Features of the pathology
Syringomyelia according to ICD-10 is assigned code G95.0. The exact causes of syringomyelia have not yet been reliably studied. Pathology can appear in a number of diseases, such as oncological diseases of the spinal cord or its injuries.
The pathology is characterized by the formation of cavities in the spinal cord. This occurs due to the rapid proliferation and further breakdown of glial tissue, as a result of which the dynamics of the cerebrospinal fluid is disrupted.
The disease can occur as a result of a congenital abnormality of the spinal cord and skeleton. Often the cause of the pathology is the improper development of the nervous system in the prenatal period. Syringomyelia can also be caused genetically. The disease is often diagnosed among the population of regions with low migration rates.
Another likely reason for the appearance of spinal cord irregularities is a lack of nutrients necessary for the formation of the nervous system during the prenatal period.
Due to the structure of the skeleton, syringomyelia of the neck or lumbar region is most common. This is due to the peculiarities of the dynamics of cerebrospinal fluid in these areas of the spine.
Syringomyelia is a chronic disease of the nervous system; it is not possible to cure the pathology. Prognosis, supportive therapy and symptoms depend on the form of the disease.
Syringobulbia is a similar disease that affects other parts of the spinal canal. Syringomyelia and syringobulbia are classified equally according to ICD 10.
Prevention
You can prevent serious complications if you treat your body with care and try to avoid infection with infections of various natures. The latter can aggravate the course of a chronic disease, provoking the most severe consequences.
A healthy lifestyle, proper and nutritious nutrition, and regular visits to a neurologist can prevent the accelerated development of syringomyelia.
Attention. There should be no self-medication before diagnosis, and certainly not after that.
How does syringomyelia develop?
A pathology caused by a congenital disorder of glial tissue is called true syringomyelia. In this case, there is a tendency to increased growth of cells in the glial tissue of the cerebrospinal fluid of the cervical and lumbar spine. In order for glial tissue to grow, it is necessary to be exposed to some provoking factor - this could be an infectious disease or injury to the spinal canal.
The rapid proliferation of cells leads to the fact that over time they die, forming cavities. Fluid accumulates in the cavities, which is why the disease is often called cystic formation of the spinal cord. Due to the accumulation of fluid, the cavity increases in size, which leads to irritation of adjacent nerve cells. As a result, nerve cells are compressed, degenerate and die.
The more the disease progresses, the more cavities are formed. As a result, the number of dying nerve cells increases.
True syringomyelia is accompanied by congenital anomalies of skeletal development. Patients often experience severe scoliosis, kyphosis, increased arm length, abnormal development of fingers (six-fingered), skull asymmetry, and bifurcation of the tongue. This form of the disease is familial in nature, that is, it is observed in several generations of the same family. Most often, true syringomyelia occurs in men aged 20-50 years.
True syringomyelia is a rare disease that occurs in no more than 30% of cases. The most common form of the disease is associated with an abnormal development of the craniovertebral junction - that is, the area where the spine connects to the skull. This is a congenital structural anomaly that provokes expansion of the spinal canal. Due to this expansion, the gray matter filling this area is gradually destroyed, causing the development of syringomyelia.
Pathological anatomy
Rice.
1. Microscopic specimen of the spinal cord for syringomyelia (cross section): 1 - pathological cavity; 2 — gliomatous pin; x 10. Macroscopic examination of the spinal cord reveals thickening of the dura and arachnoid membranes, their fusion in certain areas with each other and with the soft membrane, flattening, thickening or thinning of individual or all of its segments. In certain expanded segments of the spinal cord, compactions and fluctuations are also detected. Characteristic for S. single or multiple patol. cavities (Fig. 1) are localized mainly in the central sections of the spinal cord segments, sometimes in the brain stem; the white commissure is, as a rule, preserved. The cavities in most cases are not associated with the central canal, but extend to the posterior columns and central sections of the posterior cords; they are also found in the anterior funiculus. Their configuration is varied: round, oval, slit-shaped, hourglass-shaped, with one or more diverticula. The sizes of the cavities also vary: from narrow, detectable only when pressing on the lateral, anterior or posterior surfaces of the spinal cord, to large, occupying almost the entire diameter of the segment. Patol. the cavities can be so large that the spinal cord takes the form of a thin-walled tube and easily ruptures when it is carelessly removed from the hard shell. Most often, the cavities extend to 1-2, less often - to a larger number of segments. An observation is described in which the cavities extended from the Ssh segment to the LIT. In isolated cases, the cavities reach the medulla oblongata and the pons (pons of the brain, T.). The pons, as a rule, is the boundary of their distribution in the oral direction, however, there is a known observation when the slit-like cavity extended from the sacral segments through all parts of the spinal cord, the medulla oblongata, the right half of the pons, the right cerebral peduncle, the internal capsule, the caudate nucleus and ended at the anterior horn of the lateral ventricle of the brain.
In Syringomyelia, a combination of the described changes is often observed with atresia or narrowing of the median and lateral apertures of the fourth ventricle of the brain, expansion of the ventricles of the brain and the central canal of the spinal cord, as well as with malformations characteristic of dysraphic status, with neuroectodermal tumors (most often with spongioblastomas and ependymomas ) and angiomas.
On microscopic examination in the walls of the patol. cavities, glial fibers are found, in some cases - groups of ependymocytes (as a rule, when the cavity communicates with the central canal). Around the pathol. cavities, near them or at a distance from them focal accumulations of astrocytes, mostly fibrous, are visible, among which there are also a few protoplasmic ones. It is these accumulations of fibrous astrocytes, reaching significant sizes, that are so-called. gliomatous pins - determined when palpating the spinal cord as areas of compaction. Gliomatous pins are localized mainly near the central canal, often dorsal to it. They contain decay products of glial fibers (Rosenthal fibers), collagen fibers, numerous vessels with thickened, sclerotic walls, spongioblasts and other glial cells with signs of incomplete differentiation, multinucleated giant gliocytes. There is an opinion that focal disintegration of gliomatous pins leads to the formation of cavities. Near the cavities, rarefaction of white matter fibers, their swelling, vacuolization and fragmentation, acute swelling of neurons, their retrograde degeneration are found, binuclear neurons, astrocytes with two and three nuclei, glial macrophages, rounded or elongated areas of white matter, in which there are no fibers, are found. myelin. In certain areas of the spinal cord, myelolysis is detected - the dissolution of its substance under the influence of cerebrospinal fluid penetrating from the central canal through areas where there is no ependyma. Some scientists believe that myelolysis also leads to the formation of patol. cavities.
Symptoms of pathology
Syringomyelia disrupts the integrity of the spinal cord. This leads to the development of symptoms of nervous system dysfunction.
Syringomyelia symptoms are caused by the death of nerve cells due to an increase in the size of the spinal canal. The disease is characterized by the following symptoms:
- pain syndrome;
- skin sensitivity disorders;
- Horner's syndrome;
- atrophy of the hands;
- nail damage;
- impaired reflexes;
- eye nystagmus;
- trophic changes in the skin.
The first symptom of the development of the disease is headache. Patients may also complain of pain in the hands and lower extremities. Due to damage to nerve cells, skin sensitivity is impaired. This usually affects the skin of the torso; the lower extremities are involved in sensory disturbances much less frequently. The abundance of injuries, minor household burns and cuts makes it possible to determine the manifestation of sensitivity disorders, since during traumatic exposure the patient does not feel pain and may not notice the damage for a long time.
Horner's syndrome is characterized by a triad of symptoms of a neurological disorder - weakening of the eyelid, enlarged pupils and sunken eyes. In this case, eye nystagmus may be observed - a violation of the movement of the eyeball. Horner's syndrome is usually observed with cervical syringomyelia.
Syringomyelia of the cervical and thoracic spine may be accompanied by trophic changes in the skin and joints. This is manifested by degenerative changes in the joint tissue, as well as the formation of growths and deformation of the joints. Typically the changes affect the shoulder and elbow joints.
Patients may experience gradual atrophy of the hands with corresponding impairment of reflexes. In this case, the nail is affected and the nail plate may peel off. The development of short-term paresis of the limbs is possible.
The progressive disease leads to the development of muscle atrophy, changes in joints, and also affects the lower extremities. However, due to the slow progression of the pathology, complete immobilization of the patient does not occur.
Separately, there is syringomyelic syndrome, which is observed with meningitis and anomalies in the structure of the craniovertebral junction.
Syringomyelia, treatment of which is started in a timely manner, does not affect the patient’s life expectancy.
When does the disease appear?
Syringomyelia is a chronic disease with slow progression. It is not uncommon for a long time to pass from the formation of cavities in the spinal canal to the appearance of the first symptoms and diagnosis - up to 25 years.
Often the dynamics of the development of the disease is very slow, there is no sharp increase in symptoms. In half of the cases, the first symptoms of pathology appear in old age against the background of other diseases that are difficult to tolerate in older age (infectious diseases, pneumonia).
Sometimes the patient may not know about syringomyelia and what it is may emerge by chance during examination of other organs.
Very rarely (no more than 7% of the total number of patients) rapid development of pathology is observed. In this case, with syringomyelia, the symptoms increase over four to five years and ultimately lead to the patient’s disability.
Help in the neurology department
Patient management tactics depend on the cause of syringomyelia.
In some cases, treatment is not carried out if:
- The disease is idiopathic in nature.
- There are severe anomalies of the spine, skull and brain, for which surgery is contraindicated.
- The disease does not progress for several years.
In this case, the doctor monitors the patient over time, but most types of treatment are contraindicated. If the cause of the disease is known, the question of indications for surgery is decided.
Surgical treatment of syringomyelia is an effective method. The operation is also prescribed for the idiopathic progressive form of the disease - with the help of shunts, additional pathways for the outflow of cerebrospinal fluid are created.
Drug treatment is usually prescribed to eliminate symptoms of the disease and maintain the patient's quality of life.
Hospitalization to the neurology department for syringomyelia is mandatory if surgery is involved. Conservative treatment is possible at home.
The issue of temporary or permanent disability of the patient is decided depending on his condition. With a mild course of the disease and work that does not require precise coordination of movements or physical strength, the patient continues to work.
Diagnosis of pathology
The diagnosis is made by a neurologist. First, the doctor examines the patient and analyzes complaints. Particular attention is paid to disorders of skin sensitivity and impaired reflexes. The patient is then referred for the following examinations:
- MRI of the spine and head;
- electroencephalography of the brain;
- examination of cerebral vascular tone;
- cerebrospinal fluid examination.
A magnetic resonance imaging scan may show changes in the structure of the cerebrospinal fluid. MRI can detect the presence of cavities in the spinal canal. Also, to exclude oncological diseases of the brain, tomography is indicated.
If the joints are affected, an x-ray is indicated, which makes it possible to identify degenerative changes and deformation of the joint capsule.
Treatment of pathology
When treating syringomyelia, the severity of the disease plays an important role. At the initial stages, when the proliferation of glial tissue begins, radiation methods are used in therapy to stop this process. In this case, irradiation occurs in those parts of the spine in which pathologically rapid cell proliferation begins. Irradiated preparations of iodine or phosphorus can also be used for this purpose. When introduced into the body, these substances quickly accumulate and irradiate areas with growing tissue from the inside, allowing you to quickly influence this process.
An important stage of such treatment is to protect the thyroid gland and other organs from destructive effects. For this purpose, additional drugs are used.
Drug treatment is prescribed by a neurologist. This therapy includes the following medications:
- vitamin and mineral complexes;
- neuroprotectors;
- dehydrating drugs;
- painkillers.
Drugs are often prescribed to improve the passage of impulses along nerve fibers. For maximum effect, the use of such drugs in conjunction with physiotherapeutic methods, for example, UHF, is indicated.
Medication methods do not allow you to get rid of the cavities that have formed. This therapy is recommended to stop the progression of the disease, and is therefore used in the initial stages and as supportive measures in patients with syringomyelia.
The resulting cavities in the spinal canal cannot be cured with medication. For this purpose, surgical treatment is used. Shunting is usually practiced.
