Tolosa-Hunt syndrome (THS) is an idiopathic inflammatory condition that involves the cavernous sinus and orbital apex and is essentially a clinical diagnosis of exclusion.
The Tolosa-Hunt syndrome is a unilateral headache involving one orbit associated with ophthalmoplegia, which develops several days later.
Other Names for Tolosa-Hunt syndrome (THS)
- ophthalmoplegia, painful
- ophthalmoplegia, recurrent
- ophthalmoplegia syndrome
Unilateral ophthalmoplegia associated with pain behind the orbit and in the area supplied by the first division of the trigeminal nerve; it is thought to be due to nonspecific inflammation and granulation tissue in the superior orbital fissure or cavernous sinus.
Affected individuals may exhibit signs of paralysis (palsy) of certain cranial nerves such as drooping of the upper eyelid (ptosis), double vision (diplopia), large pupil, and facial numbness. The affected eye often abnormally protrudes (proptosis). 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).
Signs and Symptoms of Tolosa-Hunt syndrome (THS)
Many individuals with Tolosa-Hunt syndrome experience the sudden onset of severe periorbital headache, followed by painful and decreased eye movements (ophthalmoplegia). In some cases of severe ophthalmoplegia, the eye itself is unable to move or look in various directions (frozen globe).
The major symptoms of Tolosa-Hunt syndrome include chronic periorbital headache, double vision, paralysis (palsy) of certain cranial nerves, and chronic fatigue. Affected individuals may also exhibit protrusion of the eye (proptosis), be drooping of the upper eyelid (ptosis) and diminished vision. 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).
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. Symptoms may subside without medical intervention, yet recur without a noticeable pattern.
In addition, affected individuals may experience
- paralysis of various facial nerves
- drooping of the upper eyelid (ptosis).
- Other signs include double vision,
- chronic fatigue,
- vertigo or arthralgia.
- Occasionally the patient may present with a feeling of protrusion of one or both eyeballs (exophthalmos).
Clinical Features of Tolosa-Hunt syndrome (THS)
There is a severe headache involving the orbit on the affected side associated with ophthalmoplegia that develops several days after the onset of a headache. The ophthalmoplegia usually presents with a third nerve paralysis followed by involvement of the fourth, sixth, and ophthalmic division of the fifth cranial nerve. Horner syndrome due to the involvement of the carotid sympathetic plexus is not unusual. Optic neuritis has been described in some cases. The condition typically persists for several days but eventually remits spontaneously in most cases.
Differential diagnosis of Tolosa-Hunt syndrome (THS)
The following Conditions express same signs and symptoms of Tolosa-Hunt syndrome (THS). we can differentiate Tolosa-Hunt syndrome (THS) with these :
- Orbital cellulitis
- Cavernous sinus thrombosis
- Migraine headaches
- Inflammatory myofibroblastic tumor (IMT)
- Idiopathic hypertrophic patchy-meningitis (IHP)
Consider other pathological processes presenting with similar clinical features such as:
- pituitary tumors
- tuberculous meningitis
Diagnosis of Tolosa-Hunt syndrome (THS)
The diagnosis of Tolosa-Hunt syndrome is suspected based upon the presence of characteristic physical features
The diagnosis may be confirmed by a thorough clinical evaluation
- detailed patient history
- variety of specialized radiologic tests including computed tomography (CT) scan
- magnetic resonance imaging (MRI).
These examinations may reveal characteristic enlargement or inflammation of the areas behind the eye (cavernous sinus and superior orbital fissure).
Clinically it refers to the presence of a painful ophthalmoplegia secondary to surrounding cavernous sinus inflammation. Tolosa-Hunt syndrome is essentially a clinical diagnosis of exclusion.
Pathology of Tolosa-Hunt syndrome (THS)
The constant pain that characterizes the disorder is due to infiltration of lymphocytes and plasma cells, along with thickening of dura mater within the cavernous sinus.
Radiographic features of Tolosa-Hunt syndrome (THS)
CT Scan results in case of Tolosa-Hunt syndrome (THS)
May show asymmetrical enlargement in the region of the cavernous sinus on the affected side +/- contrast enhancement.
The secondary criteria are internal carotid artery narrowing, extension towards the superior orbital fissure and orbital apex.
MRI results in case of Tolosa-Hunt syndrome (THS)
May show evidence of inflammatory changes in the region of the anterior cavernous sinus, superior orbital fissure +/- orbital apex. Signal characteristics are nonspecific (clinical scenario essential to diagnosis) but may include:
- T1: involved region is isointense to hyperintense compared with muscle
- T2: involved area is hyperintense
- T1 C+ (Gd): may show contrast enhancement during active phase with resolution of enhancement following treatment
Sometimes a biopsy may need to be obtained to confirm the diagnosis, as it is useful in ruling out a neoplasm.
Differentials to consider when diagnosing THS include craniopharyngioma, migraine, and meningioma.
What are the Causes of Tolosa-Hunt syndrome (THS)
While the exact cause of Tolosa-Hunt syndrome is unknown, one theory is an abnormal autoimmune response linked with an inflammation in a specific area behind the eye (cavernous sinus and superior orbital fissure). In some cases, inflammation may be due to a clumping of a certain type of cell (granulomatous inflammation).
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).
Autoimmune disorders are caused when the body’s natural defenses against “foreign” or invading organisms (e.g., antibodies) begin to attack healthy tissue for unknown reasons. Other possible causes may include generalized inflammation and constricted or inflamed cranial blood vessels.
Treatment of Tolosa-Hunt syndrome (THS)
In most cases, the pain associated with Tolosa-Hunt syndrome subsides with short-term use of steroid drugs. Pain is usually reduced in untreated cases within fifteen to twenty days. With steroid treatment, pain typically briskly subsides within twenty-four to seventy-two hours – and this brisk steroid response aids in the diagnosis. Affected individuals may be vulnerable to recurrent future attacks.
The condition is often successfully amenable to steroid treatment.
Treatment of THS includes immunosuppressives such as
- corticosteroids (prednisolone)
- steroid-sparing agents (such as methotrexate or azathioprine).
- Radiotherapy has also been proposed.
The Tolosa-Hunt syndrome shows a good response to corticosteroids. Methylprednisolone 80 mg daily produces rapid resolution of symptoms, and the dose can be progressively reduced as response occurs.
Prognosis of Tolosa-Hunt syndrome (THS)
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. Roughly 30–40% of patients who are treated for THS experience a relapse