Tolosa–Hunt syndrome

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Tolosa–Hunt syndrome
Other namesPainful ophthalmoplegia
Tolosa-hunt ophtalmoplegia.jpg
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 of the left eyelid, exotropia (outward deviation) of the primary gaze of the left eye, and paresis (weakness) of the left third, fourth and sixth cranial nerves.
Specialty Neurology   OOjs UI icon edit-ltr-progressive.svg

Tolosa–Hunt syndrome is a rare disorder characterized by severe and unilateral headaches with orbital pain, along with weakness and paralysis (ophthalmoplegia) of certain eye muscles (extraocular palsies). [1]

Contents

In 2004, the International Headache Society provided a definition of the diagnostic criteria which included granuloma. [2]

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. [3] Symptoms may subside without medical intervention, yet recur without a noticeable pattern. [4]

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). [3] [4]

Causes

The cause of Tolosa–Hunt syndrome 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).[ citation needed ]

Diagnosis

Tolosa–Hunt syndrome 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. [3] These tests include a complete blood count, thyroid function tests and serum protein electrophoresis. [3] Studies of cerebrospinal fluid may also be beneficial in distinguishing between Tolosa–Hunt syndrome and conditions with similar signs and symptoms. [3]

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 inflammatory changes in the cavernous sinus, superior orbital fissure and/or orbital apex. [3] 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.[ citation needed ]Sometimes a biopsy may need to be obtained to confirm the diagnosis, as it is useful in ruling out a neoplasm. [3] Other diagnoses to consider include craniopharyngioma, migraine and meningioma. [3]

Treatment

Treatment of Tolosa–Hunt syndrome includes immunosuppressives such as corticosteroids (often prednisolone) or steroid-sparing agents (such as methotrexate or azathioprine). [3]

Radiotherapy has also been proposed. [5]

Prognosis

The prognosis of Tolosa–Hunt syndrome is usually considered good. Patients usually respond to corticosteroids, and spontaneous remission can occur, although movement of ocular muscles may remain damaged. [3] Roughly 30–40% of patients who are treated for Tolosa–Hunt syndrome experience a relapse. [3]

Epidemiology

Tolosa–Hunt syndrome is uncommon internationally. There is one recorded case in New South Wales, Australia. [3] Both sexes, male and female, are affected equally, and it typically occurs around the age of 60. [1]

Related Research Articles

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Inflammation of the geniculate ganglion of the facial nerve is a late consequence of varicella zoster virus (VZV) known as Ramsay Hunt syndrome (RHS), commonly known as herpes zoster oticus. In regards with the frequency, less than 1% of varicella zoster infections involve the facial nerve and result in RHS. It is traditionally defined as a triad of ipsilateral facial paralysis, otalgia, and vesicles close to the ear and auditory canal. Due to its closeness to the vestibulocochlear nerve, the virus can spread and cause hearing loss, tinnitus, and vertigo. It is common for diagnoses to be overlooked or delayed, which can raise the likelihood of long-term consequences. It is more complicated than Bell's palsy. Therapy aims to shorten its overall length, while also providing pain relief and averting any consequences.

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Bell's palsy is a type of facial paralysis that results in a temporary inability to control the facial muscles on the affected side of the face. In most cases, the weakness is temporary and significantly improves over weeks. Symptoms can vary from mild to severe. They may include muscle twitching, weakness, or total loss of the ability to move one or, in rare cases, both sides of the face. Other symptoms include drooping of the eyelid, a change in taste, and pain around the ear. Typically symptoms come on over 48 hours. Bell's palsy can trigger an increased sensitivity to sound known as hyperacusis.

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The abducens nerve or abducent nerve, also known as the sixth cranial nerve, cranial nerve VI, or simply CN VI, is a cranial nerve in humans and various other animals that controls the movement of the lateral rectus muscle, one of the extraocular muscles responsible for outward gaze. It is a somatic efferent nerve.

<span class="mw-page-title-main">Oculomotor nerve</span> Cranial nerve III, for eye movements

The oculomotor nerve, also known as the third cranial nerve, cranial nerve III, or simply CN III, is a cranial nerve that enters the orbit through the superior orbital fissure and innervates extraocular muscles that enable most movements of the eye and that raise the eyelid. The nerve also contains fibers that innervate the intrinsic eye muscles that enable pupillary constriction and accommodation. The oculomotor nerve is derived from the basal plate of the embryonic midbrain. Cranial nerves IV and VI also participate in control of eye movement.

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<span class="mw-page-title-main">Cavernous sinus thrombosis</span> Medical condition

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References

  1. 1 2 "Tolosa–Hunt syndrome". Who Named It. Retrieved 2008-01-21.
  2. La Mantia L, Curone M, Rapoport AM, Bussone G (2006). "Tolosa–Hunt syndrome: critical literature review based on IHS 2004 criteria". Cephalalgia . 26 (7): 772–781. doi:10.1111/j.1468-2982.2006.01115.x. PMID   16776691. S2CID   31366123.
  3. 1 2 3 4 5 6 7 8 9 10 11 12 Danette C Taylor, DO. "Tolosa–Hunt syndrome". eMedicine. Retrieved 2008-01-21.
  4. 1 2 "Tolosa Hunt Syndrome". National Organization for Rare Disorders, Inc. Retrieved 2008-01-21.
  5. Foubert-Samier A, Sibon I, Maire JP, Tison F (2005). "Long-term cure of Tolosa–Hunt syndrome after low-dose focal radiotherapy". Headache. 45 (4): 389–691. doi:10.1111/j.1526-4610.2005.05077_5.x. PMID   15836581. S2CID   42261396.