Susaka syndrome

Not long ago, at the end of the last century (1979), Dr. J. Susek with a group of co-authors for the first time made a description of the disease, which was later named after him. This is a rare autoimmune pathology, which is characterized by microangiopathy in the arterioles of the cochlea of the inner ear, the retina of the eye, which leads to hearing loss and impaired vision, as well as the brain, manifested in the inability to maintain balance and memory loss. Patients with this syndrome live in the fullest sense of the day, for they do not remember anything about yesterday's day.

This syndrome was previously considered a form of systemic disease "red lupus" with a negative agglutination reaction, however, this assumption was not confirmed. Another one of its name is retino-cochleo-cerebral vasculopathy, i.e. Inflammatory damage of the cerebral blood vessels, as well as the retina and the auditory nerve.

[1], [2], [3], [4]

Epidemiology

Susak syndrome is more common among women than among men in the ratio of 5: 1. The epidemiology of the disease, defined only at the end of the twentieth century, has not been thoroughly studied. Undoubtedly one - the disease is rare: according to different data in the world recorded from 100 to 250 cases of the syndrome. The interval of the onset of the disease, known to date, is 9-72 years, but most of the cases are women of the age group of 20-40.

[5], [6], [7]

Causes of the susak syndrome

Until now, the causes of the sudden development of this disease are unknown, although it sometimes appeared after a viral infection. Other known risk factors are hormone replacement therapy and pregnancy.

All these data lead to the hypothesis that Susak syndrome is preceded by dysfunctions of the immune system, changes in the hormonal background and blood rheology.

Currently, this disease is attributed to mental disorders, not taking into account the fact that the patient's behavior is absolutely adequate to the situation.

[8], [9], [10], [11], [12], [13], [14]

Pathogenesis

The basis of the pathogenesis of the syndrome is the immune response of the body, for whatever reason perceived endothelial cells, a continuous layer lining the blood vessels of the cochlea of the inner ear, the retina of the eye and the brain, as an antigen. The consequences of this immune attack is the swelling of the cells, and the partial or absolute disorder of blood flow in the arterioles of the organ.

[15], [16], [17], [18], [19], [20], [21], [22]

Symptoms of the susak syndrome

The Susak syndrome is defined by the symptomatic triad:

• sensorineural hearing loss on both sides;
• periodically appearing shroud, clouding before both eyes, double vision, short-term blindness of one eye, paracentral scotoma in the field of vision, occasionally - persistent decline in vision (ischemic retinopathy);
• fatigue and sleep disorders, weakness and dizziness, severe headaches and amnesia, sometimes convulsions and autonomic disorders (symptoms of subacute encephalopathy).

In this case, the early stages of the disease are not always characterized by the presence of all the symptoms. The first signs of the syndrome developed in about 90% of patients showed up as a visual impairment, about 70% complained of hearing impairment. Less than half of patients in the debut of the disease were treated for neuropsychiatric disorders. Dizziness, partial loss of hearing and vision, severe headache and photophobia are very common complaints that are addressed to the doctor at the initial stages of the disease.

On the magnetic resonance tomogram, multiple foci that accumulate contrast in the white substance of the brain, the cerebellum and callus body, and the solitary ones in gray are notable, which does not accompany other types of vasculitis.

Vascular transformations in the retina with symptoms of arteriolar occlusion and lack of demyelination are also inherent in this pathology.

Complications and consequences

The consequences and complications of late or incorrect diagnosis of Susak syndrome can lead to complete or partial disability. There were episodes of sudden regression of the disease, which occurred without any therapy.

[23], [24], [25], [26], [27], [28], [29], [30]

Diagnostics of the susak syndrome

Blood tests for Susak syndrome from other vasculitides are distinguished by the absence of any changes in blood. Also, instrumental diagnostics (ultrasound, dopplerography) does not reveal systemic vascular disorders.

In magnetic resonance tomography, small focal lesions of the white matter of the brain along the lateral parts of the ventricles, as well as the corpus callosum and cerebellum, like lesions in multiple sclerosis, are seen. There are also single zones in which a contrast marker accumulates. This contrast is not typical for systemic lupus erythematosus.

On the tomogram of patients with Susak syndrome, MRI foci in the gray matter of the brain are occasionally seen, however, in contrast to multiple sclerosis, they are never located in the spinal cord.

To study the state of the retina in modern diagnostics, instrumental methods are used: electroretinography, electro-oculogia, ophthalmoscopy, fluorescent angiography and recording of evoked visual potentials of the cerebral cortex.

At the same time, an increase in retinal arterioles, microaneurysms, and telangiectasia is found. Often there are small retinal hemorrhages, accompanied by edema in the field of microangiopathies, caused by increased permeability of the vascular walls.

Hearing disorders are detected with the help of otoacoustic emission, study of stem auditory evoked potentials, and other otoneurological examinations.

[31], [32]

Differential diagnosis

Based on the results of diagnostic activities and anamnesis, differential diagnosis is performed with other systemic vasculitis, multiple sclerosis and other ear and eye diseases that have similar symptoms.

[33], [34], [35], [36], [37], [38]

Treatment of the susak syndrome

Drug therapy is carried out by glucocorticosteroids, mainly - Methylprednisolone.

The action of the drug, similar to prednisolone, however, the ability to delay the sodium it has less, accordingly it is better tolerated. Methylprednisolone is absorbed more slowly and lasts longer than hydrocortisone and prednisolone.

The average dosage ranges from 4 to 48 mg per day. The doctor prescribes the treatment and dosage regimen.

Long-term therapy with the drug (to reduce side effects and maximum efficiency) is carried out according to the scheme: morning intake twice a day, twice the daily dosage. With prolonged use of drugs, a gradual cancellation with a gradual decrease in dosage is made.

Contraindicated in the presence of hypertension severe, mental disorders, violations of adrenal function, osteoporosis, ulcerative diseases of the gastrointestinal tract, because it can cause similar side effects and aggravate the pathological process. It is not prescribed in the postoperative period, patients with syphilis, during an exacerbation of endocarditis, an open tuberculosis process, with inflammation of the kidneys, as well as - to pregnant and lactating women, with caution - for the elderly.

Side effects when taking the drug may be
Sodium supersaturation, hyperhydration, hypokalemia, hypocalcemia, weakness in muscles, decreased immunity, menstrual cycle disorders.

In severe forms of the disease in combination with pulse therapy Methylprednisolone (the drip injection of ultrahigh doses of hormones for several days) used cytostatics - drugs that inhibit the process of pathological cell division. Basically, these drugs are used in the treatment of malignant neoplasms, but in some cases they are used in the treatment of autoimmune diseases.

For example, Azathioprine is an immunosuppressant that simultaneously exerts some overwhelming cell division action. The intake of oral, daily dosage is calculated: 1.5 -2 mg per 1 kg of body weight and divided into two or four doses.

Do not prescribe pregnant and lactating women, children, with hypersensitivity, in cases of oppression of hematopoiesis. Has, like all cytostatics, a lot of side effects, especially with prolonged use, including. Causes chromosomal changes, dyspeptic phenomena, violations of the liver, hematopoiesis, carcinogenic, teratogen. During the treatment period, the blood composition should be monitored weekly.

In some cases, the symptoms of coagulopathy were observed, and patients, respectively, were prescribed medications that reduce blood clotting and platelet aggregation.

For example, an indirect anticoagulant Warfarin, which prevents thrombosis, inhibiting vitamin K.

The treatment plan and dose are individually prescribed by the attending physician. The daily dosage usually assumes an oral intake of not more than 5 mg, divided into two doses.

Pregnant and lactating women Warfarin is contraindicated. Also, do not prescribe the drug for hepatic and renal failure, bleeding and acute hypertension. Overdose of this drug is fraught with bleeding.

The most simple antiplatelet agents are usual Aspirin, which is most often used to prevent thrombosis. The action is manifested after no more than half an hour from the moment of reception. Antiaggregant ability develops at a dosage of 75 mg to 325 mg. It is able to cause damage to the mucous membrane of the stomach, so take it after eating, squeezed with enough liquid, preferably - milk or alkaline mineral water. Contraindicated in pregnant and lactating women.

Vasculopathies (vasculitis) require a certain diet and diet. Six meals a day are recommended. In the daily diet should not be more than eight grams of table salt.

Daily in the diet should be present foods containing vitamins B1, B6, C, K and A. When vasculopathy the body loses a significant amount of calcium, to compensate for it, daily use skimmed milk products.

According to some data, a good effect in the treatment of vasculopathy (vasculitis) gives an application in the complex therapy of ginkgo biloba preparations. To activate brain functions (improve memory, eliminate inhibition), an extract of leaves of this plant is used. Unextracted drugs (alcohol tincture or powder from dried leaves) enliven the functioning of nerve cells, microcirculation of blood, visual and auditory nerves, restore elasticity and strength to the walls of blood vessels, improve blood flow.

In combination with medicines and vitamins, physiotherapy is also combined, in particular, hyperbaric oxygenation - the use of oxygen in pressure chambers.

With ischemia and dystrophy of the eye retina, in order to improve blood circulation, magnetotherapy, electroreflexotherapy, laser stimulation of the retina are used.

With sensorineural hearing loss, a physician can also prescribe physiotherapeutic treatment: laser procedures, electrophoresis, ultrasound therapy, transcranial magnetotherapy.

In vasculitis and vasculopathy, the use of hardware methods for purifying blood from immune complexes - hemosorption and plasmapheresis.

Alternative treatment

In alternative medicine, there are many recipes for reducing inflammation of the vessels, strengthening their walls and preventing bleeding.

However, before proceeding with alternative treatment, be sure to consult with your doctor, maybe some recipe will be successfully integrated into the therapeutic scheme.

Vigorously acting on the walls of blood vessels is green tea, which can be drunk 1/2 cup three times a day, but only not before going to bed, as it has an exciting effect.

Get rid of vasculitis alternative treatment suggests, using half an hour before breakfast, infusion of fatty thick bean. In the evening, a teaspoon of dried shredded leaves brew a glass of boiling water, insist all night. In the morning, drain, dissolve in it a teaspoon of honey and drink.

To strengthen physical and mental health used healing Altai tea. However, for its preparation it is necessary to find a black sheet of a badan. These are the lower leaves of the plant, dried and overwintered under snow. It's best to cut them off from the badan in the spring. Its properties are due to these leaves. They just need to be dried, they will be suitable for two years. Chopping the leaves, they are added to any mixture to brew herbal tea or to regular tea.

Anti-inflammatory effect is infusions of leaves and berries of black currant, grass elecampane, immortelle, St. John's wort, flowers of marigold, roots and leaves of burdock, licorice roots.

Leaves of black currant are used for cooking infusion - brew in a thermos a tablespoon of chopped leaves with a glass of boiling water, insist for two hours, strain. Eat four or five times a day for half a cup.

As a vessel strengthening the walls of the vessels, rue fragrant and berries of chokeberry, dogrose, cranberries, currants are used.

Drink from rue scented: for one teaspoon of herbs - 250 ml of boiling water, brew for five minutes. Strain and drink twice a day.

Juice made from chokeberry (aronia) takes one or two tablespoons three times a day to strengthen the vessels. It is recommended to brew tea from dry berries of black chokeberry. Brew two tablespoons of dried berries with two glasses of boiling water, boil over a low heat for ten minutes, then insist for at least five hours. Drink three times a day, adding honey.

Homeopathy is a special area of clinical medicine characterized by a different approach to the principles of treatment. It does not use impressive doses of chemicals with a wide range of effects on patients with the same diagnosis.

Dosages and treatment regimens are prescribed by a homoeopathic doctor only individually, the recommended doses of the drug, as in traditional medicine, are not found in classical homeopathy.

The complex of symptoms in the patient is carefully studied and a drug is prescribed that can cause similar symptoms in a healthy person. Homeopathy treats not a disease, but a person, that is, the whole complex of symptoms inherent in a particular patient, with one drug. In this case, the drug is prescribed in ultra low doses.

In homeopathy there are preparations for conditions, the description of which is similar to the first signs and the residual symptoms of Susak syndrome.

For example:

• Baryta carbonica - the patient shows all the signs of a lack of memory: he forgets the order of everyday ordinary actions, his duties, words, ceases to be guided in the usual locality; a veil before the eyes, weakness of vision, lacrimation; hearing loss, abnormality of the auditory nerve; paralysis due to acute circulatory disorders of the brain;
• Baryta sulphurica - shooting, aching, spasmodic, pressing pains in the temporal, frontal, occipital parts of the head, dizziness in standing position, walking; reduction of visual acuity, shroud, specks, front sight; noise in the ears, hearing loss, ripple, pain; painless paresis, weak memory, fainting;
• Chininum sulphuricum (Hininum sulfuricum) - hearing impairment, at times with severe headache;
• Gelsemium - memory impairment, headaches, inflammation or retinal detachment and other visual disturbances, impaired coordination of movement.

[39], [40], [41], [42]

Surgical treatment of the effects of Susak syndrome

One of the signs of Susak syndrome is ischemic retinopathy in combination with occlusion of the central artery of the retina, independent restoration of the vision in this case is extremely rare. Acute circulatory disorders in the retina and optic nerve almost always cause partial, and sometimes - absolute blindness. The prognosis of diseases of the vessels of the optic nerve is very heavy, but not hopeless. There are different methods of surgical improvement of the retina hemodynamics (for example, laser coagulation), their application depends on the assessment of the severity of the disorders.

The hearing is corrected by the selection of the hearing aid. With the preservation of only a small part of the receptor apparatus of the cochlea, cochlear implantation is used. These operations entered the world practice in the late 80s of the last century.

Prevention

The Susak syndrome arises suddenly due to unidentified exact causes, therefore the main preventive measure is a healthy lifestyle, which prevents frequent viral infections that preceded in many cases the occurrence of this disease.

Acute hearing and vision impairment, especially in young people (high-risk group includes pregnant women) should cause increased attention - mandatory hospitalization and thorough diagnosis: magnetic resonance imaging, studies of acoustic stem evoked potentials, since an untimely and incorrectly diagnosed diagnosis may result to invalidate the patient.

[43], [44], [45]

Forecast

The duration of the Susak syndrome, usually from one to five years, then the condition stabilizes. Some studies describe cases of recurrent disease over a period of 18 years.

However, even with a short duration of the disease, vision and hearing may not recover.

[46], [47], [48]