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| __NOTOC__
| | #REDIRECT[[Tolosa-Hunt syndrome]] |
| {{CMG}} {{AE}} {{JC}}
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| ==Overview==
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| '''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>
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| 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>
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| ==Causes==
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| 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]]).
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| ==Signs and symptoms==
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| 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>
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| 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 (medical)|vertigo]] or [[arthralgia]]. Occasionally the patient may present with a feeling of protrusion of one or both eyeballs ([[exophthalmos]]).<ref name=eMedicine/><ref name=nord/>
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| ==Diagnosis==
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| 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/>
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| [[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]].
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| Sometimes a [[biopsy]] may need to be obtained to confirm the diagnosis, as it is useful in ruling out a [[neoplasm]].<ref name=eMedicine/>
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| Differentials to consider when diagnosing THS include [[craniopharyngioma]], [[migraine]] and [[meningioma]].<ref name=eMedicine/>
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| ==Treatment==
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| 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/>
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| [[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>
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| ==Prognosis==
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| 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/>
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| ==Epidemiology==
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| 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/>
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| == References ==
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| <references/>
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| {{commons category|Tolosa–Hunt syndrome}}
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| {{Headache}}
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| {{DEFAULTSORT:Tolosa-Hunt syndrome}}
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| [[Category:Neurological disorders]]
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| [[Category:Syndromes]]
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| [[Category:Rare diseases]]
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| [[Category:Abducens nerve]]
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