- Tolosa-Hunt syndrome
Name = Tolosa-Hunt syndrome
DiseasesDB = 31164
ICD9 = ICD9|378.55
eMedicineSubj = neuro
eMedicineTopic = 373
MeshID = D020333
Tolosa-Hunt syndrome (THS) is a rare disorder characterized by severe and unilateral
headacheswith 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]
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 sinusand 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).
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, thyroidfunction tests and serum protein electrophoresis. Studies of cerebrospinal fluidmay also be beneficial in distinguishing between THS and conditions with similar signs and symptoms. MRIscans of the brain and orbitwith and without contrast, magnetic resonance angiographyor digital subtraction angiographyand a CT scanof the brain and orbit with and without contrast may all be useful in detecting inflammatorychanges in the cavernous sinus, superior orbital fissure and/or orbital apex.
biopsymay 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 Migraneand Meningioma.
Treatment of THS is usually completed using
corticosteroids(often Prednisone) and immunosupressive agents (such as Methotrexateor Azathioprine). Corticosteroids act as analgesiaand reduce pain (usually within 24-72 hours), as well as reducing the inflammatory mass, whereas immunosupressive agents help reduce the autoimmuneresponse. Treatment is then continued in the same dosages for a further 7-10 days and then tapered slowly. Radiotherapyhas 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]
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
TSA is uncommon in both the
United Statesand internationally. Both genders, male and female, are affected equally, and typically occurs around the age of 60.
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