Tholos-Hunt Syndrome: Diagnosis and Treatment

Despite the high level of development of medicine, some pathologies remain unexplored until the end. To one of these diseases is the syndrome of Tholos-Hunt. Currently, only the symptoms of this pathology are known, while the causes are not yet fully disclosed. Most likely, this is due to the fact that the disease is very rare and open not so long ago. In addition, the Tholos-Hunt syndrome is often "masked" for other pathologies, and it is easy to confuse. The main symptom is the damage to the eyes and cranial nerves. It is known that one of the properties of this disease is that it lends itself to hormonal therapy, after which there is a rapid improvement. Nevertheless, relapse can occur at any time.

The description of the Tholos-Hunt syndrome

The first mention of this disease appeared relatively recently. It was described in 1961 by the English ophthalmologist Hunt. In addition to the main name, pathology is also called in another way, for example, "chameleon". This is due to the fact that her symptoms resemble many other ailments. Among them: head tumors, infectious encephalitis, myositis orbit and even diseases of the hematopoietic system. In addition, the pathology of Tholos-Hunt is called a symptom of the upper globular fissure, which does not accurately reflect the essence of the problem. The clinical picture of this disease depends on which nerves of the brain were affected. Most often, the syndrome of Tholos-Hunt is characterized by pain in the area of the orbit, the inability to move the sight, by diplopia. With timely begun hormonal therapy, a complete cure or achievement of a stable remission is possible.

The etiology of the Tholos-Hunt syndrome

Due to the fact that the pathology has various manifestations and begins suddenly, it has not yet been possible to establish the exact etiology. This is also due to the low incidence of the syndrome. For this reason, doctors do not have the opportunity to properly study this disease. There are several assumptions according to which pathology can develop. There are the following etiological factors that are available in patients with the Tolosa-Hunt syndrome:

1. Malformation. This term implies an abnormal development of the vascular system of the eyes. As a result of malformation, there is mixing of arterial and venous blood, which should not be normal. This violation is more common among the female population.
2. Autoimmune aggression. This factor is the trigger mechanism of many diseases. Nevertheless, to answer the question why the immune cells begin to destroy the tissues of the body, it is not possible. In most cases, "aggression" occurs after the transferred stresses and long-term ongoing infectious processes.
3. Various neoplasms of the brain and cranial nerves. It can be both benign tumors, and cancerous.

Mechanism of the development of the syndrome

Considering the fact that the etiology of the disease remains unknown, pathogenesis can not be fully studied either. Regardless of the reasons for the development of pathology, only certain structures of the eye and nervous tissue are affected. As is known, the second name of the disease is the syndrome of the upper globular fissure. This is due to pathogenesis, since the nerves and vessels that pass through it are affected. First of all, there are changes in the ophthalmic vein and artery. As a result, trophic tissue is disrupted. In addition, the cavernous sinus is damaged, which adjoins the upper orbital gap. Arteries, blood supplying it, become inflamed, and the tissue is hypertrophic. As is known, the following cranial nerves pass through the cleft : block, drain, part of the trigeminal and oculomotor nerves . In some cases all these pairs are affected. At the same time, the ability to rotate the eyeball suffers. Sometimes the function of one or two pairs of nerves is disrupted.

Tholos-Hunt syndrome: symptoms of the disease

Pathology most often makes itself felt in the elderly and senile age. This syndrome can be affected by both women and men. The clinical picture of the disease develops suddenly, without any prerequisites. Allocate the following symptoms:

1. Pain in the orbital region. Unpleasant feelings first appear in the forehead, superciliary arches, head. Later, the intensity of pain increases, it spreads to the eyes.
2. Diplopia. This symptom appears after the development of pain syndrome. It seems to the patient that all the objects he looks at are bifurcated. Concentrating the view is difficult.
3. Violation of the mobility of the eyeball - ophthalmoplegia. More often observed on one side. Its degree depends on the intensity and number of affected nerves.
4. Edema of the conjunctiva.
5. Exophthalmos. It is observed in the absence of treatment and frequent relapses.
6. Strabismus. It occurs when the nerves are affected only on one side.
7. Subfebrile temperature and deterioration.

These symptoms usually increase gradually and replace each other. In some cases, they are all observed simultaneously. The signs of the disease can suddenly disappear, just as they appeared. Nevertheless, without treatment, pathology always makes itself felt again. The frequency of relapses is different for all, as well as the duration of remission.

Tholos-Hunt syndrome: diagnosis of pathology

To reveal this pathology is not easy, since its symptoms are similar to many other diseases. Therefore, the Tholos-Hunt syndrome is considered a diagnosis of exclusion. First of all, the patient is asked about the features of the course of the disease (from which it began, how the symptoms developed). After that, laboratory studies and instrumental diagnostics are carried out. It is very important to conduct an ophthalmologic and neurological examination. To exclude tumors of the brain, an MRI is performed. It is also important to conduct ultrasound and X-ray examination of the eyeballs. The presence of vascular malformation is determined by angiography. In addition, one of the diagnostic criteria is the effectiveness of hormone therapy. If in the laboratory and instrumental examinations there are no pronounced changes, and the symptoms quickly stop after treatment, then, most likely, the diagnosis is the syndrome of Tholos-Hunt. Prednisolone is used for treatment most often.

Differential diagnosis with other diseases

This disease is compared with other pathologies of the eyes and nervous system. First of all, it is necessary to exclude the inflammatory processes of the brain and its membranes, as well as tumors. When suspected of meningitis or encephalitis, spinal puncture is performed. In order to exclude benign tumors and cancer, the patient undergoes MRI, CT of the brain and X-ray of the skull. The disease is differentiated with lymphomas, a cavernous sinus cyst and its thrombosis. Also, similar symptoms can be observed in system pathologies, such as sarcoidosis, myositis, migraine, etc. To make an accurate diagnosis, the patient should be examined by various specialists: ophthalmologist, neuropathologist, vascular surgeon, endocrinologist.

Tholos-Hunt syndrome treatment

Treatment of the syndrome of Tholos-Hunt is the administration of drugs that suppress the immune system. To do this, hormone-containing medications are used: "Prednisolone", "Hydrocortisone". These drugs have an effect with other autoimmune pathologies, but with this disease the symptoms are already in 3-4 days. Treatment of the Tholos-Hunt syndrome with prednisolone is carried out at the rate of 1-2 mg / kg of body weight. If the drug is used in the form of a solution, then it is prescribed from 500 to 1000 mg per day. In addition, anesthetics and vitamins are used.

Tolos-Hunt syndrome

Usually with this diagnosis, the patients' work capacity is preserved. It is necessary to be sure that the patient has the Tolos-Hunt syndrome. Disability in this pathology is rare. However, with frequent relapses, 3 groups can be appointed. The patient should be transferred to easy work that does not require visual efforts. Also, with persistent nerve damage, the patient can not drive the vehicle due to a violation of the movement of eyeballs and diplopia.

Tholos-Hunt syndrome prophylaxis

It is impossible to predict the development of the Tholos-Hunt syndrome in advance, so there is no primary prevention. If symptoms such as pain in the eyes and forehead, diplopia are present, then a thorough diagnosis should be carried out. In the syndrome of Tholos-Hunt, secondary prevention is necessary. It includes timely hormonal therapy, support for immunity. It is also necessary to avoid stressful situations and inflammatory processes.