What is syringomyelia and is there a cure for this disease?
Syringomyelia is a chronic disorder of the central nervous system (usually the spinal cord). According to various sources, the disease occurs from 3 to 17 cases per 100,000 population. The essence of the disease is that glial cells (auxiliary nerve tissue) grow uncontrollably in the spinal cord. Over time, these cells die off, forming cavities in the spinal cord, which gradually fill with cerebrospinal fluid. The consequence of this condition is the death of neighboring neurons, which manifests itself in various neurological symptoms. Most often, "foci" of syringomyelia develop in the cervical (neck) (less often – thoracic (chest) spinal cord, but can also spread to the brain stem.
Causes of Syringomyelia
The reasons that cause the proliferation of glial tissue in the spinal cord are not known to date. Nevertheless, in medicine, two types of the disease are distinguished, which are characterized by different risk factors for its development.
True syringomyelia (congenital)
This is a congenital and hereditary disease, a defect in the development of glial tissue. Why this failure occurred is unknown, but an infectious disease that the mother suffered during pregnancy from, or the child themselves in the first months of life, can become the impetus for the excessive multiplication of glial cells. A spinal cord injury (e.g., birth) can also be a trigger. Most often, true syringomyelia manifests itself in late preschool and early school age.
Not true syringomyelia (acquired)
This condition develops due to an anomaly in the structure of the junction of the skull and spine (craniovertebral junction). Due to the expanded spinal canal, the gray matter does not have a strong "support", and therefore begins to collapse over time, forming the same cavities as in true syringomyelia. Hemorrhages and spinal cord injuries also contribute to the development of the disease. The disease usually manifests itself between the ages of 20-40.
Symptoms of syringomyelia
Since syringomyelia most often develops in the cervical spinal cord, the most characteristic symptom is loss of sensitivity of the arms and trunk (the so-called zones "jacket" and "half jacket". Moreover, the patient feels pressure/touch, but temperature and pain sensitivity is seriously impaired or completely absent. Because of this, serious injuries and burns are possible, constituting a threat to life, which the patient may not even notice and do not seek help in time.
Also often there is atrophy of the hands (less often of the lower extremities) with the loss of reflexes. Various arthropathies, joint deformities, osteoporosis, dysfunction of the bladder, and other disorders can develop.
If the process has captured the brain stem, then the patient develops nystagmus (eyes make repetitive, uncontrolled movements), disorders of bulbar functions (swallowing, speech), and loss of facial sensitivity.
True syringomyelia, which usually develops even in uterus, is often accompanied by deformation of the thoracic cells, scolio zom and other pathologies of the musculoskeletal system, asymmetry of the skull and skeleton in general, high palate, forked tongue, malocclusion, polymastia (additional nipples), polydactyly (six-fingered hands) and other developmental anomalies.
Syringomyelia is also dangerous for its complications:
- The addition of a secondary infection with the development of bronchopneumonia, urethritis or pyelonephritis;
- Infection of wounds that constantly occur in the patient, and their further suppuration and even sepsis;
- The consequences of injuries, wounds, burns that were not detected in time by the patient,
-Bulbar paralysis, which often leads to respiratory arrest (and, as a result, death).
However, regardless of the type of syringomyelia, it is usually a sluggish disease that rarely leads to complete disability of the patient. At the same time, there is also progressive syringomyelia, which is characterized by the emergence of more and more cavities in the spinal cord. This condition is already life-threatening and requires urgent surgical intervention.
Diagnosis and treatment of syringomyelia
As with any complaints of a neurological nature (dizziness, sensory disturbances, paralysis, muscle weakness, back pain, etc.), it is necessary to consult a neurologist. The most accurate diagnosis of syringomyelia is carried out using MRI of the spine. X-ray will detect bone structure abnormalities, but will not detect cavities in the spinal cord, so it can only be used as an auxiliary tool. If for some reason an MRI cannot be performed, myelography is performed.
With regard to treatment, the only way to treat syringomyelia itself is an operation designed to eliminate spinal cord compression and normalize the free circulation of cerebrospinal fluid. At the same time, in order to reduce the rate of progression of the disease, irradiation of the affected areas of the spinal cord with radioactive iodine or phosphorus is used. As for drug treatment (vitamins, neuroprotective agents, analgesics, muscle relaxers, etc.), it is aimed at relieving symptoms, but does not cure the disease itself.
Patients with syringomyelia should carefully monitor their movements to minimize the risk of injury. Also special moderate gymnastics aimed at preventing muscle atrophy and the formation of contractures is recommended.