What is the Tolosa-Hunt syndrome? Causes, symptoms, treatment

The syndrome of the Tholos-Hunt syndrome (upper eye socket syndrome, painful ophthalmoplegia, periarteritis of the carotid siphon) is a fairly rare disease, which combines a pronounced pain syndrome in the peri-ocular area and orbit with the dysfunction of one or more cranial nerves.

Causes of the disease

The reasons for this state are not fully understood and debatable (discussed by scientists). There is a fairly large number of conditions that can mimic the Tolosa-Hunt syndrome (orbital myositis, infectious inflammation the external wall of the cavernous sinus, systemic diseases, brain tumors and orbits, vascular transformations, etc.). Proceeding from this, such a diagnosis should be a "diagnosis of exclusion" (that is, it is set by excluding other possible diseases).

Equally, this disease affects men and women, a high incidence is noted in the elderly and senile age. Clinical manifestations appear rapidly, acutely, without previous symptoms or gradually increasing, after a viral infection that can go unnoticed, after hypothermia, stress.

Often a provoking factor is not found.

Clinical signs

In some patients, in addition to pain, exophthalmos and edema of the conjunctiva can be observed.

The disease, as a rule, begins precisely with severe pain in the area behind the eyeball, in the temporal, frontal, superciliary area. After a few days (usually no later than 14 days, less often at the same time), double vision in the eyes, limitation of mobility of the eyeball and strabismus on the side of pain are added. Approximately 1/4 of patients with all the nerves that pass through the upper orbital gap develop a complete impairment of movements of the eyeball in all leads.

More often there are "incomplete" forms of the Tolosa-Hunt syndrome, in which the branches of the oculomotor, trigeminal, optic nerves are involved in the pathological process in various combinations. In some patients exophthalmos and edema of the conjunctiva of the eyeball are observed. The average duration of pain is about two months. Also, in isolated cases, there may be an increase in body temperature to subfebrile values, nonspecific changes in the clinical analysis of blood.

Diagnostics

With what does this disease have to be differentiated? The causes of the development of similar symptoms are huge: tumors of the middle cranial fossa, pterygoid, pituitary gland, parasellar tumors, retrobulbar volumetric processes, cavernous sinus tumors, cavernous sinus thrombosis, carotid artery aneurysm, periostitis, osteomyelitis, leukemia infiltration in the region of the upper globular fissure.

Do not forget about such diseases as myasthenia gravis, diabetes, thyroid pathology, temporal arteritis, meningitis, multiple sclerosis, migraine with aura. All of the above conditions can lead to ophthalmoplegia and impairment of the functions of the cranial nerves. That is why a patient with syndromes of movement of the eyeball and pain in one half of the face requires close attention. The research should be comprehensive and multifaceted. Consultation of the oculist for examination of fields and visual acuity, eye day is necessary. The neurologist should carefully collect the anamnesis, conduct a full clinical examination.

Of the additional methods of research should be noted neurovisualization methods (CT and MRI of the brain and Turkish saddle), angiography, echography of orbits.

According to modern criteria for diagnosing, the Tolosa-Hunt syndrome can be set only in one case: when magnetic resonance imaging (MRI) of the brain revealed granulomatous inflammation of the outer wall of the cavernous sinus. Under ideal conditions, a biopsy should be performed for diagnosis. Recent studies have shown that half of patients with confirmed diagnosis of the Tholos-Hunt syndrome present changes in the venous bed of the orbital region. If the granuloma is not detected during MRI, it is more appropriate to diagnose "upper globular fissure syndrome" and guide the patient under dynamic observation. There are also clinical diagnostic criteria that include such manifestations and conditions:

1. "Burning" or "tearing" pain of a permanent nature behind the eye socket or in the orbital temporal-frontal region;
2. impaired mobility of the eyeball, which occurred immediately or within 14 days after the onset of pain syndrome;
3. the defeat of other nerves that pass through the upper orbital gap (III, IV, VI cranial nerves, I branch of the trigeminal nerve, vegetative fibers);
4. symptoms intensify for several days, even weeks;
5. characterized by the presence of spontaneous remissions, often without residual effects;
6. the disease can return in a few months or years;
7. with a thorough examination, they do not find other reasons capable of causing this condition;
8. regress symptomatology within 72 hours after the onset of immunosuppressive therapy.

Treatment

The only pathogenetically significant method of treating the Tolosa-Hunt syndrome is the use of steroids. The effect of their appointment appears in the first three days, which is one of the criteria for the correctness of the diagnosis. However, do not forget that a good response to the use of steroid drugs can be in other conditions, imitating the Tolosa-Hunt syndrome, such as pachymeningitis, chordoma, lymphoma, aneurysm, carcinoma and others.That is why there are difficulties with the reliability of the diagnosis, which requires a thorough and thorough examination of the patient. The use of other medicines is possible for the purpose of symptomatic treatment. Use analgesics and anticonvulsants to reduce pain syndrome, general-purpose drugs, vitamin therapy.