Tolosa-Hunt Syndrome

Tolosa-Hunt Syndrome

Introduction

The Tolosa-Hunt syndrome is a rare syndrome with an estimated annual incidence of one case per million per year. It is characterized by painful ophthalmoplegia) and is caused by an idiopathic granulomatous inflammation of the cavernous sinus. This syndrome was first described in 1954, and its exquisite responsiveness to glucocorticoid treatment was recognized a few years later

Definition

It's a rare disorder, describes episodic orbital pain associated with paralysis of one or more of the third, fourth and/or sixth cranial nerves.

Causes or etiology

The exact cause of Tolosa-Hunt syndrome is not known, but the disorder is thought to be associated with inflammation of specific areas behind the eye (cavernous sinus and superior orbital fissure).

The inflammation produces pressure and secondary dysfunction of the structures within the cavernous sinus, including cranial nerves III, IV, and VI, as well as the superior divisions of cranial nerve V.

History (Not necessary but in case)

First classified by International Headache Society in 2004 and now is a part of Classification ICHD – II. ICD-10 for Tolosa Hunt Syndrome is G44.850

Signs & Symptoms

• Chronic periorbital headache
• Decreased eye movements (ophthalmoplegia)
• Double vision
• Paralysis (palsy) of certain cranial nerves
• Chronic fatigue
• Protrusion of the aye (proptosis)
• Drooping of the upper eyelid (ptosis)
• Diminished vision
• All three ocular motor cranial nerves may be involved, in various combinations.
• Pupillary reactions may be normal, or there may be either parasympathetic (oculomotor nerve) or sympathetic (third order neuron Horner's syndrome) involvement

In most cases, symptoms associated with Tolosa-Hunt syndrome affect only one side (unilateral).

Symptoms will usually subside without intervention (spontaneous remission) and may recur without a distinct pattern (randomly).

Diagnosis

The following criteria must be met for a definitive diagnosis of this disorder:

• Eye pain on one side of the head that persist for at the least eight weeks if untreated
• Associated irritation or damage to the third, fourth or sixth cranial nerves
• Relief of pain within 48 hours upon the administration of steroids
• Specialized testing that rules out other conditions such as neoplasm, infection or aneurysm

The diagnosis may be confirmed by a thorough clinical evaluation, detailed patient history, and a variety of specialized radiologic tests (computed tomography (CT) scan, and magnetic resonance imaging (MRI). These examinations may reveal characteristic enlargement or inflammation of the areas behind the eye (cavernous sinus and superior orbital fissure).

Treatment

In most cases the pain with Tolosa Hunt syndrome relieves with the short term use of steroids. The pain is usually gotten rid of easily, but the outbreaks are more than likely to be recurring.

The best Corticosteroids are the treatment of choice for Tolosa-Hunt syndrome (THS), usually providing significant pain relief within 24–72 hours of therapy initiation.

Oral Prednisone 1 mg/kg/day Azathioprine and Methotrexate can be beneficial if no response to steroid

Prognosis

Symptom improvement, especially pain relief, is usually seen 24 to 72 hours after starting steroids, with the majority of patients reporting improvement within one week. Cranial nerve palsies improve gradually and can take anywhere from two to eight weeks for recovery. It is unusual to have residual deficits after steroid treatment.

Relapses tend to occur in about 40% to 50% of patients and can be ipsilateral, contralateral, or bilateral. Relapses are more common in younger patients than in older patients. It is unclear as to whether steroids help prevent relapses.

Síndrome de Tolosa-Hunt

Introducción

El síndrome de Tolosa-Hunt es un síndrome raro con una incidencia anual estimada de un caso por millón al año. Se caracteriza por oftalmoplejía dolorosa y es causada por una inflamación granulomatosa idiopática del seno cavernoso. Este síndrome se describió por primera vez en 1954 y unos años más tarde se reconoció su exquisita respuesta al tratamiento con glucocorticoides.

Definición

Es un trastorno raro, describe dolor orbitario episódico asociado con parálisis de uno o más de los nervios craneales tercero, cuarto y/o sexto.

Causas o etiología

Se desconoce la causa exacta del síndrome de Tolosa-Hunt, pero se cree que el trastorno está asociado con la inflamación de áreas específicas detrás del ojo (seno cavernoso y fisura orbitaria superior).

La inflamación produce presión y disfunción secundaria de las estructuras dentro del seno cavernoso, incluidos los nervios craneales III, IV y VI, así como las divisiones superiores del nervio craneal V.

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