Tolosa–Hunt syndrome

Symptoms are usually limited to one side of the head. In most cases, the individual affected will experience intense, sharp pain and paralysis of muscles around the eye. Symptoms may subside without medical intervention, yet recur without a noticeable pattern. Patients with this disorder describe it as almost like being stabbed in the head. The pain also comes from behind the eyes, forehead, and around the temple area. Not only is the disorder painful, but it is also severe. In addition, affected individuals may experience paralysis of various facial nerves and drooping of the upper eyelid (ptosis). Other signs include double vision, fever, chronic fatigue, vertigo or arthralgia. Occasionally, the patient may present with a feeling of protrusion of one or both eyeballs (exophthalmos). Tolosa-Hunt Syndrome should not be mistaken for idiopathic inflammatory orbital pseudotumor (IIPO). These also can return unpredictably, sometimes with months or years. ==Causes==

The cause of Tolosa–Hunt syndrome is not known. The disorder is thought to be, and often assumed to be, associated with inflammation of the areas behind the eyes (cavernous sinus and superior orbital fissure). These granulomatous inflammations involve lymphocytes, plasma cells, and multinucleate giant cells. Clinical cases have shown that the disorder consists of the inflammation of multiple cranial nerves, with the highest prevalence of ocular motor nerves. In some cases, it also involves the inflammation of sensory nerves, specifically the trigeminal nerves. == Physiology ==

Tolosa-Hunt Syndrome is highly impacted by the inflammation of the cranial nerves, especially those that are located around the cavernous sinus. These include: • Ocular Motor Nerves • Oculomotor Nerve (Cranial Nerve III) is important for eye coordination and movement. These include saccades, eye tracking, and eye fixations. This impacts 80% of patients. • Abducens Nerve (Cranial Nerve VI) helps move the eye laterally. Involvement of this nerve causes medial (towards the nose) deviation. Patients have been reported to have an impact on this nerve at least 70% of the time. • Trochlear Nerve (Cranial Nerve IV) helps move the eye downward and is impacted in patients 29% of the time. In some cases, inflammation can also impact other cranial nerves. These include: • Sensory Nerves (trigeminal nerves) • Ophthalmic branch of the trigeminal nerve (V1). This nerve is important for the forehead, eye, and upper nose. Seems to be impacted 30% of the time. • Maxillary branch of the trigeminal nerve (V2), which is important for sensing cheeks, upper lip, and upper teeth, is occasionally impacted. • Mandibular branch of the trigeminal nerve (V3), which impacts sensation to the lower part of the face, such as the jaw, part of the teeth, and the ability to chew, is also occasionally affected. A recent study involving a 14-year-old boy showed that the seventh cranial nerve has also been impacted, but not much is known about this one as compared to the other nerves. ==Diagnosis==

Symptoms come from the International Classification of Headache Disorders which was done in 2013. Differential diagnosis Differential diagnoses is as follows: == Treatment ==

Treatment of Tolosa–Hunt syndrome includes immunosuppressives such as corticosteroids (often prednisolone) or steroid-sparing agents (such as methotrexate or azathioprine). as an addition. For patients to be given additional form of treatment, they need to perform follow up check ups. Although there are known medications for patients, there is not much known about the instructions on administering the medications. Most medications given to patients with this disorder are based on other corticosteroids and other steroids, in which patients will be given a high dosage at first, and as it goes on, the dosage is decreased. Most patients will experience pain relief between 24–72 hours after the medications are given, but these times depend on the type of symptoms that the patient is experiencing. ==Prognosis==

Tolosa–Hunt syndrome typically has a good prognosis. Patients usually respond to corticosteroids, and spontaneous remission can occur, although movement of ocular muscles may remain damaged. Roughly 30–40% of patients who are treated for Tolosa–Hunt syndrome experience a relapse. ==Epidemiology==

Tolosa-Hunt syndrome affects all age groups, but most cases have been in adults aged 40–50 years, with the average age being 41 years. Currently, there is about one case per million a year. == History ==

Tolosa-Hunt Syndrome was first recognized in 1954, when Dr. Eduardo Tolosa wrote a case study that involved inflammation of the tissues surrounding the arteries. The diagnostic tools were based on observations, as imaging and other forms of diagnosis were unavailable at the time. The patient he worked with had pain in their eyes and eye muscles. These symptoms were very similar to those of an aneurysm, with additional neurological symptoms. He found that this patient had inflammation on their carotid siphon, which can be found in the cavernous sinus. Later on in 1961, Dr. William E. Hunt and colleagues investigated several cases involving this disorder. Similar to the case that Dr. Eduardo Tolosa was working with, the cases they dealt with involved pain around the eyes. These symptoms would last for days or weeks. Patients would get attacks from months or years at a time, with other neurological deficits. Dr. Smith and Dr. Taxdal coined the name "Tolosa-Hunt Syndrome" after Dr. Hunt and Dr. Tolosa, who examined the very first cases dealing with the disorder in 1966. In their "Painful Ophthalmoplegia - The Tolosa-Hunt Syndrome" paper, they described a total of 5 cases involving this neurological disorder. All cases involved similar symptoms to the original findings. They found out that when patients were given systemic corticosteroids, the symptoms would be reduced. ==References==