|
|
| Line 1: |
Line 1: |
| __NOTOC__
| |
| {{CMG}} {{AE}} {{JC}}
| |
| {{Infobox disease |
| |
| Name = Tolosa–Hunt syndrome |
| |
| Image =Tolosa-hunt_ophtalmoplegia.jpg|
| |
| Caption = Neuro-ophthalmologic examination showing [[ophthalmoplegia]] in a patient with '''Tolosa–Hunt syndrome''', prior to treatment. The central image represents forward gaze, and each image around it represents gaze in that direction (for example, in the upper left image, the patient looks up and right; the left eye is unable to accomplish this movement). The examination shows [[Ptosis (eyelid)|ptosis]] of the left eyelid, [[exotropia]] (outward deviation) of the primary gaze of the left eye, and [[paresis]] (weakness) of the left [[Oculomotor nerve|third]], [[Trochlear nerve|fourth]] and [[Abducens nerve|sixth]] [[cranial nerves]].|
| |
| }}
| |
|
| |
|
| ==Overview==
| |
| '''Tolosa–Hunt syndrome''' ('''THS''') is a [[rare disease|rare disorder]] characterized by severe and unilateral [[headaches]] with extraocular [[:wikt:palsy|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.<ref name=wni>{{cite web |url=http://www.whonamedit.com/synd.cfm/3307.html |title=Tolosa–Hunt syndrome | author= | publisher=Who Named It |accessdate=2008-01-21 |work=}}</ref>
| |
|
| |
| In 2004, the International Headache Society provided a definition of the diagnostic criteria which included [[granuloma]].<ref name="pmid16776691">{{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 (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}}</ref>
| |
|
| |
| ==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]]).
| |
|
| |
| ==Signs and symptoms==
| |
| Symptoms are usually limited to one side of the head, and in most cases the individual affected will experience intense, sharp pain and paralysis of muscles around the eye.<ref name=eMedicine><{{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 |work=}}</ref> Symptoms may subside without medical intervention, yet recur without a noticeable pattern.<ref name=nord>{{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=}}</ref>
| |
|
| |
| In addition, affected individuals may experience paralysis of various facial nerves and drooping of the upper eyelid ([[ptosis (eyelid)|ptosis]]). Other signs include [[Diplopia|double vision]], [[fever]], chronic [[fatigue (medical)|fatigue]], [[vertigo]] or [[arthralgia]]. Occasionally the patient may present with a feeling of protrusion of one or botheyeballs ([[exophthalmos]]).<ref name=eMedicine/><ref name=nord/>
| |
|
| |
| ==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 patient's symptoms.<ref name=eMedicine/> These tests include a [[complete blood count]], [[thyroid]] function tests and [[serum protein electrophoresis]].<ref name=eMedicine/> Studies of [[cerebrospinal fluid]] may also be beneficial in distinguishing between THS and conditions with similar signs and symptoms.<ref name=eMedicine/>
| |
|
| |
| [[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 [[inflammation|inflammatory]] changes in the cavernous sinus, superior orbital fissure and/or orbital apex.<ref name=eMedicine/> Inflammatory change of the orbit on cross sectional imaging in the absence of cranial nerve palsy is described by the more benign and general nomenclature of [[orbital pseudotumor]].
| |
|
| |
| Sometimes a [[biopsy]] may need to be obtained to confirm the diagnosis, as it is useful in ruling out a [[neoplasm]].<ref name=eMedicine/>
| |
|
| |
| Differentials to consider when diagnosing THS include [[craniopharyngioma]], [[migraine]] and [[meningioma]].<ref name=eMedicine/>
| |
|
| |
| ==Treatment==
| |
| Treatment of THS is usually completed using [[corticosteroids]] (often [[prednisolone]]) and immunosuppressive agents (such as [[methotrexate]] or [[azathioprine]]).<ref name=eMedicine/> Corticosteroids act as [[analgesia]] and reduce pain (usually within 24–72 hours), as well as reducing the inflammatory mass, whereas immunosuppressive agents help reduce the [[autoimmune]] response.<ref name=eMedicine/> Treatment is then continued in the same dosages for a further 7–10 days and then tapered slowly.<ref name=eMedicine/>
| |
|
| |
| [[Radiotherapy]] has also been proposed.<ref name="pmid15836581">{{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}}</ref>
| |
|
| |
| ==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.<ref name=eMedicine/> Roughly 30–40% of patients who are treated for THS experience a [[relapse]].<ref name=eMedicine/>
| |
|
| |
| ==Epidemiology==
| |
| THS is uncommon in both the [[United States]] and internationally. In New Zealand, there is only one recorded case .<ref name=eMedicine/> Both genders, male and female, are affected equally, and it typically occurs around the age of 60.<ref name=wni/>
| |
|
| |
| ==References==
| |
| {{reflist|2}}
| |
|
| |
| [[Category:Neurological disorders]]
| |
| [[Category:Syndromes]]
| |
| [[Category:Rare diseases]]
| |
| [[Category:Abducens nerve]]
| |