Susac syndrome

Not so long ago, at the end of the last century (1979), Dr. J. Susek and a group of co-authors first described 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 deterioration of vision, as well as the brain, expressed in the inability to maintain balance and memory loss. Patients with this syndrome live in the full sense of the word today, because they remember nothing about yesterday.

This syndrome was previously considered a form of the systemic disease "lupus" with a negative agglutination reaction, however, this assumption was not confirmed. Another name for it is retino-cochleocerebral vasculopathy, i.e. an inflammatory lesion of the cerebral blood vessels, as well as the retina and auditory nerve.

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Epidemiology

Susak syndrome is more common among women than among men in a ratio of 5:1. The epidemiology of the disease, identified only at the end of the 20th century, has not been thoroughly studied. One thing is certain - the disease is rare: according to various sources, from 100 to 250 cases of the syndrome have been registered in the world. The currently known age range of onset of the disease is 9-72 years, but most of the patients are women in the age group of 20-40 years.

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Causes Susac syndrome

The reasons for the sudden development of this disease are still unknown, although it has sometimes occurred after a viral infection. Other known risk factors include hormone replacement therapy and pregnancy.

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

Currently, this disease is classified as a mental disorder, without taking into account the fact that the patient’s behavior is absolutely adequate to the situation.

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Pathogenesis

The basis of the pathogenesis of the syndrome is the immune response of the body, which for some reason has perceived the 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 are swelling of the cells, and partial or absolute disorder of blood flow in the arterioles of the organ.

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Symptoms Susac syndrome

Susak syndrome is defined by a symptomatic triad:

• sensorineural hearing loss on both sides;
• periodically appearing veil, fogging in front of both eyes, double vision, short-term blindness of one eye, paracentral scotomas in the visual field, occasionally – persistent decrease in vision (ischemic retinopathy);
• fatigue and sleep disturbances, weakness and dizziness, severe headaches and amnesia, sometimes convulsions and autonomic disorders (symptoms of subacute encephalopathy).

At the same time, the early stages of the disease are not always characterized by the presence of all symptoms. The first signs of the development of the syndrome in about 90% of patients manifested themselves in the form of visual impairment, about 70% complained of hearing impairment. Less than half of patients at the onset of the disease sought help for neuropsychiatric disorders. Dizziness, partial loss of hearing and vision, severe headache and photophobia are very common complaints that people seek help for at the initial stages of the disease.

Magnetic resonance imaging shows multiple foci accumulating contrast in the white matter of the brain, cerebellum and corpus callosum, and single foci in the gray matter, which does not accompany other types of vasculitis.

Vascular transformations in the retina with symptoms of arteriolar occlusion and absence of demyelination are also characteristic of 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 have been episodes of sudden regression of the disease, which proceeded without any therapy.

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Diagnostics Susac syndrome

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

Magnetic resonance imaging shows small focal lesions of the white matter of the brain along the lateral ventricles, as well as the corpus callosum and cerebellum, similar to lesions seen in multiple sclerosis. There are also isolated areas in which the contrast marker accumulates. Such contrast is not typical for systemic lupus erythematosus.

MRI lesions in the gray matter of the brain are occasionally visible on the tomogram of patients with Susak syndrome, but, 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, electrooculography, ophthalmoscopy, fluorescent angiography and registration of evoked visual potentials of the cerebral cortex.

In this case, dilation of retinal arterioles, microaneurysms, and telangiectasias are detected. Small retinal hemorrhages are often encountered, accompanied by edema in the area of microangiopathy, caused by increased permeability of the vascular walls.

Hearing impairments are detected using otoacoustic emission, brainstem auditory evoked potential studies, and other otoneurological examinations.

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Differential diagnosis

Based on the results of diagnostic measures and anamnesis, differential diagnostics are carried out with other systemic vasculitis, multiple sclerosis and other diseases of the ear and eye that have similar symptoms.

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Treatment Susac syndrome

Drug therapy is carried out with glucocorticosteroids, mainly Methylprednisolone.

The drug's action is similar to prednisolone, however, its ability to retain sodium is less, so it is better tolerated. Methylprednisolone is absorbed more slowly and acts longer than hydrocortisone and prednisolone.

The average dosage is from 4 to 48 mg per day. The treatment regimen and dosage are prescribed by the doctor.

Long-term therapy with the drug (to reduce side effects and maximize effectiveness) is carried out according to the scheme: morning intake every other day of a double daily dosage. With prolonged use of the drug, gradual withdrawal is carried out with a step-by-step reduction in dosage.

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

Side effects when taking the drug may include
sodium overload, hyperhydration, hypokalemia, hypocalcemia, muscle weakness, decreased immunity, and menstrual irregularities.

In severe forms of the disease, cytostatics were used in combination with Methylprednisolone pulse therapy (drip administration of ultra-high doses of hormones over several days) - drugs that inhibit the process of pathological cell division. These drugs are mainly 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 has some cell division suppressing action. Oral administration, daily dosage is calculated as: 1.5-2 mg per 1 kg of body weight and divided into two or four doses.

Do not prescribe to pregnant and lactating women, children, in case of hypersensitivity, in cases of hematopoiesis suppression. Like all cytostatics, it has a lot of side effects, especially with prolonged use, including chromosomal changes, dyspeptic phenomena, liver dysfunction, hematopoiesis, carcinogenic, teratogenic. During the treatment period, it is necessary to monitor the blood composition every week.

In some cases, symptoms of coagulopathy were observed, and patients were accordingly prescribed drugs that reduce blood clotting and platelet aggregation.

For example, the indirect anticoagulant Warfarin, which prevents thrombus formation by inhibiting vitamin K.

The treatment regimen and dosage are individually prescribed by the attending physician. The daily dosage usually involves oral administration of no more than 5 mg, divided into two doses.

Warfarin is contraindicated for pregnant and lactating women. The drug is also not prescribed for liver and kidney failure, bleeding and acute hypertension. An overdose of this drug is fraught with bleeding.

The simplest antiplatelet agents include regular Aspirin, which is most often used to prevent thrombus formation. The effect appears no more than half an hour after taking it. The antiplatelet ability develops at a dosage of 75 mg to 325 mg. It can cause damage to the gastric mucosa, so it should be taken after meals with a sufficient amount of liquid, preferably milk or alkaline mineral water. Contraindicated for pregnant and lactating women.

Vasculopathies (vasculitis) require a certain diet and nutrition regime. Six meals a day are recommended. The daily diet should not contain more than eight grams of table salt.

The daily diet should include foods containing vitamins B1, B6, C, K and A. With vasculopathy, the body loses a significant amount of calcium; to compensate for this, it is necessary to consume low-fat dairy products daily.

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

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

In case of ischemia and retinal dystrophy, magnetic therapy, electroreflexotherapy, and laser stimulation of the retina are used to improve blood circulation.

In case of sensorineural hearing loss, the doctor may also prescribe physiotherapy treatment: laser procedures, electrophoresis, ultrasound therapy, transcranial magnetic therapy.

In cases of vasculitis and vasculopathies, hardware methods of blood purification from immune complexes are used – hemosorption and plasmapheresis.

Alternative treatment

In folk medicine, there are many recipes for reducing vascular inflammation, strengthening their walls and preventing bleeding.

However, before starting folk treatment, it is imperative to consult with your doctor; perhaps some recipe will successfully fit into the therapeutic regimen.

Green tea has a strengthening effect on the walls of blood vessels; you can drink 1/2 cup three times a day, but not before bed, as it has a stimulating effect.

Folk medicine suggests getting rid of vasculitis by drinking an infusion of bergenia crassifolia half an hour before breakfast. In the evening, brew a teaspoon of dried crushed leaves with a glass of boiling water, leave overnight. Strain in the morning, dilute a teaspoon of honey in it and drink.

To strengthen physical and mental health, healing Altai tea is used. True, to prepare it, you need to find a black leaf of bergenia. These are the lower leaves of the plant, dried and overwintered under the snow. It is best to pick them off the bergenia in the spring. The tea owes its properties to these leaves. They just need to be dried, they will be good for two years. After crushing the leaves, they are added to any mixture for brewing herbal tea or to regular tea.

Infusions of blackcurrant leaves and berries, elecampane herb, immortelle, St. John's wort, calendula flowers, burdock roots and leaves, and licorice roots have an anti-inflammatory effect.

Blackcurrant leaves are used to make an infusion - brew a tablespoon of crushed leaves in a thermos with a glass of boiling water, leave for two hours, strain. Drink four or five times a day, half a glass.

Rue and chokeberries, rose hips, cranberries and currants are used to strengthen the walls of blood vessels.

Drink from fragrant rue: for one teaspoon of the herb – 250 ml of boiling water, brew for five minutes. Strain and drink twice a day.

Chokeberry juice (aronia) is taken to strengthen blood vessels, one or two tablespoons three times a day. It is recommended to brew tea from dried chokeberry berries. Brew two tablespoons of dried berries with two glasses of boiling water, boil on low heat for ten minutes, then leave to infuse for at least five hours. Drink three times a day, adding honey.

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

Dosages and treatment regimens are prescribed by a homeopathic doctor only individually; there are no recommended doses of the drug in classical homeopathy, as in traditional medicine.

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

In homeopathy there are remedies for conditions whose description is similar to the first signs and residual effects of Susak's syndrome.

For example:

• Baryta carbonica (Baryta carbonica) - the patient shows all the signs of memory loss: forgets the order of everyday routine actions, his duties, words, loses orientation in the usual place; a veil before the eyes, weak vision, lacrimation; hearing loss, pathology of the auditory nerve; paralysis due to acute cerebrovascular accident;
• Baryta sulphurica (Baryta sulphurica) – shooting, aching, spasmodic, pressing pains in the temporal, frontal, occipital parts of the head, dizziness when standing, when walking; decreased visual acuity, veil, spots, flies before the eyes; tinnitus, hearing loss, pulsation, pain; painless paresis, weak memory, fainting;
• Chininum sulphuricum (Quininum sulphuricum) – hearing loss, sometimes with severe headache;
• Gelsemium – memory impairment, headaches, inflammation or detachment of the retina and other visual disorders, impaired motor coordination.

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Surgical treatment of the consequences of Susak syndrome

One of the signs of Susak's syndrome is ischemic retinopathy combined with occlusion of the central retinal artery; spontaneous restoration of 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 for optic nerve vascular diseases is very severe, but not hopeless. There are various methods of surgical improvement of retinal hemodynamics (for example, laser coagulation), their use depends on the assessment of the severity of the disorders.

Hearing is corrected by selecting a hearing aid. If only a small part of the cochlear receptor apparatus is preserved, cochlear implantation is used. These operations entered world practice in the late 80s of the last century.

Prevention

Susak's syndrome occurs suddenly for unknown reasons, so the main preventive measure is a healthy lifestyle that prevents frequent viral infections, which in many cases preceded the onset of this disease.

Acute hearing and vision impairment, especially in young people (the high-risk group includes pregnant women), should attract increased attention - mandatory hospitalization and thorough diagnostics: magnetic resonance imaging, studies of acoustic brainstem evoked potentials, since an untimely and incorrect diagnosis can lead to patient disability.

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Forecast

The duration of Susak syndrome is usually from one to five years, after which the condition stabilizes. Some studies describe cases of recurrent course of the disease for 18 years.

However, even if the disease is short-lived, vision and hearing may not be restored.

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