In neurology, tolosa-hunt syndrome is a special form of sinus cavernosus syndrome characterized by the failure of various cranial nerves. In tolosa Hunt syndrome, paralysis of the ocular musculature is present due to granulomatous inflammation. The prognosis is favorable, but recurrences often occur.
What is Tolosa-Hunt syndrome?
Tolosa-Hunt syndrome is a special form of cavernous sinus syndrome that results in neurologic deficits. The cavernous sinus is a venous blood duct of the brain, with various cranial nerves located in its lateral wall. Accordingly, in sinus cavernosus syndrome, there is a failure of the cranial nerves. Cranial nerves III, IV, VI, V1 and V2 are affected by compressions. The cause of these compressions may be a tumor, as well as septic or aseptic thrombosis. Equally often, fistulas or trauma trigger the syndrome. Tolosa-Hunt syndrome is a final conceivable cause of sinus cavernosus syndrome. Thus, the clinical symptoms of Tolosa-Hunt syndrome largely resemble those of sinus cavernosus syndrome and are causally related to it. Tolosa-Hunt syndrome is a granulomatous inflammatory disease of the cavernous sinus that causes compression of the cranial nerves, resulting in the symptoms of sinus cavernosus syndrome. Eduard Tolosa and William Edward Hunt first described the condition in the 20th century.
Causes
The clinical symptoms of Tolosa-Hunt syndrome are characterized by granulomatous inflammation. This inflammation gives rise to small and nodular cellular collections on the cavernous sinus, also known as granulomas. In granulomatous inflammation, cellular accumulations of monocytes, macrophages, and epithelioid cells or [Langhans giant cells]] are present in the inflamed tissue. Lymphocytes may also be present in the inflamed area. Such inflammations are characteristic, for example, in the context of diseases such as tuberculosis, sarcoidosis, leprosy, or syphilis. They correspond to either small focus epitheloid cell reaction, granulomatous epitheloid cell reaction, mixed cell granulomas or histiocytic granulomas. The etiology of granulomatous inflammation in Tolosa-Hunt syndrome has not yet been determined. It is possible that malignant diseases underlie the syndrome in individual cases. The disease affects almost exclusively adults. With 300 known cases, the syndrome is an extremely rare neurologic eye disease.
Symptoms, complaints, and signs
Tolosa-Hunt syndrome causes sharp pain behind the eye that shoots abruptly into the structures. The inflammation further causes paralysis of the eye muscles. Parts of the oculomotor nerve, as well as parts of the trochlear nerve and abducens nerve, may be affected by the deficits. If the oculomotor nerve is affected, there may be an accommodation disorder of the eye. The upper eyelid usually droops. In contrast, gaze deviation is characteristic of trochlear nerve palsy. The eye rolls outward or deviates vertically. Double vision is characteristic of abducens palsy. The affected eye lags behind the healthy eye when looking to the side. The individual pareses are usually present simultaneously in Tolosa-Hunt syndrome. The consequence is ophthalmoplegia, i.e. a comprehensive paralysis of the external or internal eye muscles. Preorbital pain in the eye is considered an early symptom. Paralysis symptoms do not appear until later. Symptoms often resolve on their own within eight weeks.
Diagnosis and course of the disease
The diagnosis of Tolosa-Hunt syndrome is made by a functional test of the cranial nerves and the neurologic findings collected during the test. Visual assessment also takes place. Laboratory tests are used to determine the inflammatory cause. Malignant diseases must be excluded by imaging diagnostics. Regular monitoring is also useful in the further course of the disease in order to detect any degenerations early enough. The prognosis for Tolosa-Hunt syndrome is considered favorable. Permanent gaze paralysis usually does not occur. The manifestations usually regress quickly. Nevertheless, painful recurrences may occur in the future.
Complications
As a rule, Tolosa-Hunt syndrome leads to severe visual complaints. In the worst case, this can also lead to complete blindness of the affected person.Particularly in young people, blindness can lead to severe psychological complaints or even depression. In particular, the muscles in the eyes become paralyzed in Tolosa-Hunt syndrome, so that the affected person can no longer move or close their eyes. This can also lead to disturbances in the sleep rhythm. The eye itself can also not be held properly and rolls off. Furthermore, there is often pain in the eyes, which can spread to the ears or head. In many cases, the symptoms are not permanent. Furthermore, Tolosa-Hunt syndrome can also lead to spontaneous healing. Treatment of Tolosa-Hunt syndrome is usually done with the help of eye drops and can significantly alleviate the symptoms. This also prevents complete blindness. However, a positive course of the disease cannot be completely predicted. However, the patient’s life expectancy is not negatively affected by the disease.
When should you see a doctor?
As a rule, the affected person with Tolosa Hunt syndrome is dependent on medical treatment to ensure that no further complications or complaints occur. This may also not result in self-healing, so the affected person must always see a doctor. The sooner a doctor is consulted for Tolosa-Hunt syndrome, the better the further course of this disease usually is. A doctor must be consulted in Tolosa-Hunt syndrome if the affected person suffers from sudden eye complaints. As a rule, a drooping eye occurs, which does not disappear on its own. Furthermore, paralysis of the eye muscles can also indicate Tolosa-Hunt syndrome and should also be examined by a doctor. In some cases, the symptoms disappear on their own after a few weeks, although they should still be checked by a doctor. In the case of Tolosa-Hunt syndrome, an ophthalmologist should usually be consulted. The further course depends thereby very strongly on the exact expression of the complaints, so that thereby no general course can be given.
Treatment and therapy
Tolosa-Hunt syndrome is treated symptomatically. Because the cause has not yet been conclusively determined, no causative therapies exist to date. Symptomatic treatment is usually not via eye drops but focuses intravenous drug administration. Patients are administered high doses of corticosteroids. As lipophilic hormones, all corticosteroids act on receptors in the cytosol and nuclei. The active ingredient diffuses freely through the cell membrane to reach the relevant structures. Meanwhile, medicine suspects that corticosteroids also act on membrane receptors. The receptors inside the cell can be divided into two different types. The first type is specific for mineral corticoids. In contrast, the second type responds to glucocorticoids. The specificity of all internal receptors probably depends on 11beta-hydroxysteroid dehydrogenase-1 activity, in which dehydrogenation of the ß-OH group occurs. Usually, the symptoms of Tolosa-Hunt syndrome regress after three to five days of intravenous corticosteroid administration. In isolated cases, ocular muscle dysfunction persists. If this is the case, eye movement therapy can be given in addition to drug therapy. Ideally, cranial nerve palsies can be reversed by specific training. In this way, the cranial nerves may be reactivated or patients may at least learn compensatory strategies that improve their quality of life. If symptoms recur, patients benefit from corticosteroid treatment as early as possible, as this prevents paralysis from recurring at best.
Prevention
The etiology of Tolosa-Hunt syndrome is unknown to date. For this reason, no useful preventive measures to avoid the disease are yet available.
Follow-up
Tolosa-Hunt syndrome is characterized by a marked sensation of pain in the eye as well as paralysis. Occasionally, neurologic symptoms such as dizziness are also present. The affected person often perceives the symptoms as very distressing. The symptoms are triggered by an inflammation in the eye socket. In some cases, they disappear on their own and heal without therapy. Nevertheless, follow-up care is recommended in order to accompany the healing process medically.It aims at the complete cure of the syndrome without late sequelae. The recurrence of the eye disease is to be prevented. Before starting the therapy, a differential diagnosis is made, since different triggers are possible for the symptoms. For clarification, the removal of a tissue sample may be necessary. As part of the aftercare, any spread of the paralysis to areas of the brain must be prevented. Treatment and aftercare is performed by an ophthalmologist. Medication is used to fight the inflammation. The specialist checks the progress of healing, if necessary he varies the dosage or prescribes additional painkillers for the patient. Follow-up care continues until the patient is healed. Even if the patient remains symptom-free, he or she should attend ophthalmological check-ups. In this way, any reappearing symptoms can be detected early.
What you can do yourself
In this condition, self-help measures by no means replace medical therapy, but they can be taken in parallel with treatment as a support. Since the cause of Tolosa Hunt syndrome is unknown, self-therapy focuses on relieving the pain caused by the inflammatory processes in the area behind the eye sockets. Painkillers that also have an anti-inflammatory effect help against this in acute cases: These include ibuprofen, diclofenac and ASA (aspirin). Violent, jerky movements of the head and tension, such as lifting and carrying loads, should be avoided as far as possible so that the inflamed tissue is not further irritated. Even if the eyes themselves are not affected by this disease, reducing the brightness and cooling the forehead, for example with a damp washcloth, can help to better endure the headache. Since rest and quiet are generally also important for the healing process, those affected should therefore lie as still as possible until the medication takes effect and the pain subsides. In the long term, attempts should be made to eliminate factors in everyday life that promote inflammation, for example by changing one’s diet or avoiding stress. This can also help to prevent recurrence of the disease, if possible, or mitigate it as it progresses.