Tolosa-Hunt Syndrome

What is Tolosa-Hunt Syndrome?

This is also known as THS and is a syndrome where the cavernous sinus that is located behind the eyes develops inflammation causing severe pain in the eye as well as damage or irritation of the nerves of the face. Females and males are equally affected by this syndrome which normally affects individuals more than 20 years of age. This is a very rare disorder that is distinguished by severe headaches affecting only one side together with extraocular palsies. This normally involves the 3rd, 4th, 5th and 6th cranial nerves as well as pain around the back and sides of the eye along with paralysis and weakness of certain eye muscles.

This syndrome is noticeable as periorbital or hemicranial pain, ophthamoplegia and sensory loss. Several arrangements of lesions of the cranial nerve have been depicted in the syndrome but all localize the site of the lesion to be the “cavernous sinus” or “superior orbital fissure”. It is a condition that reacts well to therapy with steroids but which can reoccur months or even years after the first attack in up to 50% of the cases. The etiology is not known although it shares histopathogical features with idiopathic orbital pseudotumor.

It is an atypical situation and the majority of the medical literature reports cases rather than series of cases. THS cases have been certificated globally and at every ages. Females and male appear to be affected equally. The age division is consistent apart from being rare before age 20 years of age. There are just a rare few pediatric cases reported in the literature.

Symptoms

Symptoms include some of the following:

  • Affect only one side of the head – unilateral
  • Muscles around the eye paralysis
  • Intense sharp pain
  • Spontaneous remission
  • Recur without distinct pattern
  • May exhibit paralysis or palsy of certain facial nerves
  • Upper eyelid drooping – ptosis
  • Spinning sensation – vertigo
  • Joint pain – arthralgia
  • Vision that is double
  • Atypical protrusion of one or both eyeballs – exophthalmos
  • Fever
  • Chronic fatigue

Causes

The precise cause of THS is not known, but the syndrome is believed to be linked with the areas behind the eyes being inflamed. There are some conditions that have similar features or diseases linked with this syndrome and their subsequent causes:

Headaches

Headaches that are unilateral with pain on only one side of the head and this is often caused by temporal arteritis and migraine.

Ophthalmoplegia

Ophthalmoplegia is a symptom that is characterized by eye muscles paralysis. This is usually caused by a brain tumor, brain injury, meningitis, multiple sclerosis, diabetes, and trauma.

Extraocular palsies

Extraocular palsies are problems of the extraocular motor nerve palsie where each of these nerves is accountable for each precise muscle. Reasons consist of diabetic neuropathy, head trauma, brain tumor, multiple sclerosis, aneurysm and cerebrovascular accident.

Diplopia

Diplopia is a indication caused by graves’ ophthalmopathy, multiple sclerosis, botulism, myasthenia gravis, tumor or blood clot behind the eyes, injury to the head, swelling of eye muscle, damage to nerve muscles providing eye control, and much more.

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Chronic fatigue

Chronic fatigue is a circumstance that is a form of fatigue that is severe and is not lessened by simply resting and the major cause is still not known but it is believed it is due too an infection.

Exophthalamos

Exophthalamos is a protrusion of one or both eyes. Possible causes include blood clot of the eye, eye tumor, aneurysem, sinusitis, tumor, retracted eyelid, inflammatory disease and much more.

Arthralgia

Arthralgia is stabbing and sharp pains in the joint. Causes by an injury, arthritis, infection as well as other ailments.

Vertigo

Vertigo is a disorder with the feeling of spinning or floating even when being still. Causes comprise side effect from medications, infection, disorders and injuries.

Treatment

The “International Headache Society” criteria for THS is:

  • Eye pain that is one-sided for 8 weeks on average when left untreated
  • Combined damage or irritation to the 3rd, 4th or 6th cranial nerves
  • Pain is reduced within 48 hours of beginning to take steroid medication.
  • Other disorders have been eliminated thru testing.

Often, the last condition is the more important, since many disorders may cause signs and symptoms comparable to THS. MRI or magnetic resonance imaging, angiography or CT computed tornography can aid to ascertain if something else, such as a tumor, is the cause of the eye pain. If THS is present the inflammation of the sinus cavity can in most cases be seen in these tests as well.

Fortunately, THS is not a disorder that is fatal and may be managed with steroid drugs for instance prednisone. This normally offers relief from pain within 24 to 72 hours of beginning to start taking the drugs. The problems with vision as well as numbness of the forehead can take weeks to months for resolution and often the symptoms may never go totally away.

Often as many as 30% to 40% of individuals might experience a recurrence or relapse of THS normally on the same side. Since numerous conditions might have comparable symptoms, individuals need to report any symptoms that are new or side effects from treatment to their physicians.

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