Syringomyelia
Syringomyelia is a disease of the central nervous system, which is characterized by a chronic slowly progressive course. The anatomical essence lies in the formation of elongated cavities in the spinal cord. In situations where cavities extend beyond the spinal cord and pass into the area of the medulla oblongata, the pathology is called syringobulbia.
The content of the article:
Symptoms of syringomyelia
Diagnosis of syringomyelia
Complications of syringomyelia
Treatment of syringomyelia
Prognosis for syringomyelia
Prevention of syringomyelia
Syringomyelia
Pathology consists in excessive growth and then death of glial cells, which perform a supporting and protective function in nerve tissues. Glial cells are much more numerous than the neurons they protect. In a normal state, the glia layer makes up about half of the entire nervous tissue and, in addition to its main functions, plays an important role in metabolic processes, participates in the formation of a protective layer of myelin neurons.
When the quantity and quality of glial cells are violated, voids form in nerve tissues, respectively, the normal interaction of nerve cells stops and a threat to neurons is created. The cavities formed in the nervous tissue are filled with fluid (lycovrom), increase in size, provoking the proliferation of cysts that squeeze the nerve endings. The pathological process in syringomyelia is localized most often in the upper parts of the spinal cord — the cervical and upper thoracic.
In clinical neurology , there are several causal factors of this disease:
True or idiopathic syringomyelia is a congenital disease that is caused by a violation of the development of the spinal cord during intrauterine embryonic development.
Posttraumatic syringomyelia is an acquired form of the disease caused by spinal injuries.
Syringomyelia, provoked by spinal arachnopathy (chronic lumbago) or arachnoiditis, occurs most often as a complication after surgery for intervertebral hernias of the upper spine.
Syringomyelia as a consequence of spinal cord tumors (tumor of the foramen magnum, cyst of the posterior cranial fossa, intracerebral tumors, extramedullary cysts and tumors), compression (stenosis) of nerve endings.
Syringomyelia caused by compression of the spinal cord by non-tumor formations, for example, intervertebral hernias.
The causes of the development of syringomyelia are not reliably known, despite many studies and attempts to identify factors that provoke the onset of the disease. Congenital pathology occurs due to disorders of the development of the spinal cord at the stage of intrauterine development of the embryo.
Reliable information about the factors that lead to the development of intrauterine spinal cord tissue defects is unknown. Theoretically, disorders of the formation of nerve cells are associated with malnutrition, alcoholism, infectious diseases, exposure to radiation during pregnancy.
Secondary or acquired syringomyelia can develop as a consequence and complication of diseases such as tumors, circulatory disorders, injuries, heavy physical exertion. Recent studies prove that with insufficient blood supply to the central canal of the spinal cord, pathological processes occur, leading to the death of glial cells. The place of dead cells is occupied by intercellular fluid, which leads to the formation of a cyst in the central trunk of the spinal cord.
Symptoms of syringomyelia
For a long period, the disease can occur without obvious signs, which significantly complicates its timely diagnosis. For syringomyelia , the following symptoms are characteristic:
Segmental dissociated sensitivity disorders, in which the patient’s skin loses the ability to feel pain, touch and temperature effects. Depending on the degree of the disease, the areas of the body that lose sensitivity may have different sizes. According to the type of distribution, they are called by their shape — the type of “collar”, “half-jacket” and “jacket”.
In the future, these symptoms can go from a disorder of skin sensitivity to a deeper form — loss of sensation in the joints and muscles.
A characteristic sign of the disease is a sharp decrease in muscle volume, up to atrophy and impaired mobility of the hands.
Vascular disorders and trophic ulcers often appear in the affected area.
The disease is often accompanied by prolonged attacks of dull aching pain in the neck, chest, between the shoulder blades, shoulder joints and arms.
Depending on the area of damage to the nervous tissue, thickening of the skin, compaction and deformation of the joints occur in patients.
Osteoporosis is one of the most common manifestations of the disease. Due to the violation of metabolic processes, calcium is washed out of the bones, from which they become very fragile, which is fraught with fractures and dislocations of the joints.
With the progression of the disease, the patient develops atrophic paralysis of the hands. When the brain stem is involved in the pathological process, loss of sensitivity and paresis of the face, drooping eyelid, atrophy of the tongue, hearing impairment, swallowing and speech function are noted.
With syringomyelia of the upper thoracic region, skeletal deformation (kyphoscoliosis) may occur
. Vegetative disorders manifest themselves in the form of increased sweating in the affected area, a change in the normal skin tone towards redness or cyanosis.
In patients diagnosed with syringomyelia, wounds heal very poorly, even minor injuries and cracks. Often spots form on the skin and nail plates are affected.
Diagnosis of syringomyelia
To identify the type of syringomyelia, the nature of the development and degree of the disease, the following diagnostic methods are used:
Magnetic resonance imaging of the spine in various projections is the main method for diagnosing the disease. The study allows you to visually visualize the state of the spinal cord, reliably find out the area and nature of the lesion of nerve tissues.
X-ray examination is carried out to identify bone tissue disorders, atrophic and hypertrophic processes, osteoporosis, assessment of the condition and degree of damage to damaged joints.
Electromyography is performed most often in the case when it is not possible to apply MRI. This method makes it possible to assess the degree of motor neuron disorders through bioelectric signals coming from the spinal cord.
Otoneurological examination is valuable in case of suspected development of syringobulbia and damage to the nerve tissues of the brain stem.
It is important to assess the psychological state of the patient during the diagnosis of syringomyelia, therefore it is advisable to include a psychologist’s consultation in the list of studies.
Complications of syringomyelia
Syringomyelia provokes a number of diseases of internal organs — often concomitant pathologies are stomach ulcers, disruption of the endocrine glands (pituitary gland, adrenal glands). Due to a violation of the circulatory supply, the development of oxygen starvation of the heart muscle is possible.
With the addition of secondary infection, the respiratory organs (bronchopneumonia) and the genitourinary system (pyelonephritis, urethritis) may be involved in the pathological process in the patient. The danger of the disease flowing into the brain stem is the possibility of developing bulbar paralysis, which leads to respiratory arrest.
Treatment of syringomyelia
At the moment, a complete cure for syringomyelia is impossible. With the help of therapeutic and preventive methods, neurologists have the opportunity to relieve acute symptoms and stop the development of the disease.
The progress of treatment largely depends on the stage at which the disease is detected. At the initial stage, when pathological reproduction of glial cells occurs in the tissues of the spinal cord, therapy is aimed at suppressing their growth. For this purpose , the following methods are used:
Radiotherapy — irradiation of the affected area of the spinal cord. The main purpose of this method is to stop the growth of glia. In recent years, the effectiveness of this technique is increasingly in question due to the fact that in most cases the disease can only be slightly suspended, but it continues to progress.
Treatment with radioactive iodine is a recognized method using the isotope of iodine I-131. The drug is taken in the form of capsules, the dose is calculated individually for each patient. Radioactive iodine suspends the reproduction of glial cells and serves as a prevention of the formation of cavities.
Drug therapy is intended to alleviate the patient’s condition. It is carried out in courses every six months and includes a whole complex of drugs for pain relief (analgesics, NSAIDs), drugs for improving blood circulation and metabolic processes, vitamins, drugs for removing liquor, anticholinesterase agents to improve nerve signal conduction. In addition, in some cases there is a need for anticonvulsive drugs and antidepressants.
Physiotherapy techniques are aimed at relieving pain and improving mobility. The most effective in these cases is the use of UHF, radon baths, electrophoresis, acupuncture, physical therapy, massage.
The question of surgical intervention arises in the most extreme cases with a rapidly progressive form of the disease, a violation of the outflow of cerebrospinal fluid from the brain, a complex combination of syringomyelia with anomalies of the craniovertebral junction. Surgical intervention can have a different character — drainage and shunting of cavities, decompression of the spine, dissection of adhesions forming spinal cord stenosis.
The operation is not able to completely rid the patient of the disease, therefore it is prescribed only in exceptional cases. One of the new methods of treating the disease is dissection of the terminal thread of the spinal cord. Its essence is based on the theory that the cause of congenital syringomyelia and scoliosis is a violation of the blood supply to the spinal cord due to its stretching.
Prognosis for syringomyelia
The disease does not shorten the patient’s life expectancy. With regular comprehensive treatment and constant monitoring of the patient’s condition, he can lead a normal lifestyle. Syringobulbia is a threat to life, since the respiratory organs and the genitourinary system are involved in the pathological process.
Prevention of syringomyelia
Preventive measures primarily concern avoiding the risk of infectious diseases that can provoke the progression of the disease and are fraught with sepsis. Patients are recommended to lead a healthy lifestyle, fully and properly eat, and regularly undergo treatment with a neurologist.
