what is toulouse hunt syndrome?

Answer 1

Tolosa-Hunt syndrome describes a rare , granulomatous inflammatory process adjacent to the cavernous sinus or within the superior orbital fissure and is a diagnosis of exclusion. The syndrome manifests as hemicranial or periorbital pain, ophthalmoplegia and sensory loss. Several combinations of cranial nerves have been described as being involved in the syndrome, but all localise the site of the lesion to the cavernous sinus or superior orbital fissure. It is a syndrome which responds readily to treatment with steroids, but may reoccur months or years after the initial attack in approximately 50% of cases.1

Epidemiology. The precise incidence of Tolosa-Hunt syndrome is not known as most of the published literature reflects individual case reports. Cases of Tolosa-Hunt syndrome have been documented worldwide and at all ages. Males and females appear to be equally affected. The aetiology is as yet unclear.

Presentation. Hunt's original description describes the following features as being necessary in order to make the diagnosis:- 2

Persistent hemicranial or periorbital "gnawing" pain, which may occur before or after other symptoms and signs.
Cranial nerve involvement which may affect any, or all of, the 3rd, 4th,5th (1st division) and 6th cranial nerves. The optic nerve and periarterial sympathetic nerves may also be involved.
Symptoms may last any length of time from days to weeks and may reoccur at intervals of months to years.
If untreated, spontaneous remissions may occur, although may be associated with some residual neurological deficit.
There is no evidence of other pathology.

Since this original description other features have been added by subsequent case reports and presentations of the syndrome have been known to include:-
Pain: peri-orbital, retro-orbital, frontal or temporal. Described as severe,"gnawing" or"stabbing" in nature.
Pupillary reactions: normal, or with sympathetic (Horner's syndrome) or parasympathetic (oculomotor nerve) involvement.
Optic disc: normal, pale or swollen.
Visual acuity: normal or impaired. Rarely,loss of acuity may be permanent.
Cranial nerves: occassionally the maxillary and mandibular branch of the trigeminal nerve may also become involved.
Nausea and vomiting.
Differential Diagnosis. Several other conditions may present in a similar manner to Tolosa-Hunt syndrome including :-
Trauma.
Vascular lesions such as cavernous artery aneurysm or thrombosis.
Tumours, such as pituitary adenoma,meningioma, giant cell tumour,metastases.
Infection e.g. sinusitis, herpes zoster, TB.
Sarcoidosis.
Wegener's granulomatosis.
Eosinophilic granuloma.
Diabetic ophthalmoplegia.
Migraine.
Investigations. The diagnosis of Tolosa-Hunt syndrome is a diagnosis of exclusion. Investigations primarily are aimed at ruling out other causes of signs and symptoms. Tolosa-Hunt syndrome is suspected,investigations which are likely to be performed may include:-3

FBC
U+E's, blood glucose, liver function tests.
C reactive protein
HbA1C
Syphilis serology
Anti-nuclear antibody, anti- double stranded DNA and anti smooth muscle antibodies.
Serum protein electrophoresis.
Lumbar puncture and examination of CSF.
MRI/CT scan
Biopsy
Management. The treatment of Tolosa-Hunt syndrome is oral cortico-steroids to which there is usually a dramatic response within 72 hours.4 Before the advent of CT/MRI scanning, the response to steroids was used as a diagnostic test.

Prognosis. Tolosa-Hunt syndrome usually responds rapidly to treatment with oral steroids, although recurrences may occur months or years later in up to 50% of patients. Rarely patients may have residual cranial nerve damage. As Tolosa-Hunt syndrome is a diagnosis of exclusion, it is recommended that patients with a diagnosis of Tolosa-Hunt must be re-investigated at intervals for at least 2 years, even if the initial MRI scans were negative, to prevent the possibility of more sinister pathology being missed.

Answer 2

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Answer 3

Tolosa-Hunt Syndrome

Individuals with Tolosa-Hunt syndrome experience severe pain behind or around one eye that begins suddenly, followed by double vision and numbness of the forehead. Tolosa-Hunt syndrome can be relieved by steroid medication, but vision problems may persist. (http://rarediseases.about.com/b/a/119478.htm?terms=severe+eye+pain)

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Answer 4

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Answer 5

2

Answer 6

1

Answer 7

. . . unfortunately it's the diagnosis instigated by Toulouse Lautrec through his life-long hunt for longer legs.

(if you didn't already know he was a short-arsed, French, crap, supposedly "modern" artist who finally succumbed to either gonorrhoea or syphilis) . . .

Answer 8

It is a special kind of inflammation behind the eye, which causes pain and difficulty moving the eyes.

Answer 9

when people have a sudden and desperate desire to go to toulose, they leave without any travel plans and spend their lives hunting for toulose, usually end up in somewhere else, lol.

Answer 10

Why don't you google it and find out?