Tolosa-Hunt syndrome

Infobox_Disease
Name = Tolosa-Hunt syndrome


Caption =
DiseasesDB = 31164
ICD10 =
ICD9 = ICD9|378.55
ICDO =
OMIM =
MedlinePlus =
eMedicineSubj = neuro
eMedicineTopic = 373
MeshID = D020333

Tolosa-Hunt syndrome (THS) is a rare disorder characterized by severe and unilateral headaches with extraocular palsies, usually involving the third, fourth, fifth, and sixth cranial nerves, and pain around the sides and back of the eye, along with weakness and paralysis (ophthalmoplegia) of certain eye muscles.<cite web |url=http://www.whonamedit.com/synd.cfm/3307.html |title=Tolosa-Hunt syndrome | author= | publisher=Who Named It |accessdate=2008-01-21 |format= |work=]

In 2004, the International Headache Society provided a definition of the diagnostic criteria which included granuloma.cite journal |author=La Mantia L, Curone M, Rapoport AM, Bussone G |title=Tolosa-Hunt syndrome: critical literature review based on IHS 2004 criteria |journal=Cephalalgia |volume=26 |issue=7 |pages=772–81 |year=2006 |pmid=16776691 |doi=10.1111/j.1468-2982.2006.01115.x |url=http://www.blackwell-synergy.com/openurl?genre=article&sid=nlm:pubmed&issn=0333-1024&date=2006&volume=26&issue=7&spage=772]

Causes

The exact cause of THS is not known, but 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).

igns and Symptoms

Symptoms are usually limited to one hemisphere of the brain, and in most cases the individual effected will experience intense, sharp pain and paralysis of muscles around the eye.<cite web |url=http://www.emedicine.com/neuro/topic373.htm |title= Tolosa-Hunt syndrome | author=Danette C Taylor, DO | publisher=eMedicine |accessdate=2008-01-21 |format= |work=] Symptoms may subside without medical intervention, yet recur without a noticeable pattern.cite web |url=http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Tolosa%20Hunt%20Syndrome |title= Tolosa Hunt Syndrome | author= | publisher=National Organization for Rare Disorders, Inc. |accessdate=2008-01-21 |format= |work=]

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).

Diagnosis

THS is usually diagnosed via exclusion, and as such a vast amount of laboratory tests are required to rule out other causes of the patients symptoms. These tests include a complete blood count, thyroid function tests and serum protein electrophoresis. Studies of cerebrospinal fluid may also be beneficial in distinguishing between THS and conditions with similar signs and symptoms.

MRI scans of the brain and orbit with and without contrast, magnetic resonance angiography or digital subtraction angiography and a CT scan of the brain and orbit with and without contrast may all be useful in detecting inflammatory changes in the cavernous sinus, superior orbital fissure and/or orbital apex.

Sometimes a biopsy may need to be obtained to confirm the diagnosis, as it is useful in ruling out a neoplasm.

Differentials to consider when diagnosing THS include Craniopharyngioma, a Migrane and Meningioma.

Treatment

Treatment of THS is usually completed using corticosteroids (often Prednisone) and immunosupressive agents (such as Methotrexate or Azathioprine). Corticosteroids act as analgesia and reduce pain (usually within 24-72 hours), as well as reducing the inflammatory mass, whereas immunosupressive agents help reduce the autoimmune response. Treatment is then continued in the same dosages for a further 7-10 days and then tapered slowly.

Radiotherapy has also been proposed.cite journal |author=Foubert-Samier A, Sibon I, Maire JP, Tison F |title=Long-term cure of Tolosa-Hunt syndrome after low-dose focal radiotherapy |journal=Headache |volume=45 |issue=4 |pages=389–91 |year=2005 |pmid=15836581 |doi=10.1111/j.1526-4610.2005.05077_5.x |url=http://www.blackwell-synergy.com/openurl?genre=article&sid=nlm:pubmed&issn=0017-8748&date=2005&volume=45&issue=4&spage=389]

Prognosis

The prognosis of THS is usually considered good. 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 THS experience a relapse.

Epidemiology

TSA is uncommon in both the United States and internationally. Both genders, male and female, are affected equally, and typically occurs around the age of 60.

References