate: Most commonly used immune-suppressive medicines to reduce the immune system’s reaction to the medication.
- Radiotherapy: Doctors sometimes prescribe radiotherapy for patients with a relapse or corticodependence.
Even though the Tolosa-Hunt Syndrome is a rare condition, if you are ever diagnosed with it, make sure you take the right medications and follow the care instructions provided by your doctor for complete recovery.
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References
Tolosa–Hunt syndrome. (2018, July 03). Retrieved from https://en.wikipedia.org/wiki/Tolosa–Hunt_syndrome
Tolosa-Hunt Syndrome: Background, Pathophysiology, Epidemiology. (2018, April 12). Retrieved from https://emedicine.medscape.com/article/1146714-overview
Tolosa Hunt Syndrome. (n.d.). Retrieved from https://rarediseases.org/rare-diseases/tolosa-hunt-syndrome/
