Tolosa Hunt Syndrome

Tolosa Hunt Syndrome

In neurology, the Talosa-Hunt syndrome is a special form of the cavernous sinus syndrome, which is characterized by the failure of various cranial nerves. In Tolosa-Hunt syndrome, there is paralysis of the eye muscles, which is 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 manifests itself in neurological deficits. The cavernous sinus is a venous blood conduit in the brain, in the lateral wall of which lie various cranial nerves. In the case of cavernous sinus syndrome, there is accordingly a failure of the cranial nerves. Cranial nerves III, IV, VI, V1 and V2 are affected by compression. The cause of these compressions can be both a tumor and a septic or aseptic thrombosis. For persistent hyperplastic primary vitreus (phpv) dictionary definitions, please visit foodanddrinkjournal.com.

Fistulas or traumas just as often trigger the syndrome. Tolosa-Hunt syndrome is a final conceivable cause of cavernous sinus syndrome. The clinical symptoms of the Tolosa-Hunt syndrome are largely similar to those of the cavernous sinus syndrome and are causally related. Tolosa-Hunt syndrome is a granulomatous inflammatory disease of the cavernous sinus that causes compression of the cranial nerves, thus producing the symptoms of cavernous sinus 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 collections of cells in the cavernous sinus, also known as granulomas. In granulomatous inflammation, there are cell accumulations of monocytes, macrophages and epitheloid cells or [Langhans giant cells]] in the inflamed tissue.

Lymphocytes can also be found in the inflamed area. Such inflammations are characteristic, for example, in the context of diseases such as tuberculosis, sarcoid, leprosy or syphilis. They correspond either to small-focal epitheloid cell reactions, granulomatous epitheloid cell reactions, mixed-cell granulomas, or histiocytic granulomas.

The etiology of the granulomatous inflammation associated with Tolosa-Hunt syndrome has not yet been elucidated. In individual cases, the syndrome may be due to malignant diseases. The disease affects almost exclusively adults. With 300 known cases, the syndrome is an extremely rare neurological eye disease.

Symptoms, Ailments & Signs

Tolosa-Hunt syndrome causes sharp pain behind the eye that suddenly shoots into the structures. The inflammation also causes paralysis of the eye muscles. Parts of the oculomotor nerve as well as parts of the trochlear nerve and the abducens nerve can be affected by the failures. If the oculomotor nerve is affected, there may be an accommodation disorder of the eye.

The upper eyelid usually hangs down. In contrast, gaze deviation is characteristic of trochlear paresis. The eye rolls outward or deviates vertically. Double vision is characteristic of abduzen paresis. The affected eye lags behind the healthy eye when looking to the side. In Tolosa-Hunt syndrome, the individual pareses are usually present at the same time.

The result is an ophthalmoplegia, i.e. a comprehensive paralysis of the outer or inner eye muscles. The preorbital pain in the eye is considered an early symptom. Symptoms of paralysis appear later. Symptoms often resolve on their own within eight weeks.

Diagnosis & course of disease

The diagnosis of Tolosa-Hunt syndrome is made via a functional test of the cranial nerves and the neurological findings collected. A visual inspection is also carried out. The inflammatory cause is detected by laboratory tests via inflammation diagnostics. Malignant diseases must be excluded by imaging diagnostics.

Regular checks are also useful in the further course in order to detect any degeneration early enough. The prognosis for Tolosa-Hunt syndrome is considered favorable. Permanent gaze paralysis usually does not occur. The symptoms usually resolve quickly. Nevertheless, painful recurrences can occur in the future.

Complications

As a rule, Tolosa-Hunt syndrome leads to severe visual problems. In the worst case, the affected person can become completely blind. Especially in young people, blindness can lead to severe mental health problems or depression. The muscles in the eyes in particular are paralyzed in Tolosa Hunt syndrome, so that those affected can no longer move or close their eyes.

This can also lead to disturbances in the sleep rhythm. The eye itself cannot be held properly and rolls off. In addition, there is often pain in the eyes, which can spread to the ears or head. In many cases, the symptoms are not permanent. Spontaneous healing can also occur in Tolosa-Hunt syndrome.

Tolosa-Hunt syndrome is usually treated with eye drops and can significantly relieve the symptoms. This also prevents complete blindness. However, a positive course of the disease cannot be fully predicted. However, the life expectancy of the patient is not negatively affected by the disease.

When should you go to the doctor?

As a rule, the person affected with Tolosa-Hunt syndrome is dependent on medical treatment so that no further complications or symptoms arise. Self-healing is also not possible, so that the affected person must always consult a doctor. The sooner the doctor is consulted for Tolosa Hunt syndrome, the better the further course of this disease is usually.

A doctor should be consulted for Tolosa Hunt syndrome if the person affected suffers from sudden eye problems. As a rule, this leads to a hanging eye, which does not go away on its own. Paralysis of the eye muscles can also indicate Tolosa Hunt syndrome and should also be examined by a doctor. In some cases, the symptoms subside on their own after a few weeks, although a check-up by a doctor should still be carried out.

In Tolosa-Hunt syndrome, an ophthalmologist should usually be consulted. The further course depends very much on the exact severity of the symptoms, so that no general course can be given.

Treatment & Therapy

Tolosa-Hunt syndrome is treated symptomatically. Since the cause has not yet been finally clarified, there are no causal therapies to date. The symptomatic treatment usually does not take place with eye drops, but focuses on intravenous administration of medication. Patients are given high doses of corticosteroids. As lipophilic hormones, all corticosteroids act on the receptors in the cytosol and cell nuclei.

The active substance diffuses freely through the cell membrane and thus reaches the relevant structures. Medicine now suspects that corticoids also have an effect on membrane-bound receptors. The receptors inside the cell can be divided into two different types. The first type is specific to mineralocorticoids. The second type, on the other hand, reacts to glucocorticoids. The specificity of all internal receptors presumably depends on 11beta-hydroxysteroid dehydrogenase-1 activity, in which dehydration of the ß-OH group takes place.

Usually, symptoms of Tolosa-Hunt syndrome resolve after three to five days of intravenous corticosteroids. In individual cases, eye muscle disorders persist. If this is the case, eye movement therapy can be used in addition to drug therapy. Ideally, the cranial nerve paralysis can be reversed through targeted training.

The cranial nerves may be reactivated in this way, or the patients at least learn compensatory strategies that improve their quality of life. If the symptoms recur, the patients benefit from corticosteroid treatment as early as possible, since this way, at best, there are no signs of paralysis again.

Prevention

The etiology of Tolosa-Hunt syndrome is still unknown. For this reason, no sensible preventive measures to avoid the disease have been available so far.

Aftercare

Tolosa-Hunt syndrome is characterized by a pronounced sensation of pain in the eye and signs of paralysis. Occasionally there are neurological symptoms such as dizziness. Those affected often perceive the symptoms as very distressing. The symptoms are triggered by an inflammation in the eye socket.

In some cases, they regress on their own and heal without treatment. Nevertheless, aftercare is recommended in order to accompany the healing process medically. It aims to completely cure the syndrome without long-term effects. A recurrence of the eye disease should be prevented. Before the start of therapy, a differential diagnosis is made, since different triggers can be considered for the symptoms.

It may be necessary to take a tissue sample for clarification. As part of the follow-up care, the paralysis must be prevented from spreading to areas of the brain. The treatment and aftercare is carried out by an ophthalmologist. The inflammation is treated with medication. The specialist monitors the healing progress, varies the dosage if necessary or prescribes additional painkillers for the patient.

Follow-up care continues until healing occurs. Even if the patient remains symptom-free, he should attend an ophthalmological check-up appointment. In this way, recurring symptoms can be detected at an early stage.

You can do that yourself

With this disease, self-help measures in no way replace medical therapy, but they can be taken in parallel with treatment as support.

Since the cause of Tolosa-Hunt syndrome is unknown, self-treatment focuses on relieving the pain caused by the inflammatory processes in the area behind the orbits. In acute cases, painkillers that also have an anti-inflammatory effect help against these: These include ibuprofen, diclofenac and ASA (aspirin). Violent, jerky movements of the head and tension, for example from lifting and carrying loads, should be avoided as far as possible so that the inflamed tissue is not further irritated.

Although this condition does not affect the eyes themselves, reducing the brightness and cooling the forehead, such as with a damp washcloth, can help make the headache more manageable. Since rest and protection are generally also important for the healing process, those affected should lie as still as possible until the medication takes effect and the pain subsides.

In the long term, an attempt should be made to eliminate pro-inflammatory factors in everyday life, for example by changing your diet or avoiding stress. This can also help to prevent the disease from reoccurring or to mitigate it as it progresses.

Tolosa Hunt Syndrome