Causes, symptoms and treatment of syringomyelia of the cervical and thoracic spine. Syringomyelia disease: true causes and treatment Syringomyelia of the spine

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 of the nervous system that 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 affecting the spinal cord. If this pathological condition progresses in a person, then areas of proliferation of connective tissue, the so-called glia, form in the substance of the spinal cord. 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, pathological voids with fluid are formed instead of healthy nervous tissue. 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 the clinical signs of the disease.

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 not appear for a long time. 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

This type of disorder is characterized by the following symptoms:

• 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, increased sweating is observed in the upper part of the body, as well as on the face. As syringomyelia begins to develop, these symptoms are also accompanied by peeling skin and dryness. 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 - a characteristic symptom of the 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 on 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 appear, making an accurate diagnosis will not be difficult. Later, other instrumental diagnostic methods are prescribed to confirm it. 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 progresses rapidly and the clinical symptoms are more than pronounced, then surgical treatment of the disease is indicated. 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 hospital conditions and under the constant supervision of doctors. Only in this case will it be effective. Treatment at home with various folk remedies is unacceptable, as it can lead to the development of various complications.

Forecast

Syringomyelia is not fatal. Patients with this disease remain able to work for a long time. 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.

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Many people are interested in syringomyelia - what it is and how such a disease manifests itself. This is a lesion that is characterized by a slow, chronic progression. If treatment is untimely or incorrectly carried out, there can be a variety of complications that lead to paralysis or even death of the patient.

Features of the disease

What it is, syringomyelia, is a must-know for all those who have been diagnosed with this disease. It occurs in a chronic form and is characterized by the formation of cavities in the spinal cord filled with fluid. As a result, the cyst grows, which then puts pressure on the vertebrae, as well as the nerve endings. All this leads to tissue damage, poor posture and spinal deformation.

In addition, this negatively affects motor activity, as there is weakness of the limbs, stiffness of movement, and pain in the back. Often one or more signs are observed, and their combination can be very different.

The disease is completely incurable and enters a chronic stage. To relieve symptoms, symptomatic or neuroprotective therapy is used. In particularly severe cases, surgery is performed to remove excess fluid from the cyst.

The development of the disease proceeds very slowly, it all depends on the factor that provoked the pathological process, so several years may pass before the exacerbation occurs. Sometimes the disease can affect the brain, and in this case the pathological process can occur rapidly and in a very complex form.

Main forms of the disease

It is important to know not only what it is, syringomyelia, but also what forms of this disease exist. There are several forms of development of the disease, in particular, such as:

• prohornal;
• bulbar;
• posthorn;
• mixed;
• vegetative-trophic.

In addition, there may be true syringomyelia, which is a congenital disease caused by a dysfunction of the spinal cord during the embryonic development of the child. The post-traumatic form is an acquired disease caused by trauma.

Syringomyelia can be caused by the development of malignant neoplasms, as well as compression of nerve endings. The disease can be triggered by intervertebral hernias.

Provoking factors

Knowing what it is, syringomyelia, and what the causes and symptoms are, you can promptly recognize the course of the disease and carry out comprehensive treatment to prevent the development of complications. The causes of the disease are associated with congenital defects of glial tissue, which is represented by the cells of the nervous system surrounding neurons. These cells take part in the transmission of nerve impulses.

During syringomyelia, excessive growth of glial tissue is observed in the brain and spinal cord of the thoracic and cervical region. The pathological process can be triggered by an early infection or injury.

A special feature is that they are able to pass liquid. Therefore, liquid accumulates in the cavity, which gradually leads to an increase in size. The formation of cavities provokes compression of nearby nerve cells, their destruction and subsequent death.

Main symptoms

Syringomyelia of the spine can occur without obvious signs for a long time, which makes its diagnosis much more difficult. Among the main symptoms we can highlight the following signs:

• impaired sensitivity in the limbs;
• deterioration of skin sensitivity;
• tremor of the limbs;
• amyotrophy;
• deformation of the skull, spine, chest;
• poor skin regeneration.

Initially, there is a violation of sensitivity, as a result of which the skin loses the ability to feel pain and the effects of temperature. Depending on the severity of the disease, some parts of the body may lose sensation.

With the subsequent development of symptoms, sensory disturbances can develop into a deeper form, and at the same time there is a loss of sensitivity in the muscles and joints. A characteristic sign is a sharp decrease in muscle volume, up to their atrophy and impaired hand mobility. Trophic ulcers and vascular disorders quite often occur in the affected area.

Syringomyelia is often accompanied by prolonged attacks of aching pain in the chest, neck, shoulder blades, shoulder joints and arms. Osteoporosis is considered one of the common manifestations of the disease.

Signs of syringobulbia

The presence of a cavity in the medulla oblongata is called syringobulbia. This disease differs from syringomyelia in the symptoms and nature of the lesion. In this regard, the glossopharyngeal, hypoglossal and vagus nerves suffer. This pathology provokes symptoms such as:

• numbness of the palate and tongue;
• deterioration of taste;
• hoarseness of voice;
• hearing impairment.

The formation of a cyst in the medulla oblongata can also be observed in other parts of the spine. Among the complications of syringobulbia are atrophy of tissues located in the facial area, and in some cases even respiratory arrest.

Carrying out diagnostics

Syringomyelia of the cervical and spinal column is quite complex, so it is important to carry out timely diagnosis to determine the presence of the disease. Among the main diagnostic methods are the following:

• Magnetic resonance imaging;
• X-ray examination;
• electromyography;
• otoneurological examination.

Electromyography allows you to assess the degree of motor neuron impairment through certain signals coming from the spinal cord. An otoneurological examination is carried out in case of suspected syringobulbia and damage to the nerve fibers of the brain stem. In addition, it is advisable to consult a psychologist when conducting a diagnosis.

Features of treatment

It is impossible to completely cure cervicothoracic syringomyelia. With the help of therapeutic and preventive techniques, acute symptoms can be eliminated and the development of the pathological process can be stopped.

The specifics of therapy largely depend on the specific stage of the disease. At the initial stage of the disease, when pathological proliferation of glial cells occurs in the tissues of the spinal cord, treatment is aimed at suppressing their growth. For this, methods such as:

• X-ray therapy;
• treatment with radioactive iodine;
• drug therapy;
• physiotherapeutic techniques.

Surgical intervention is performed only in exceptional cases with a rapidly progressing form of the disease. The operation does not completely get rid of the disease, so it is performed in the most extreme cases.

Drug treatment

Treatment of syringomyelia is carried out with the use of medications and is aimed at eliminating the main symptoms that provoke discomfort. Neuroprotectors will help reduce the fluid content in the cavity and reduce pressure on surrounding tissues.

Painkillers and ganglion blockers will help get rid of dull or sharp pain with cervical syringomyelia. The drug “Proserin”, which can be combined with radon baths or UHF, will help normalize neuromuscular impulses. Such a procedure will only allow you to eliminate the discomfort for a while, without in any way affecting the course of the disease itself.

Surgical intervention

For syringomyelia of the cervical spine, surgical intervention may be performed. The operation is prescribed in the presence of voids, a congenital disorder of the outflow of cerebrospinal fluid from the brain. Surgically, it is possible to eliminate the formed cysts, as well as correct existing disorders of the structure of the spine.

In addition, cystic tumors can be drained to remove excess fluid. Pathology diagnosed in the initial stages is treated with X-rays or radioactive iodine. X-ray therapy makes it possible to prevent pathological proliferation of glial tissue. As a result, small cavities will not grow in the future and cause significant harm to health.

The peculiarity of therapy with radioactive iodine is that the substance used accumulates in the affected cells, and then they are irradiated from the inside, and as a result, the disease does not develop further. To avoid its excessive accumulation in the thyroid gland, you need to use the Lugol solution in advance.

Complication of the disease

Complications of syringomyelia of the thoracic or cervical spine are characterized by the occurrence of a number of diseases of the internal organs. Often, concomitant diseases may be a disruption of the functioning of internal organs and endocrine glands. Due to deterioration of blood supply, oxygen starvation of the heart muscle is possible.

With secondary infection, the patient’s respiratory organs and genitourinary system may be involved in the pathological process. The danger of the disease occurring in the brain stem area is the likelihood of bulbar palsy, which leads to respiratory arrest.

Prognosis after treatment

Thoracic syringomyelia can occur even in young children, but they do not pay any attention to the disease. The disease is diagnosed mainly at the age of 20-40 years. It develops gradually, and periodic remissions are also possible. In some cases, sudden deterioration is observed, up to the occurrence of transverse damage to the spinal cord, provoked by hemorrhages.

The prognosis for life is favorable; patients retain their ability to work for a long time, adapting to possible changes provoked by the disease.

Preventive actions

Prevention measures, first of all, relate to avoiding the danger of infectious diseases, which can provoke the progression of the disease and lead to the development of sepsis. The patient is advised to lead a healthy lifestyle, eat properly and nutritiously, and undergo regular examination and treatment by a neurologist.

In addition, it is important to prevent the progression of the disease, as well as to prevent the occurrence of injuries, burns and infections. All preventive measures are selected by the doctor strictly individually depending on the patient’s condition.

According to ICD 10, syringomyelia received code G95.0 and belongs to class VI as a disease of the nervous system.

Syringomyelia of the spinal cord is a rare disease. It occurs in 8 people out of 100,000. Men suffer from this disease more often than women.

People whose work is most predisposed to this disease are associated with heavy physical activity.

In total, the disease can last 20-40 years and only in rare cases affects life expectancy. Death can only occur due to complications or concomitant diseases.

In very rare cases, the cause of death may be progressive bulbar disorder, which is accompanied by heart rhythm and breathing disorders.

Causes

Syringomyelia can be either congenital or acquired. Congenital develops when the embryo is not formed correctly, if disturbances occur during the formation of the spinal cord.

Contribute to the development of SM and congenital pathologies of the development of certain brain structures - Dandy-Walker syndrome, Arnold-Chiari anomaly.

In congenital syringomyelia, most of the cavities are formed in the upper thoracic and lower cervical parts of the spinal cord. It is extremely rare that they form in the lumbosacral and thoracic regions.

The development of acquired syringomyelia is promoted by spinal cord injuries.

Symptoms

The most dangerous thing is that in the early stages syringomyelia manifests itself unnoticed; symptoms appear much later. Some of the first symptoms are weakness in the hands, severe weight loss, and a decrease in temperature and pain sensitivity in the hands.

At this time, a patient with syringomyelia can inflict numerous burns and cuts on himself, as he cannot react in time and withdraw his hand. The skin of such patients is often bluish, strewn with boils and ulcers. Panaritiums may develop on the fingers.

Over time, sensory loss spreads to other areas of the body. A common occurrence is where areas of sensitive skin may alternate with areas of normal skin.

Another characteristic symptom of this disease is shooting or burning pain, which can occur in any part of the body. Also in patients with syringomyelia It is not uncommon for the spine to be curved.

Syringomyelia in later stages leads to the development of Horner's symptom - drooping of one eyelid and constriction of the pupil, resulting from paralysis of the cervical spine.

Second stage of syringomyelia - decreased muscle tone, their weakness and decrease in volume. It becomes difficult for the patient to move. Paresis of the arms develops first, followed by the legs. Syringomyelia can also affect sweating - it may decrease or disappear altogether, or significantly increase after eating spicy food.

As the disease progresses, calcium is washed out of the patient's bones and they break easily. Due to decreased sensitivity, patients do not feel pain during a fracture.

A neurologist can diagnose syringomyelia. The diagnosis is made on the basis of MRI or CT. The disease syringomyelia is easily diagnosed; the photo perfectly illustrates the processes occurring in the spinal cord.

Treatment

It is impossible to completely cure syringomyelia. The development of medicine at the current stage makes it possible to eliminate its manifestations and stop its development.

If you start treating the disease in time, you can live long and happily with it. To do this, you need to follow some recommendations - eat right and lead a healthy lifestyle.

Patients with syringomyelia need to consume fermented milk products as much as possible - they will help replenish calcium deficiency in the body. You also need to eat as many foods rich in vitamin A as possible - cabbage, spinach, carrots and beets.

X-ray therapy has a beneficial effect on syringomyelia. In this case, the affected organ or part of the body is irradiated with X-rays and the development of the disease is delayed.

The result of this treatment is that the patient’s pain decreases or disappears altogether, discomfort in the hands disappears, and the area of ​​insensitive areas decreases. This treatment is effective only in the early stages of the disease.

No less effective is proserine therapy, which can eliminate certain symptoms. Proserin helps improve the transmission of impulses between cells and is effective only in the initial stages of the disease.

Delay pathological glial growth Radioactive iodine and phosphorus also help.

Patients suffering from syringomyelia are also indicated for sanatorium-resort treatment and radon baths.

Based on many years of experience, it has been established that in the case of syringomyelia, when symptoms are pronounced, surgical treatment can also be effective. Surgery often preserves the functionality of the spinal cord and prevents damage to it.

For syringomyelia, drug treatment is also used, but it does not affect the cyst formed in the spinal canal. The main effect of this therapy is a reduction in swelling around the spinal cord.

Prevention

The main measures to prevent syringomyelia include timely diagnosis of the disease.

In order to prevent the development of this disease, spinal injuries should be prevented, and if they occur, they should be treated as effectively as possible.

Secondary prevention can be aimed at preventing the progression of the disease, preventing the occurrence of injuries, burns and infections.

If left untreated, syringomyelia can lead to serious complications. So, the most common complications include:

• bronchopneumonia;
• urethritis;
• sepsis;
• pyelonephritis;
• stopping breathing.

In this video, experts clearly explain everything about syringomyelia.

Patients with syringomyelia can live a full life and continue to work. Given the increased fragility of bones, you should not choose a job associated with heavy physical activity and injury.

Given the reduced sensitivity of the skin of patients with syringomyelia, they should always dress appropriately for the weather to avoid overheating or hypothermia.

Don't be scared when you hear a diagnosis like this. If you don’t give up and start timely treatment, you can get rid of most of the symptoms.

Syringomyelia is a disease of the nervous system, the anatomical essence of which is the formation of cavities in the spinal cord. In cases where the cavities are not limited to the spinal cord, but also extend to the brain stem, they speak of syringobulbia. Sometimes these cavities communicate with the central canal, being, as it were, its expansion, which gives the picture of hydromyelia.

Pathological anatomy and pathogenesis

In most cases, cavities are localized in the lower cervical segments and upper thoracic segments. Upon external examination of the spinal cord, covered with membranes, it may appear completely normal. More often, mildly expressed phenomena of adhesive arachnoiditis and hypertrophic pachymeningitis are found. After opening the membranes, the spinal cord in the area of ​​the cervical thickening appears swollen and fluctuating. The sections reveal a cavity of larger or smaller size, which has an irregular, often slit-like shape.

Causes of syringomyelia

The main reason is a defect in embryonic development, namely a delay in the formation of the posterior suture in the area of ​​closure of the halves of the brain tube. This disrafi” (from the Latin raphe - seam) is accompanied by improper formation of glia, the cells of which retain the ability to grow.

In the presence of the indicated endogenous prerequisites - dysraphism and glia delayed in their development - some external causes (especially often trauma) can cause syringomyelia. In most cases, the disease develops without any apparent external cause.

People suffering from syringomyelia usually have more or less signs of the so-called dysraphic condition - status dysraphicus. These include kyphoscoliosis, funnel chest, asymmetrical skull, acromegaloid features of the facial skeleton, irregular foot shape, nipple anomalies (irregular location, hypermastia), disproportionately long limbs, extra ribs, spina bifida and some others.

The dysraphic status itself is not a disease. This is only a constitutional type, predisposed to certain forms of nervous system suffering, in particular syringomyelia.

Much controversy has arisen about the possibility of syringomyelia as a result of former hemorrhage in the spinal cord, acute or chronic myelitis, or softening of the spinal cord due to vascular disorders. Currently, it can be considered generally accepted that all of these sufferings can lead to the formation of cavities, but do not give rise to true syringomyelia.

Gardner put forward a "hydrodynamic theory" of the origin of syringomyelia. In the embryo, the central canal of the spinal cord and the ventricular system form a single closed system. Later, after the formation of the foramen of Magendie, the ventricular system begins to communicate with the subarachnoid space, and the central canal is obliterated. In the case of stenosis of the foramen of Magendie, the cerebrospinal fluid, according to Gardner's hypothesis, shifts towards the central canal with each cardiac systole and after a few years leads to its expansion. In this case, with the greatest regularity, the hydromyelitic cavity naturally forms in the cervical and upper thoracic region. This permanent fluid pressure can lead to rupture of the ependymal canal and the formation of syringomyelitic cavities near the central canal. Stenosis of the foramen of Magendie may be caused by a malformation or mechanical obstruction. Gardner noted a narrowing of the foramen of Magendie in all 74 cases of syringomyelia he observed; in 92% of cases, the stenosis was associated with an abnormal downward displacement of the cerebellar tonsils (Arnold-Chiari syndrome). The hydrodynamic theory suggests that the syringomyelitic volosts are in direct connection with the central canal. Indeed, isotopes introduced into the syringomyelitic cavity are found in the brain cisterns within a few hours. The liquid obtained by aspiration from the syringomyelitic cavity exhibits all the characteristic properties of cerebrospinal fluid.

This theory opens up certain therapeutic prospects: by cutting adhesions in the area of ​​the Magendie foramen, stop the progression of the disease.

Syringomyelia symptoms

The clinical manifestations of syringomyelia can be easily deduced from the anatomical essence of suffering. Hence the triad characteristic of syringomyelia:

• atrophic paresis of the hands
• loss of temperature and pain sensitivity in the arms and upper body
• trophic and vasomotor disorders of the arms and upper body

In the further course of the disease, proliferating glia, or a growing cavity, causes disruption of the spinal cord. Then segmental disorders are complicated by conduction symptoms in areas of the body located below the source of the disease. The triad characteristic of syringomyelia includes leg paresis, leg anesthesia, pelvic disorders, pathological reflexes, and absence of abdominal reflexes.

In the sensitive area, a symptom pathognomonic for syringomyelia is dissociated anesthesia: the absence of temperature and pain sensitivity while maintaining muscle-articular and tactile sensitivity. Sometimes sensitivity in the hands remains (at a lower localization of the process), dissociated anesthesia captures only the skin of the upper body in the form of a “vest”. As the disease progresses, the hypoesthesia zone gradually moves down the body. Acute pain is not typical for syringomyelia; Dull pains occur frequently, they are localized in the right or left arm, shoulder girdle or side and can be very intense, even painful. Patients often complain of paresthesia, a feeling of numbness, tightness, burning, coldness in the area of ​​the body corresponding to the affected segments of the spinal cord. Involvement of the spinal cord columns in the process causes conduction sensitivity disorders.

Syringomyelia symptoms from the motor sphere appear later, when the process moves to the anterior horns. Syringomyelia is characterized by atrophic paresis of the upper extremities. Atrophy most often begins with the small muscles of the hand, innervated by the VIII cervical and I thoracic segments: the interosseous spaces sink. The hand takes the form of a claw-shaped paw. Less commonly, atrophy begins in the shoulder girdle and back muscles. Unlike other forms of chronic damage to the anterior horns, atrophy in syringomyelia is rarely accompanied by fasciculations. Reflexes from the hands decrease, as with any peripheral paresis. It is especially characteristic of syringomyelia that not all reflexes are reduced or extinguished at the same time: on a paretic limb with atrophic muscles, one reflex is reduced or even completely absent, and the other is increased. In the lower extremities, an increase in tendon reflexes, the appearance of clonus and pathological reflexes are noted. Abdominal reflexes, especially the upper ones, are often upset already in the initial stages of the disease. In advanced cases, abdominal reflexes are usually lost. Pelvic disorders are rarely very deep.

Vegetative-trophic disorders occupy a prominent place in the clinical picture of syringomyelia. Cyanosis and sweating are often detected. Unilateral hyperhidrosis is especially suspicious of syringomyelia. Dull or painful (acute) irritation of the skin of the chest and back causes the appearance of red spots of dermographism, which last a very long time. Edema (urticarial) dermographism often appears. Sometimes vasomotor disorders resemble the picture of erythromelalgia or. Horner's syndrome is often diagnosed. Painless felons are extremely characteristic.

Trophic changes in joints and bones are often detected. Kyphoscoliosis of the thoracic spine is especially characteristic. Sometimes enlargement of the bones and hyperplasia of the soft tissues of the hand occur, leading to cheiromegaly (large hand). Osteoporosis is usually found in the bones of the upper extremities, so fractures are possible under the influence of a relatively minor injury. Approximately 20% of patients have neurodystrophic osteoarthropathy (“Charcot’s joint”). Most often the elbow is affected, less often the shoulder and other joints of the arm. The affected joint is usually sharply increased in size, and when moving, a rough crunching sound of articular processes of bones and sequestra rubbing against each other is often heard. The X-ray picture is characterized by “a combination of deep destruction with excessive creation, a combination of atrophic and hypertrophic processes.” As a rule, these gross changes are painless.

Cerebrospinal fluid in most cases of syringomyelia is unchanged. Sometimes only a mild protein-cell dissociation is detected, caused by a partial blockade of the subarachnoid space by a thickened area of ​​the spinal cord.

Pneumoencephalographic examination of patients with syringomyelia almost always reveals chronic asymmetric open internal hydrocephalus, as well as moderate external hydrocephalus. Obviously, syringomyelia in all cases is not a purely spinal disease, but a suffering of the entire central nervous system. This view is also supported by the fact that most patients have varying degrees of organic personality decline: pronounced concreteness of thinking associated with the difficulty of forming abstract concepts, limited general horizons, difficulty in counting, weakened memory, underestimation of the severity of their illness.

Atypical forms of syringomyelia occur when the process is unusually localized. These include:

• lumbosacral form;
• dorso-lumbar form;
• syringomyelia, which is associated with two lesions;
• Syringobulbia.

The lumbosacral form of syringomyelia is characterized by localization of disorders not in the arms, but in the legs and lower torso. This form is very rare.

In the dorso-lumbar form, dissociated anesthesia in the thoracic segments, pelvic disorders, spastic lower paraparesis, and vegetative-trophic disorders in the lower extremities, characteristic of syringomyelia, are observed.

Sometimes there are cases of syringomyelia in which the clinical symptoms depend on the localization of the process in two areas of the spinal cord, located nearby or far from one another. The classic cervicothoracic type described in detail above can be complicated by the lumbosacral form, or syringobulbia. This combination occurs quite often.

Syringobulbia has greater practical significance and better defined clinical characteristics than other atypical forms. Pathohistological changes characteristic of syringomyelia are localized in syringobulbia in the medulla oblongata.

The symptomatology of syringobulbia consists mainly of bulbar disorders: voice, speech, swallowing are impaired, and tongue atrophy appears. Rarely, stridor is observed due to laryngeal paralysis and may require tracheostomy. A segmental type of facial sensitivity disorder is very typical; hypoesthesia is more pronounced in the lateral parts of the face; Sensitivity is noticeably better near the mouth and nose. Sensory impairment usually has the character of dissociated hypoesthesia. Hypoesthesia (or anesthesia) of the cornea is noted. Nystagmus is detected (often rotatory). Horizontal nystagmus in syringomyelia is associated with damage to the upper cervical segments, in particular the fasciculus longitudinalis medialis.

Sometimes there is hearing loss and dizziness due to damage to the nuclei of the VIII nerve. At high localizations, the facial nerve is involved in the process.

Course and prognosis of syringomyelia

It is possible that the first symptoms of the disease occur in children, but they are not paid attention to. Syringomyelia is diagnosed at the age of 20-40 years. It gradually develops, partial remissions are possible. Occasionally, sudden deterioration is observed, up to the development of a transverse lesion of the spinal cord, caused by hemorrhage into the syringomyelitic cavity. The prognosis for life is favorable, for recovery - poor. Patients usually remain able to work for a long time, adapting to the changes caused by the disease. Death occurs more often from intercurrent diseases, complications (sepsis), and very rarely from bulbar phenomena.

Diagnosis of syringomyelia

Recognizing typical cases of syringomyelia does not present much difficulty. The diagnosis is made mainly based on the presence of a characteristic set of symptoms in the patient:

• atrophic paresis of the arms, combined with spastic phenomena in the legs;
• dissociated anesthesia in the form of a “jacket” or “half-jacket”;
• significant vegetative-trophic disorders in the hands;
• kyphoscoliosis;
• Horner's syndrome;
• chronically progressive course of the process, not accompanied by acute pain.

There are cases of syringomyelia that are extremely difficult to diagnose. A lumbar puncture and a carefully collected medical history can provide significant assistance in such cases. Sometimes the diagnosis can be confidently made only after more or less long-term observation. Syringomyelia must be differentiated from hematomyelia, intramedullary tumor, brachial plexus neuritis, leprosy, amyotrophic lateral sclerosis, neurological complications of cranio-vertebral anomalies, sensory neuropathies and congenital insensitivity to pain.

With hematomyelia, all phenomena develop in a stroke-like manner, acutely, following an injury, and the general condition of the patient is noticeably disturbed. The course of the disease is regressive.

An intramedullary tumor (most often a glioma) can clinically manifest itself as symptoms of syringomyelia.

Spinal tumors are characterized by a faster progression, the presence of signs of spinal cord compression, and characteristic changes in the cerebrospinal fluid. With syringomyelia, the process occupies a greater extent along the length of the spinal cord than with a tumor. In the most difficult cases, a differential diagnosis is made only during surgery for the tumor.

Syringomyelia is similar to brachial plexus neuritis by the presence of weakness and atrophy of the muscles of the upper extremities, sensory and trophic disorders in the area of ​​the body innervated by the lower cervical and upper thoracic segments. Dissociated hypoesthesia on the trunk or face, pyramidal signs, pelvic disorders, changes in abdominal reflexes, and kyphoscoliosis speak against neuritis and in favor of syringomyelia. The progressive course of the disease is also not typical for brachial plexus neuritis.

The nervous form of leprosy (lepranervosas, Lepra maculoanaesthetica) causes paresis of the arms, atrophy of the small muscles of the hand, anesthesia, and trophic disorders, as does syringomyelia. Differential diagnosis of these two forms can sometimes present great (in rare cases insurmountable) difficulties. It should be borne in mind that the nervous form of leprosy is much less common than syringomyelia, and that leprosy is associated with certain localities. Segmental anesthesia characteristic of syringomyelia does not occur in leprosy, which is characterized by spotty anesthesia or neuritic sensitivity disorders. Spastic paresis and pathological reflexes usually do not occur in leprosy, since the disease is based on chronic interstitial polyneuritis. Pigment spots of various shapes, within which hypoesthesia, loss of eyebrows, thickening of peripheral nerves (especially often nn. ulnaris, auricularis major, peroneus communis) are noted, are characteristic of leprosy.

In doubtful cases, it is necessary to search for Hansen's rods in the nasal mucus and examine the patient by a leprologist.

Amyotrophic lateral sclerosis differs from syringomyelia in the absence of sensory disorders, changes in the skin and its appendages, and kyphoscoliosis.

Cases of progressive or stationary syringomyelia caused by congenital anomalies in the foramen magnum region have attracted much attention in recent years. Most often we are talking about Arnold-Chiari anomaly (displacement of the cerebellar tonsils into the spinal canal, sometimes combined with assimilation of the atlas) and basilar impression (funnel-shaped depression into the cranial cavity of the clivus of the occipital bone and the occipitovertebral joint).

Anomalies of the bone and nervous structures of the cranio-vertebral junction area are accompanied by the development of syringomyelitic syndrome, both due to ischemic necrosis caused by compression of the spinal vessels in the area of ​​the foramen magnum, and due to the progressive expansion of the central canal. The assumption of a cranio-vertebral anomaly is especially justified in cases of damage to the upper cervical segments and the brain stem. However, segmental defects in such secondary syringomyelitic syndrome can extend to the lower cervical region.

Crucial in recognizing anomalies of the cranio-vertebral region are a thorough x-ray examination (assimilation of the atlas with the occipital bone, location of the epistrophy tooth in the foramen magnum) and especially myelography (detection of a characteristic shadow of the cerebellar tonsils in the spinal canal during pneumomyelography). A significant increase in protein content in the cerebrospinal fluid may indicate the compression genesis of syringomyelia syndrome.

Severe defects in pain sensitivity are also observed in very rare forms of sensory neuropathies caused by degeneration of dorsal root fibers and congenital insensitivity to pain. Unlike syringomyelia, in such patients anesthesia is not strictly segmental in nature, and trophic disorders reach an unusually pronounced degree, including acroosteolysis and mutilation of the terminal phalanges of the fingers.

Treatment of syringomyelia

In the early stages of the disease, X-ray therapy has a certain effect, but the improvement achieved is usually unstable and the disease continues to progress slowly. In the presence of advanced changes, irradiation does not bring relief at all. In recent years, assessment of the effectiveness of radiotherapy for syringomyelia has become increasingly restrained, and, according to some authors, even doubtful. It is very important to protect your hands from damage (burns, frostbite, injuries). Symptomatic treatment is carried out according to the general rules of rehabilitation therapy; You just need to avoid thermal procedures to avoid burns. In rare cases of intense and persistent pain and paresthesia, stereotactic intervention on the thalamic nuclei may be necessary. In cases of progressive damage to the diameter of the spinal cord, as well as the brain stem (syringoencephalomyelia), a contrast study is indicated, and in the case of significant thickening of the brain, surgical emptying of the cystic cavity (Pussepp operation) is described. Observations are described when the condition of patients with syringomyelia significantly improved after decompression of the posterior cranial fossa with separation of adhesions in the area of ​​the Magendie foramen. It is possible that this is a consequence of the injury that caused the late onset of syringomyelia.

The article was prepared and edited by: surgeon

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 rightfully be called the scourge of modern man. The appearance of some of them is caused by an incorrect lifestyle, while others arise after infection. The latter group also 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: At the 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 extremities. 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 in the 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.

The use of radioactive iodine or phosphorus has a significant effect on the formation of cavities. These elements are capable of inhibiting the growth of glial cells.

In the later stages of the disease, surgical intervention is prescribed. 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.