Syringomyelia is a chronic disease of the central nervous system, in which cavities form in the substance of the spinal cord, and sometimes in the medulla oblongata. True syringomyelia is associated with glial tissue pathology, in other cases, the disease is a consequence of abnormalities of the cranio-vertebral junction. The use of MRI of the spine greatly facilitated the diagnosis of syringomyelia. In cases where MRI is not possible, syringomyelitic cavities can be visualized using myelography. Syringomyelia is not curable, starting at a young age, it accompanies the patient all his life. Patients are treated with symptomatic and neuroprotective therapy, in severe cases – surgical drainage of cavities.
General information
True syringomyelia is caused by a congenital defect of glial tissue, in which excessive growth of glial cells occurs in the gray matter of the thoracic and cervical spinal cord and in the brain stem (syringobulbia). The process of reproduction of glial cells can be started with traumatic injury or infectious disease. Pathologically multiplied glial cells die and cavities lined with glial cells form in the gray matter of the brain. These cells allow fluid to pass through, which accumulates in the cavity and causes its gradual increase. In this case, compression, degeneration and death of adjacent nerve cells occur: motor and sensory neurons.
The steady progression of syringomyelia is due to the constant increase in the cavities formed, accompanied by the death of an increasing number of neurons. With true syringomyelia, congenital deformities and abnormalities of the spine are often revealed: scoliosis, chest deformities, malocclusion, asymmetric structure of the facial skull and other parts of the skeleton, high palate, ear dysplasia, additional nipples of the mammary glands, forked tongue, six-fingered, etc. True syringomyelia is familial in nature and occurs mainly in men aged 25 to 40 years.
In about 65% of cases of syringomyelia, it is not true, but is associated with anomalies of the junction of the skull and spine (cranio-vertebral joint). Such anomalies of the structure lead to the expansion of the spinal canal. The gray matter of those segments of the spinal cord that are at the level of a significant expansion of the spinal canal is destroyed, which leads to the appearance of a clinical picture characteristic of syringomyelia. Cavities similar to those formed with true syringomyelia can form on the site of dead neurons as a result of severe spinal injury, hemorrhage or spinal cord infarction.
Syringomyelia symptoms
In most cases, syringomyelia cavities are formed in the posterior horns of the spinal cord, where there are sensitive neurons responsible for pain and temperature sensitivity. At the same time, large areas with the loss of the corresponding types of sensitivity are determined on the skin, most often they are located on the upper limbs and trunk and with a unilateral lesion have the appearance of a “half—jacket”, and with a bilateral one – a “jacket”. Due to sensitivity disorders in syringomyelia, patients are often injured and get burns, which is often the reason for the first visit to the doctor. But even before the formation of obvious loss of sensitivity, the patient in these areas has sensitive disorders in the form of pain and paresthesia (burning, crawling goosebumps, etc.), which may also be a reason to visit a doctor. Proprioceptive sense and tactile sensitivity in syringomyelia remain intact. Long-term dull aching pains in the neck, interscapular area, arms and chest are characteristic. Loss of sensitivity in the lower part of the trunk and legs is quite rare.
Syringomyelia is characterized by pronounced neurotrophic disorders: thickening of the skin and its cyanosis, poor healing of even minor wounds, deformities of joints and bones, osteoporosis. Patients with syringomyelia often have characteristic changes in the hands: the fingers are thickened, the skin is dry and rough, numerous scars from wounds and burns are visible, there are often suppurated wounds or fresh burns, panaritia are not uncommon. When the lateral horns of the upper thoracic segments of the spinal cord are affected, heiromegaly is observed — a pronounced thickening of the hand. Trophic lesions of the joints (more often shoulder and elbow) in syringomyelia are accompanied by melting of their bone elements with the formation of cavities. A sharp increase in the affected joint is characteristic, there is no pain during movement and noise is heard due to the friction of bone fragments in the joint.
With the progression of syringomyelia, the cavities formed in the spinal cord increase and can capture its anterior horns, which leads to a decrease in muscle strength, impaired movement and the appearance of muscle atrophy. The so-called sluggish paresis of the extremities develops, characterized by a decrease in muscle tone, atrophy and a decrease in deep reflexes. With syringomyelia of the cervical spinal cord, Gorner’s syndrome is noted (drooping of the eyelid, dilation of the pupil, occlusion of the eyeball). When the motor pathways are involved in the process, lower paraparesis is noted, sometimes accompanied by a violation of urination.
If, with syringomyelia, a cavity is formed in the brain stem (syringobulbia), the disease begins with a violation of sensitivity in the external parts of the face. Later, speech disorders, swallowing, breathing (bulbar paresis) gradually increase, atrophy of the tongue, soft palate and half of the face develop. With syringomyelia, secondary infection may occur with the development of bronchopneumonia or urological diseases (urethritis, pyelonephritis). The development of bulbar paralysis in syringobulbia can lead to respiratory arrest and death of the patient.
Diagnostics
X-ray diagnostic methods can reveal trophic manifestations of syringomyelia in the form of osteoporosis, destruction of bone elements of the joint, etc. MRI of the spine allows you to accurately diagnose syringomyelia, and if it is impossible to carry it out, myelography. These examination methods make it possible to see syringomyelic cavities in the spinal cord and trunk.
Treatment
In the initial period of syringomyelia, when there is an active reproduction of glia, treatment methods aimed at suppressing this process are used. Such treatment allows you to stop the progression of the disease and reduce its manifestations. For this purpose, X—ray therapy is used – irradiation of the affected segments of the spinal cord and treatment with radioactive iodine or phosphorus, which tend to accumulate in rapidly multiplying glial cells and irradiate them “from the inside”. Before treating syringomyelia with radioactive iodine, the patient is prescribed a Lugol solution, the iodine from which fills the thyroid cells and thereby protects them from the penetration of radioactive iodine.
Drug therapy of syringomyelia is carried out by a neurologist and consists in the use of dehydrating agents (acetazolamide, furosemide), vitamins, neuroprotectors (glutamic acid, pyrocetam), bendazole. Analgesics (metamizole, aminophenazone) and ganglioblockers (pachycarpine) are indicated for relieving pain syndrome in syringomyelia.
A relatively new method in the treatment of syringomyelia is neostigmine therapy, which improves the conduction of nerve impulses. But it is not aimed at the cause of the disease, but only allows you to temporarily improve neuromuscular conduction. It is possible to combine such therapy with UHF or rhodon baths.
Surgical treatment of syringomyelia is discussed with a large and increasing neurological deficit in the form of central paresis of the legs and peripheral paresis of the hands. Spinal surgeries are performed for drainage of syringomyelic cavities, decompression of the spinal cord, removal of adhesions.
Forecast
As a rule, true syringomyelia is characterized by a slowly progressive course and does not reduce life expectancy. Patients remain able to work for a long period of time. However, if infectious complications occur, sepsis may develop. Syringobulbia is severe, since the involvement of the respiratory center and the vagus nerve in the process leads to a fatal outcome.
Methods of primary prevention of syringomyelia do not yet exist. Secondary prevention is aimed at preventing the progression of the disease, preventing infections, injuries and burns.