Syringomyelia: symptoms and treatment

Syringomyelia is a chronic, slowly progressing disease of the nervous system that occurs in the result of a disruption in the laying of the neural tube during embryogenesis (during fetal formation in the womb mother). In this pathology in the substance of the spinal cord, the areas of proliferation of connective tissue (glia) are formed, which then break up with the formation of cavities. Cavities are filled with cerebrospinal fluid and tend to grow in all directions. It turns out that instead of a normal nerve tissue, voids with a liquid form. Accordingly, the functions of those parts of the spinal cord that are replaced or squashed by the cavities are lost. This determines the clinical symptoms of syringomyelia. Treatment of syringomyelia is a rather difficult task. They use conservative and operational methods, however, it is impossible to achieve complete recovery from the disease. This article will inform you about the main symptoms and methods of treatment for syringomyelia.

General information

Syringomyelia has several more names: Morvan's disease, spinal gliosis. In those cases where cavities are formed not only in the substance of the spinal cord, but also in the region of the trunk (medulla oblongata), the disease is called syringobulbia (bulbus - in the translation "bulb the obsolete name of the medulla oblongata because of its shape).

Earlier it was assumed that syringomyelia occurs solely as a result of intrauterine disorders. However, to date, it has been possible to find out that similar cavities can also occur in as a result of tumors of the large occipital foramen, traumas of the spinal cord and brain, inflammatory processes. And, although such situations are much less common than the inherent changes, at the present time the term "syringomyelia" is no longer used to designate a single disease, but the whole group. It turns out that syringomyelia is a syndrome of the formation of specific cavities in the spinal cord due to a number of reasons. Accordingly, it is customary to isolate true syringomyelia (with congenital causes) and secondary (when exposed to external factors).

Quite often, syringomyelia is not the only violation of embryogenesis. About 80% of cases of syringomyelia are combined with the Arnold-Chiari anomaly. Also possible simultaneous existence of syringomyelia and Dandy-Walker anomaly, basilar insufficiency, signs of dysraphic status (unusual form head, forked chin, high (gothic) palate, bifurcation of the tip of the tongue and lower lip, abnormal teeth growth, irregular bite, unequal the size of the eyeballs, the "depressed" facial skull, the dysplasia of the auricles, the funnel-shaped or protruding chest, scoliosis, kyphosis, kyphoscoliosis, additional nipples, different sizes of mammary glands, splitting and deformation of vertebral arches, additional ribs, additional fingers, irregular structure brushes and feet).

Why does syringomyelia occur?

Congenital defects (malnutrition during pregnancy, alcoholism, drug addiction, toxoplasmosis, radiation exposure, chronic infections and somatic metabolic diseases in pregnant women, etc.) or the impact of external factors (trauma, tumors, myelitis and arachnoiditis, stenosis of the cervical spine, etc.) trigger an excessive process of reproduction of glial cells in the brain. At first, the multiplying cells form knots, but then they die, and in the place of their decay cavities are formed. Cavities are gradually filled with cerebrospinal fluid (CSF). The liquid presses against the walls of the cavity, causing it to expand, and thus causes compression and death of nearby nerve cells. As the process progresses, the cavities grow, and more and more new neurons die.

Science knows two types of cavities in syringomyelia:

• communicating (when the syringomyelitis cavity is connected to the central canal of the spinal cord);
• non-communicating (cavities exist in isolation and are not connected with the cerebrospinal fluid pathways of the spinal cord).

Symptoms of syringomyelia

According to different data, the incidence of syringomyelia is from, to, the case per 100 thousand population. The congenital form of the disease affects the male sex 2 times more often than the female. Since the cavity begins to increase in adolescence, it is at this time and the first symptoms appear. Although quite often the diagnosis of the disease is delayed for dozens of years because of the multitude of diverse and non-simultaneous symptoms.

Usually, the disease develops slowly, gradually accumulating symptoms, which is associated with a gradual increase in the size of the cavities. Sometimes patients connect the onset of the disease with a cough or sneeze (for example, they say that after a cough there was weakness in the hand).

Since the cavities with syringomyelia grow in all directions, it is customary to isolate several forms along the length and width of the spinal cord.

By length:

• cervicothoracic form: with the location of cavities in the cervical and thoracic parts of the spinal cord;
• lumbosacral: cavities are formed in the lumbar and sacral parts of the spinal cord. This form is very rare and is considered atypical;
• bulbar: when the cavity is localized in the medulla oblongata (syringobulbia);
• mixed: in the presence of cavities in several departments simultaneously.

By diameter:

• sensitive: when compressing sensitive spinal cord conductors;
• motor: defeat of motor neurons and pathways;
• trophic: when the lateral horns of the spinal cord are compressed;
• mixed: it is usually a consequence of the existing great "experience" of the disease.

This division has a clinical basis, because, for example, with the location of the cavity in the cervico-thoracic region of the spinal cord and compression of sensitive conductors, the first symptoms are the loss of pain and temperature sensitivity in the hands, and when the cavity is localized in the same department, but compression of motor neurons, the first appear muscular weakness in the upper limbs with complete safety sensitivity.

In the initial period of the disease, a sensitive form is more common. The main signs in this case are pain, paresthesia and sensitivity disorders.

Pain occurs in the cervico-brachial region (with cervical-thoracic form), blunt, aching, may have a burning tinge, appear as seizures, or be permanent. In the same zones, there are unpleasant feelings of crawling, tingling (that is, paresthesia). Changes in sensitivity are represented by loss of pain and temperature sensitivity in the so-called "jacket" zone (with bilateral damage) or "half-jacket" (one-sided process), that is, in the arms and an adjacent part of the trunk, while maintaining that in the abdomen, pelvis and lower extremities. This loss of sensitivity is considered specific for syringomyelia. It causes the appearance of scars from burns and various injuries (since the pain sensation does not arise from hot or traumatic effects).

With syringomyelia, it does not necessarily mean the simultaneous occurrence of all sensitive manifestations. A person can seek medical help only because of frequent burns, but with no pain at all.

Another feature of the clinical course of syringomyelia is the dissociated nature of sensitive disorders. What does this mean? This means a change in pain and temperature sensitivity with the preservation of other species (the so-called deep species sensitivity, allowing a person to determine the position of the limb in space, guess the place of touch with closed eyes). This feature is revealed only at the initial stages of the disease, because then the cavity is squeezed only by the posterior horns of the spinal cord, which are responsible for pain and temperature sensitivity. With the growth of the cavities, the conductors of deep sensitivity (the rear cord) are also involved in the process, and then all kinds of sensations are already violated.

If the cavity is initially located in the lumbosacral spinal cord, then the changes described above occur in the legs.

The motor form arises, as the debut of the disease, much less often. It is connected with the compression of the front horns. As a result, in the arms (legs in the lumbosacral form) there are involuntary jerking of the muscles, the strength and muscle tone decrease, and tendon reflexes are lost. A few weeks later, atrophy of the muscles of the upper (lower) extremities is revealed: weight loss and thinning. When the cavities begin to squeeze not only the front horns but also the front and side cords, then there is a muscle weakness in the lower extremities (with cervicothoracic form and initial weakness in the hands) with an increase in their tone, the appearance of pathological reflexes, an increase in normal tendon reflexes (knee, Achilles). The compression of the anterior and lateral cord is also accompanied by a disruption of the function of the pelvic organs (there are episodes of urinary incontinence, imperative urge to urinate, and then problems with restraint of stool).

The trophic form is associated with compression of the lateral horns of the spinal cord, where neurons of the autonomic nervous system are located. Clinically, this manifests itself (with cervicothoracic form) by thickening, dryness and roughness of the skin, blue fingertips and brushes, brittle nails, frequent panaritium, increased density of hair, pigmentation of individual skin areas, increased sweating (especially when taking hot or hot food). Wounds on the hands do not heal well, they become infected again and lead to the development of phlegmon, fistula, osteomyelitis. The joints of the upper limb are deformed, and sometimes to an incredible size. In this case, the movements in the affected joint can be completely painless (due to the defeat of sensitive conductors), although a strong crunch of rubbing bones is heard. Bones also undergo destruction, osteoporosis develops. This often leads to fractures. It turns out that joints and bones seem to melt, and in their place cavities are formed, covered with skin.

With the cervico-thoracic localization of the syringomyelitis cavity, the occurrence of Horner's syndrome is possible. It includes ptosis (descent of the upper eyelid), miosis (narrowing of the pupil) and enophthalmus (eyeball inward). The syndrome is caused by compression of autonomic nervous conductors.

Syringobulbia is characterized by the defeat of the cranial nerves: sublingual, wandering, lingopharyngeal, facial and sometimes trigeminal. This has the following clinical manifestations:

• impaired mobility of the tongue, its hypotrophy;
• blurred speech;
• nasal shade of voice;
• choking at meals;
• hoarseness of voice up to aphonia;
• violation of taste in the language;
• loss of sensation and muscle atrophy in the face;
• hanging of the soft palate;
• violation of breathing.

In addition, syringobulbia is manifested by disorders of coordination, shaky gait, dizziness, nystagmus (involuntary trembling movements of the eyes), hearing impairment.

Whatever form the disease has started, as it progresses, the stage begins when the patient has both sensitive and motor and vegetative-trophic disorders. Syringomyelia is characterized by a fairly slow progression, for dozens of years, although there are also more rapidly progressing forms. Sometimes there are periods of relative stabilization of the condition, then new symptoms do not appear (relative remission).

Diagnostics

The disease is relatively easy to diagnose in the expanded clinical stage, when the face sensitive, motor and vegetative-trophic disorders. The first symptoms are nonspecific and do not always suggest a possible syringomyelia.

With the advent of magnetic resonance imaging, the diagnosis of syringomyelia was significantly simplified (at any stage). This method can reliably confirm or deny this diagnosis. Magnetic resonance imaging creates an opportunity, in the literal sense of the word, to see cavities in the spinal cord. That is why all patients with suspected syringomyelia are sent to the MRI of the spinal cord.

Treatment

Syringomyelia still belongs to incurable diseases. All the methods available in the arsenal of medicine allow us to somewhat slow down the process, stabilize the symptoms, facilitate the life of the patient, but do not save from it forever.

The choice of a method of treatment is influenced by many aspects: the true it is syringomyelia or a secondary, initial or developed stage of the disease, the cavities with subarachnoid space or not, how sensitive are the sensory, motor and vegetative-trophic changes that are associated disease.

All patients with syringomyelia should carefully avoid injuries to the affected limbs, and carefully handle the fire. Lack of sensitivity can play a cruel joke: for example, a person in time will not withdraw his hand from the fire or feel a cut on the skin. And the resulting injuries are very poorly healed because of trophic disorders and can cause the spread of the infection.

In the initial stages of syringomyelia, when glial cells are actively multiplying, X-ray therapy is possible. The affected parts of the spinal cord are irradiated. Take several courses with a break from 6 months to several years. The purpose of such treatment is to stop the multiplication of cells, and hence the prevention of subsequent cavity formation. However, recently the effectiveness of X-ray therapy has become increasingly questioned. This is due to the fact that after a while the disease starts to progress anyway, and the rays do not allow to eliminate the already existing symptoms.

Drug treatment is also conducted by courses 2-3 times a year. To this end, use:

• painkillers. It can be analgesics (Analgin, Ketanov), non-steroidal anti-inflammatory drugs (Diclofenac, Ibuprofen) and their combination. In cases of very strong and poorly arresting pain, anticonvulsants are used (Gabapentin, Pregabalin), antidepressants (Amitriptyline, Duloxetine and others);
• drugs that improve blood circulation and metabolism in the nervous tissue (Pentoxifylline, Actovegin, Pyracetam, Glutamic acid, Nicotinic acid and others);
• vitamins of group B (Milgamma, Complivit, Neurovitan, Neurorubin) and vitamin C, folic acid;
• means for dehydration. They reduce the amount of CSF (Furosemide, Diacarb);
• anticholinesterase drugs that contribute to the improvement of neuromuscular conduction (Prozerin, Neurromidine).

The essence of such treatment lies in the generally restorative, rehabilitative effect, increasing the adaptive capacity of the organism. It can not affect the process of cavity growth.

From physiotherapy methods are used radon baths, UHF, electrophoresis. Some patients are well helped in the fight against pain syndrome acupuncture.

In addition to conservative methods of treating syringomyelia, there are also operational ones. The main indications for neurosurgical intervention are:

• rapidly progressing forms of the disease;
• increased disturbances of liquor circulation;
• combination of cavities in the dorsal and medulla oblongata with abnormalities of the craniovertebral transition (Arnold-Chiari anomaly and others).

The operations are carried out in various volumes: it can be the drainage of the formed cavities, shunting (with withdrawal cerebrospinal fluid into the abdominal cavity), dissection of adhesions compressing the spinal cord, decompression of the craniovertebral transition. Surgical treatment contributes to some improvement in the condition, slowing the progression of the disease for a while, but, unfortunately, does not lead to recovery of the patient.

Another method of surgical treatment of syringomyelia is dissection of the terminal filament in the lower part of the spinal canal. This method is based on the hypothesis of one scientist, which is that syringomyelia probably occurs in The result of insufficient blood supply to the spinal cord is closer to the central canal due to brain strain during abnormal end thread. Dissection of the thread in the coccyx region weakens the tension of the spinal cord, leads to the restoration of normal blood flow. The effectiveness of this method is being studied.

Outcome of syringomyelia

Although it is impossible to get rid of syringomyelia, it is believed that it does not lead to a decrease in life expectancy.

True syringomyelia slowly progresses, allowing the patient to remain working and maintain himself for a long time, but late stages of the disease lead to disability.

The course of secondary syringomyelia is usually more malignant, but in this case the very cause is played by the cause of syringomyelia. The emergence of respiratory disorders, swallowing, urination is fraught with the emergence of infectious complications (pneumonia, sepsis), which already carries a risk to life. Syringobulbia has a worse prognosis for life, since in its case respiratory disorders occur relatively quickly.

So, summing up the above, we can say that syringomyelia is severe neurological disease, which manifests itself as a multitude of symptoms and therefore not always is diagnosed immediately. Today's realities are such that until there are no ways to completely cure of syringomyelia.

Neurosurgeon, Cand. M. Zuev A. A. tells about syringomyelia:

Presentation on the topic "My diagnosis is syringomyelia. What should I know about my illness?