Syringomyelia disease: true causes and treatment. Syringomyelia - what is it? Symptoms, treatment of the disease Causes of the disease

Cervical syringomyelia most often occurs in people after 25-30 years of age. This disease appears unnoticed and cannot be completely cured.

It may go into remission, but the disease cannot be cured completely. The only way out will be timely treatment. It will ensure a fuller and longer life.

The process of occurrence of syringomyelia of the cervical and thoracic spine:

The spinal canal expands.
Liquid flows in this channel. She begins to avoid obstacles that appear in her path.
Cavities appear in the canal. They arise in places of detours.
Glial cells appear. Their formation is associated with the appearance of cavities.

Spinal diseases can rightly be called a scourge modern man. The appearance of some of them is due to in the wrong way life, while others arise after infection. TO last group This includes spinal tuberculosis, which is a dangerous and contagious disease.

As a result, the transmission of impulses from the brain to the organs is disrupted. Doctors have not yet figured out why such phenomena occur in the body. There are only theories to explain this disease.

Manifestations of the disease

Sudden weight loss indicates the first manifestations of syringomyelia. At the same time, the hands become noticeably weaker and sensitivity to pain and temperature decreases. Over time, it spreads to other areas of the body.

Important: On initial stage Most patients often burn themselves or cut their skin due to decreased sensitivity. At this stage you need to be careful.

The next stage is characterized by the appearance of aching pain in the limbs. Neurological pain occurs in the facial area. After this, the patient develops paresis of the upper and lower limbs. Tendon reflexes disappear and it becomes difficult to move independently.

The patient may have no sweating at all. In some cases, on the contrary, increased sweating appears.

Diagnostics

At the first symptoms of syringomyelia, the patient needs a neurological examination. Diagnostics includes:

Inspection by external signs.
Sensitivity to pain and temperature is checked.
Anamnesis collection.
Definition of reflexes.
A tomography of the spine is performed.
Tests (blood and urine) are taken.

Based on the examination results, the doctor prescribes treatment.

Treatment and prevention

It is impossible to completely get rid of the disease. But proper treatment will stop the development of the pathology and alleviate the symptoms.

Important: To eliminate pain symptoms The doctor prescribes x-ray therapy. With this therapy, a specific area of the body is irradiated. But this method is used on early stages.

Proserine therapy is also used in the early stages. This method significantly normalizes the transmission of impulses, and the patient's condition improves. But this procedure does not affect the formation of cavities in the cervical and thoracic spine.

Application radioactive iodine or phosphorus significantly affects the formation of cavities. These elements are capable of inhibiting the growth of glial cells.

On late stages diseases are prescribed surgical intervention. Often prescribed to patients whose symptoms progress.

If the first symptoms of syringomyelia of the cervical and thoracic spine occur, you should immediately consult a doctor and undergo an examination.

Main symptoms:

Facial muscle atrophy
Pain in the cervicobrachial region
Hand joint deformity
Pain sensitivity disorder
Impaired tongue mobility
Involuntary muscle twitching
Hoarseness of voice
Creepy crawling sensation
Skin tingling
Blue fingers
Loss of temperature sensitivity
Sweating
The appearance of ulcers
Decreased tendon reflexes
Cracks in the skin

Syringomyelia is a disease nervous system, which develops as a result of disruption of the neural tube in the fetus (during its stay in the mother’s womb) or as a result of injuries and diseases that affect the spinal cord. If a person progresses this pathological condition, then in the substance spinal cord areas of growth are formed connective tissue, the so-called glia. Over time, they disintegrate and cavities form, which are filled with cerebrospinal fluid. The danger also lies in the fact that they tend to grow rapidly.

As a result of the progression of syringomyelia instead of healthy nerve tissue pathological voids with liquid are formed. As a result, those parts of the human spinal cord that “came under attack” and were compressed or pinched by cavities cease to function normally. For the most part, this is what causes Clinical signs illness.

Classification

Clinicians divide syringomyelic cysts into:

communicating. A cyst forms, connecting to the canal through which the spinal cord passes. In this case, they talk about a congenital anomaly of the central nervous system;
non-communicating. The syringomyelic cavity does not have a lumen associated with the pathways along which the cerebrospinal fluid moves. In this case, the cavity is a completely isolated space. Pathology develops as a result of past illnesses.

Pathological cavities form and grow along the anterior or posterior surface of the spinal cord. The diameter of the cavities is different. They can be completely invisible, or have a diameter of up to 2 cm.

Causes

There are many factors that could provoke the progression of syringomyelia. Depending on the reasons that caused the disease, clinicians divide syringomyelia into:

primary or true;
secondary.

True

This form develops as a result of disturbances that occurred during the intrauterine development of the fetus, at the stage of the formation of its central nervous system. In the first 3 months of fetal development, under the influence of synthetic medical drugs and other unfavorable factors, abnormal formation of the spinal cord occurs, which, in turn, leads to the formation of cavities and outgrowths.

Secondary

This disease develops in a normally formed and functional spinal cord after illness and injury. The main reasons for the progression of the disease:

tumors;
injuries;
previous neurological operations;
tuberculous or purulent lesion of the membranes of the brain.

Symptoms

Symptoms of the disease may for a long time do not appear. A simple cough or sneezing can trigger the appearance of symptoms. The disease is characterized by the development of 3 groups of disorders:

vascular;
sensitive;
motor.

Sensory disorders

For violations of this type The following symptoms are typical:

a person does not feel pain in certain areas;
the patient ceases to feel temperature.

Vascular disorders

In this case, the symptoms are pronounced. At the beginning of the progression of the disease there is increased sweating upper body and also on the face. As syringomyelia begins to develop, indicated symptoms peeling of the skin and dryness also occur. As a result, deep cracks and ulcers form, which heal very poorly. The nail plates weaken and begin to crumble and break.

In severe cases, the large joints of the limbs are affected - they are affected. Curvature of the spine can also often occur.

Movement violations

In this case, the main symptom is paresis in the muscles of the hands. The patient notes that it is difficult for him to button clothes, write, lift small objects, and even lace his shoes. The muscles of the hands gradually atrophy (shrink out). As a result, a “clawed hand” is formed - characteristic symptom pathology. If a person is affected by syringomyelia, then the tendon reflexes gradually decrease, to the point that they disappear completely.

If syringomyelia also affects the lateral parts of the spinal cord, then the symptoms described above begin to manifest themselves in the legs. If syringomyelic cysts have formed in the medulla oblongata, then the nerve nuclei will gradually begin to destroy.

As a result, the following violations will occur:

decreased hearing function;
violation of the swallowing reflex;
speech disorder.

Diagnostics

If the above symptoms of syringomyelia occur, put accurate diagnosis it won't be difficult. Later, others are appointed to confirm it. instrumental methods diagnostics MRI is the most effective for syringomyelia. In the resulting images, it is possible to see the presence of pathological cavities or an increase in the size of the spinal cord.

Additional diagnostic methods:

immunological research;
rheoencephalography.

Treatment

Treatment of this spinal cord disease is very complex and is carried out throughout the patient’s life. The treatment method is selected taking into account the severity and characteristics of the pathology and includes the following:

correct mode;
conservative treatment methods;
surgery.

Drug treatment includes the following:

amino acids;
vitamin complexes;
drugs that stimulate blood circulation in the vessels of the brain;
drugs that improve the conduction of nerve impulses.

Treatment with medications is carried out in courses - 2-3 times a year.

If syringomyelia occurs rapidly and clinical symptoms are more than pronounced, then in this case it is indicated to carry out surgical treatment of the disease. Its essence lies in the fact that the surgeon installs a special device. shunts between the formed syringomyelic cavities. After this type of treatment, the patient will need to undergo a long course of rehabilitation.

Treatment of the disease is carried out only in inpatient conditions and under the constant supervision of doctors. Only in this case will it be effective. Various treatments at home folk remedies unacceptable, as it can lead to the development of various complications.

Forecast

Syringomyelia does not lead to fatal outcome. Patients with this disease long time remain operational. Often we encounter patients in whom periods of exacerbation are followed by periods of calm (25%). In 60% of patients, the disease progresses slowly, and in 15% it does not progress at all.

Is everything correct in the article? medical point vision?

Answer only if you have proven medical knowledge

Syringomyelia (from Greek words"syrinx" - cavity and "myelon" - spinal cord) is chronic illness, which affects the central nervous system. It is characterized by damage to the substance of the spinal cord and the formation of cavities in it. True syringomyelia occurs as a result of pathology of glial tissue. The disease is incurable and is present throughout a person’s life.

Causes

The reasons for the development of true syringomyelia in humans are associated with birth defect glial tissue. Glial tissue is represented by auxiliary cells of the nervous system that surround neurons, protect them and take part in the transmission of nerve impulses.

In syringomyelia, there is excessive growth of glial cells in the brain stem and gray matter of the spinal cord, its cervical and thoracic regions.

The transferred infection or injury. Pathological cells glia that have multiplied excessively then die. As a result, cavities appear in the gray matter of the brain, which are lined from the inside with glial cells. A feature of these cells is the passage of fluid. Therefore, liquid accumulates in the cavities, which gradually increases their size. The formation of cavities leads to compression of adjacent nerve cells, their degeneration and subsequent death.

Progressive syringomyelia is characterized by a constant increase in the formed cavities, which is accompanied by death large quantity neurons.

Symptoms

Symptoms of syringomyelia vary depending on which part of the spinal cord is damaged and how large the affected area is. The symptoms are as follows:

Loss of sensitivity over large areas skin. Consists of loss of temperature and pain sensitivity. The disorder is dissociated in nature, that is, it has the form of a “collar,” “jacket,” or “half-jacket.” As a result, the patient does not have a normal reaction to stimuli. He does not feel pain, cold or hot temperature. The result may be injuries and burns. The development of soreness of the skin is possible, and paresthesia, a burning or cold sensation in the affected areas of the skin are also noted.
Deformation of skeletal bones.
Atrophy and paresis of the limbs. In this case, the hands can take on the appearance of a monkey's paw.
Vegetative-trophic disorders. The patient develops nail damage and hyperhidrosis ( excessive sweating), acrocyanosis, dermographism changes.

If the patient has developmental anomalies, there are consequences of injuries, or stenosis, then syringomyelia may not manifest itself in any way for a long time. The disease in this case is well masked behind the signs of the underlying disease.

It is necessary to note this variant of the course of syringomyelia, in which the pathological process transitions to the caudal (lower) part of the brain stem. This phenomenon is called syringobulbia.

Syringobulbia has the following symptoms:

loss of sensitivity of the skin on the face;
paresis of the larynx, pharynx, soft palate;
paresis of the tongue.

Diagnostics

Diagnosis of the disease is carried out using X-ray methods. X-rays make it possible to determine whether a person has trophic manifestations of syringomyelia in the form of destruction of the bone elements of the joints, osteoporosis and other disorders.

The most accurate diagnosis of the disease is made possible by MRI of the spine. If MRI is not possible, myelography is performed. These research methods make it possible to see the presence of syringomyelic cavities in the brain stem and spinal cord.

Treatment of syringomyelia initial stage its development, when glial cells actively multiply, is aimed at suppressing and inhibiting this pathological process. Correct treatment makes it possible to stop the progression of the disease and reduce the signs of its manifestation. For treatment during this period, radiotherapy is used, that is, the affected segments of the spinal cord are irradiated. Treatment with radioactive phosphorus or iodine is also practiced, since they tend to accumulate in the multiplying glial cells and irradiate them from the inside.

If a patient is prescribed treatment for syringomyelia with radioactive iodine, Lugol's solution is first prescribed. Iodine from this medicinal product fills the cells thyroid gland. Thus, they become protected from radioactive iodine.

Drug treatment of the disease is carried out by a neurologist. It includes the prescription of dehydrating drugs - furosemide, diacarb, neuroprotectors - actovegin, glutamic acid, pirocetam, vitamins. In order to relieve pain in the problem of syringomyelia, analgesic drugs are prescribed - analgin, ketorol, as well as ganglion blockers, which include pachycarpine.

A new method in the treatment of syringomyelia is treatment with proserin, which improves the conduction of nerve impulses. However, such therapy does not eliminate the cause of the disease, but makes it possible to temporarily improve neuromuscular conduction.

A combination of therapy using proserin with radon baths or UHF.

Surgery the disease is carried out with increasing neurological deficit, which manifests itself in the form of peripheral paresis of the arms and central paresis legs By using surgical intervention The syringomyelic cavities are drained, adhesions are removed, and the spinal cord is decompressed.

Forecast

True syringomyelia is characterized by a rather slow progressive course, which does not affect life expectancy. The patient remains able to work for a long time.

The development of infectious complications in syringomyelia is high risk the appearance of sepsis.

Syringobulbia is more difficult. Involvement in pathological process and defeat vagus nerve and respiratory center can be fatal.

Below is a video - a fragment of the program "Live Healthy" about syringomyelia:

Syringomyelia of the cervical and thoracic spine is a disease in which several pathologies are observed at once. Syringomyelia cannot be called an independent disease; it develops against the background of several disorders of the functioning of the spine.

With this disease, the spinal cord in the cervical and thoracic vertebrae is damaged. Cysts form in it - small cavities with fluid. It is the cyst that has Negative influence on the brain and causes symptoms of syringomyelia.

Syringomyelia is a pathology that itself can be a symptom serious illnesses spine, such as a tumor or injury. In rare cases, syringomyelia of the spinal cord occurs without a specific cause. This type of disease is called idiopathic.

What is syringomyelia?

Syringomyelia of the cervical and thoracic spine is incurable disease, which may be acquired or congenital. Congenital syringomyelia is inherited, most often through the male line, its first symptoms appear at about 30 years of age, but can occur much later.

The danger of syringomyelia is that the disease can spread above the cervical and thoracic regions and enter from the spinal cord into the medulla oblongata, which is important department brain. A complication of syringomyelia can be destruction of not only the spinal cord, but also bone tissue thoracic and cervical spine. This pathology is quite rare and still does not have an exact scientific explanation, since with other causes of spinal cord destruction such extensive damage to the integrity of bone tissue is not observed.

The process of cyst formation is explained mass death glial tissue cells. When there is injury or disease of the spine, glial tissue begins to grow rapidly, then these same cells quickly die, and a cavity forms in their place. After some time, fluid enters the cavity through other cells of the spinal cord. It gradually fills them to the limit, and then begins to expand and increase - a cyst is formed. The cyst begins to put pressure on other parts of the spine, mainly on other nerve cells. Gradually, active neurons begin to die, and the person’s motor activity is impaired.

Causes of the disease

The exact cause of the development of syringomyelia has not yet been scientifically substantiated or established. At the same time, doctors put forward several scientific hypotheses for the occurrence of pathological condition spine.

Syringomyelia symptoms

With true, congenital syringomyelia of the thoracic and cervical spine, following signs, which may appear when intrauterine development child:

congenital scoliosis and deformed chest;
development malocclusion and jaw abnormalities (high palate);
the presence of more than one pair of mammary glands (in women) or nipples (in men);
the disease leads to deformities such as dysplasia ears, bifurcated tongue, extra fingers and toes.

The symptoms of acquired syringomyelia vary significantly. The cause of their occurrence is associated with those parts of the spinal cord where the cyst or several cysts appeared. If the violations affect rear horns spinal cord, a person’s perception is significantly impaired outside world through sensations, primarily tactile. This can become dangerous not only for health, but also for human life.

Syringomyelia of the cervical and thoracic spine affects the heat sensitivity of human skin. Its violation leads to the fact that the patient cannot adequately sense the temperature of objects and liquids, so patients with syringomyelia often experience burns. Areas may appear on the body that may be completely or partially deprived of sensitivity, and a person may feel a slight tingling in these places, the appearance of “goosebumps” and slight trembling. Typically these symptoms appear in upper parts bodies. Over time they are supplemented aching pain in the neck, shoulder blades, chest and upper limbs.

The disease provokes neurotrophic skin disorders:

wounds do not heal well;
a lot of scars appear on the skin from minor cuts and burns;
the skin begins to deform along with the joints and bones;
the skin gradually becomes thicker, dries out and flakes.

If syringomyelia damages the anterior horn of the spinal cord, symptoms indicate gradual muscle atrophy upper limbs. Over time, it becomes increasingly difficult for a person to control his arms, shoulders and neck, and a disorder may develop. motor activity eyeballs.

When a cyst forms in lower parts brain, the functioning of the facial muscles is disrupted. A person may partially or completely lose facial expressions, it becomes difficult for him to speak, swallow, move his jaws and tongue, and his face may become distorted.

Treatment of syringomyelia

For syringomyelia, treatment is supportive in nature and is aimed primarily at reducing the symptoms of the disease. Typically, syringomyelia of the cervical and thoracic spine practically does not progress, but does not decrease. The sluggish nature of the disease rarely has a significant negative impact on a person’s performance and does not in any way affect life expectancy. The only exception is a cyst that is located directly in the medulla oblongata, as it affects breathing and nutrition.

Syringomyelia can be cured if treatment is aimed at stopping the proliferation of glial cells. To do this, when diagnosing possible education cysts, the patient is injected with a certain amount of radioactive phosphorus or iodine. By irradiating cells, radioactive elements destroy them and prevent tissue from growing further.

If syringomyelia has already occurred, it can be treated with medication under the supervision of a neurologist. Dehydrating agents are used as treatment to help remove excess liquid from tissues, neuroprotectors and with strong pain syndrome analgesics in the form of injections.

As complementary therapy Vitamin courses and massage are used. Massage for syringomyelia helps speed up the outflow of fluid from the spinal cord. It can also prevent the formation of cysts after hemorrhages in the spinal cord or injuries.

Treatment of true syringomyelia of the cervical and thoracic spine occurs with the help of surgery.

In this case, drug treatment can only partially alleviate the symptoms of the disease. A complete cure for true syringomyelia is almost impossible.

Surgery is also mandatory if a cyst appears in the case of a malignant tumor.

The term syringomyelia is a collective term and includes various states, which are characterized by damage to the spinal cord with the formation of pathological cavities filled with fluid.

In 1827, the French physician Charles Prosper Olivier Angers (1796-1845) proposed the term syringomyelia, as syring from Greek means cavity (tube) and myelo means brain. Later, the term hydromyelia was used to denote enlargement of the spinal canal, and syringomyelia to denote cystic cavities without communication with the spinal canal.

Cavities in the spinal cord may be the result of a spinal cord injury, a spinal cord tumor, or congenital anomaly. The idiopathic form of syringomyelia (a form of the disease without specific reason). Fluid-filled cavities slowly expand and lengthen over time, leading to progressive damage nerve centers spinal cord, due to the pressure exerted by the fluid. This damage results in pain, weakness, and stiffness in the back, shoulders, arms, or legs. Patients with syringomyelia may have a varying combination of symptoms. In many cases, syringomyelia is combined with an abnormality of the foramen magnum, where the lower part of the medulla oblongata is located, which connects the brain and spinal cord. In addition, syringomyelia is often combined with Chiari malformation, in which part of the brain is displaced downward to medulla oblongata, thus reducing the spinal canal. Familial cases of syringomyelia are also sometimes observed, although this is rare.

Types of syringomyelia include:

syringomyelia with communication with the fourth ventricle
syringomyelia due to CSF block (without connection with the fourth ventricle)
syringomyelia due to spinal cord injury
syringomyelia and spinal dysraphism (incomplete closure of the neural tube)
syringomyelia due to intramedullary tumors
idiopathic syringomyelia

Syringomyelia occurs in about eight out of every 100,000 people. The onset of the disease is most often noted between the ages of 25 and 40. Rarely, syringomyelia can develop in childhood or in old age. Men get this disease more often than women. No geographic or racial dependence was noted. Cases of familial syringomyelia have been described.

Causes and symptoms

Most patients with syringomyelia experience headaches, often in combination with periodic pain in the arms or legs, usually more severe in half of the body. The pain may begin as dull and mild and gradually increase in intensity, or it may appear suddenly, often as a result of coughing or straining. Pain in the limbs often becomes chronic. You may also experience numbness and tingling in your arm, chest, or back. An inability to feel the ground under your feet, or tingling in the legs and feet, may also occur. Weakness in the limbs with syringomyelia leads to improper motor skills of hand movements or impaired walking. Eventually, functional use of the limbs may be lost.

The causes of syringomyelia remain unknown. No theory at present can correctly explain the basic mechanisms of the formation of cysts and cystic enlargements. One theory suggests that syringomyelia occurs as a result of pulsating cerebrospinal fluid pressure between the fourth ventricle of the brain and the spinal canal. Another theory suggests that cysts develop due to differences intracranial pressure and spinal pressure, especially in the presence of Chiari malformation. A third theory suggests that the formation of cysts is caused by the cerebellar tonsils acting like a piston and causing a large pressure drop of the cerebrospinal fluid in the subarachnoid space, and this action of fluid forces affects the spinal cord. Syringomyelia usually progresses slowly, and the course of the disease lasts for years. But sometimes there is an acute course of the disease, especially when the brain stem is involved.

Diagnostics

An examination by a neurologist may reveal loss of sensation or movement caused by compression of the spinal cord. Diagnosis is usually made using magnetic resonance imaging (MRI) of the spine, which can confirm the presence of syringomyelia and determine the exact location cystic formations and the degree of spinal cord damage. The most common location of cysts is cervical region or thoracic region spine. Least probable place localization of cysts is lumbar region spine. An MRI of the head can be of diagnostic value to determine the presence of any changes such as hydrocephalus (excess cerebrospinal fluid in the ventricles of the brain). As cystic formations increase, spinal deformity (scoliosis) may occur, which can be easily diagnosed using radiography. The degree of conduction disturbance in syringomyelia is determined using EMG.

Treatment

Diagnosis and treatment of syringomyelia requires a whole team of medical specialists, including neurologists, radiologists, neurosurgeons, and orthopedists.

Treatment, usually surgical, is aimed at stopping the progression of spinal cord damage and preserving functionality. Surgical procedures are often performed when there is spinal cord compression. In addition, surgical procedures are performed to correct deformities or apply various shunts. Surgeries were also performed to implant fetal tissue to close the cystic formations. Surgery results in stable or moderate improvement of symptoms in most patients. Many doctors believe that surgical treatment is necessary only for patients with progressive neurological symptoms. Delaying surgical treatment in cases where the disease progresses can lead to irreversible damage to the spinal cord and severe permanent neurological impairment.

Drug treatments (vasoconstrictors) are often prescribed to reduce swelling around the spinal cord. It is often recommended to avoid active physical activity which can increase venous pressure. Certain exercises, such as bending your torso forward, can reduce the risk of cystic growths expanding. Life expectancy in patients with progressive symptoms without surgical treatment ranges from 6 to 12 months.

Recovery and rehabilitation

Despite reports of complete neurological recovery after surgery, most patients experience stable or only moderate improvement in symptoms. Syringomyelia in children is characterized by much less pronounced violations sensory and pain than in adults, but much great risk development of scoliosis, which is more favorable for surgical treatment. In addition, syringomyelia does not progress equally in all patients. Some patients, usually those with milder symptoms, experience stabilization of symptoms over the course of a year. A common complication progression of symptoms is the patient’s need to adapt to life due to impairment or loss of some functions. These adjustments can result in a loss of quality of life. The task of rehabilitation is maximum possible preservation functionality using exercises and adaptive equipment, or, especially in the case of syringomyelia in children, interventions should be aimed at correcting scoliosis.

Forecast

The prognosis for patients with syringomyelia depends on the underlying cause of the cyst and the type of treatment. Untreated cases of syringomyelia in 35-50% of cases have a prognosis for long-term survival. Patients who underwent bypass surgery for spinal cord injury experienced long-term pain relief and increased survival. Recent studies have shown unsatisfactory long term forecast due to high rates of recurrence of cyst formation in other forms of syringomyelia. Surgical treatment (posterior decompression) for syringomyelia combined with Chiari malformation is considered sufficient effective treatment with a greater chance of clinical improvement. For syringomyelia in children surgical treatment effective for stabilizing scoliosis.